Neurology 40 Support Book PDF

Neurology 1 Neurology 1) Cranial nerves 3 2) Hemiplegia 18 3) Paraplegia 23 4) Peripheral neuropathy 29 5) Muscles& Neuromuscular disorders 36 6) Ataxia 43 7) Abnormal invo...

Neurology 1 Neurology 1) Cranial nerves 3 2) Hemiplegia 18 3) Paraplegia 23 4) Peripheral neuropathy 29 5) Muscles& Neuromuscular disorders 36 6) Ataxia 43 7) Abnormal involuntary movements 47 8) Ischemic stroke 54 9) Hemorrhagic stroke 72 10) Epilepsy 80 11) Multiple sclerosis 91 12) Coma 95 13) Headache 108 2  Neurology 1.Olfactory nerve  Function: sense of smell  Pathway: olfactory receptors in nasal mucosa → olfactory fibers (pierce cribriform plate at the base of skull)→ olfactory bulb (at the base of skull)→ olfactory tract → center of olfaction (Bilateral Uncus; in the temporal lobe).  Lesions of olfactory pathway: it leads to either: - 1) Bilateral Anosmia Causes : ✓ ENT causes (e.g Atrophic Rhinitis) a) Anosmia 1) Traumatic: Fracture base of skull (Loss of smell): (fracture cribriform plate) 2) Unilateral Anosmia 2) Inflammatory: basal Meningitis 3) Neoplastic: Meningioma of olfactory groove (foster Kennedy syndrome) https://radiopaedia.org/articles /foster-kennedy-syndrome b) Olfactory Hallucination Irritative lesion in the temporal lobe (Tumor or (false perception of bad smell): Epilepsy). 3  Neurology 2. Optic Nerve  Pathway of optic nerve: see Figure  Lesions of optic pathway : Site of lesion Field defect Light reflex Lesion of optic nerve Ipsilateral loss of vision Loss of direct & consensual light reflex Lesion of the optic Bitemporal Hemianopia chiasma Lesion of the optic tract Contralateral Homonymous Hemianopia Complete Lesion of the Contralateral Homonymous Preservation of the light Hemianopia reflex optic Radiation Lesion of the optic Lower quadrantic Preservation of the light contralateral Homonymous reflex Radiation Hemianopia (Upper fibers) Lesion of the optic upper quadrantic Preservation of the light contralateral Homonymous reflex Radiation Hemianopia (Lower fibers) Lesion of the occipital Contralateral Homonymous - Preservation of the light Hemianopia reflex lobe - Preservation of macular vision(double blood supply) 4  Neurology Light reflex  Stimulus: Exposure of one eye to bright light.  Response: 1) Constriction of the same side (Direct reflex) 2) Constriction of the opposite side (Consensual reflex)  Afferent: - optic nerve  Center: pretectal nucleus(midbrain)→ Bilateral Edinger Westphal Nucleus  Efferent: - oculomotor Nerve Accommodation reflex (Near Reflex)  Stimulus: Exposure of eye to near object.  Response: 1) Convergence of both eyes. 2) Miosis of both eyes 3) Constriction of ciliary muscles → increase convexity of lens  Afferent: optic nerve  Center: occipital lobe (visual center)  Efferent: oculomotor nerve 5 Neurology  A (Light reflex pathway)  B (Accommodation Reflex pathway) NOTES …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………….. 6  Neurology 3. Ocular Nerves  Oculomotor nerve (Cr. N III)  Trochlear Nerve (Cr. N IV)  Abducent nerve (cr. N VI) Oculomotor Nerve Trochlear Nerve Abducent Nerve Muscles Extraocular: Superior oblique muscle Lateral rectus muscle supplied ✓ superior rectus ✓ Inferior rectus ✓ Medial rectus ✓ Inferior oblique ✓ Levator palpebral superiuses Intraocular: ✓ Constrictor puplillae ✓ Ciliary muscle Function 1. Extraocular motility 1. Extraocular 1. Extraocular 2. Elevation of upper eyelid Motility Motility 3. Pupillary constriction (inward& downward) (outward) 4.Constriction of ciliary muscle Lesions 1. Ptosis 1. Diplopia 1. Diplopia 2. Divergent squint (eye (when looking down (when looking out) looks out & down) & in) 3. Diplopia 4. Mydriasis Common 1.Compression(Aneurysm) 1. Ischemic (DM) 1. Compression Causes of 2. Ischemic(DM) (mass) lesions 7 Neurology 4. Trigeminal Nerve  Pathway of Trigeminal nerve:  Function of the Trigeminal nerve: Motor division Sensory Division Muscles of Mastication: 3 Divisions 1) Masseter muscle Ophthalmic 2) Temporalis Muscle Maxillary 3) Pterygoids (medial& lateral) Mandibular Carry sensations from the face Reflexes supplied by Trigeminal Nerve: General sensation from oral cavity a) Corneal reflex(aff.5-eff.7) General sensation from anterior 2/3 b) Palatal reflex (aff.5—eff.10) of the tongue c) Jaw reflex (aff.5 – eff.5) Pain & Temp: Spinal Nucleus in medulla Motor nucleus in the pons Nuclei Deep sensations: Mesencephalic nucleus in midbrain Touch sensation: Main sensory nucleus in pons 8  Neurology  Lesion of the trigeminal nerve: Motor Affection Sensory Affection a) Weakness of muscles of  Peripheral lesion: mastication on the same side a) Loss of sensation on the same side of the face (sparing the angle of the mandible) b) Deviation of the jaw to the b) Loss of general sensation of the anterior 2/3 of the side of weak muscles tongue  Central lesion: (Mainly affects spinal sensory nucleus of the medulla) a) Ipsilateral dissociated sensory loss of the face(loss of pain temp with preservation of touch & deep Sensation) b) Clinical picture varies according to the site of lesion (see below)  Lesion from Above (lower pontine tumor)→ affects lower face  Lesions starting from below (upper cervical lesion) → affects upper face.  Lesion starting from periphery (Tabes dorsalis)→ affects central face  Lesion starting from midline (syringobulbia)→ affects outer face 9 Neurology Trigeminal Neuralgia  Definition: severe attacks of pain along one or more of the sensory branches of the trigeminal nerves, usually (maxillary or mandibular).  Etiology: 1) Idiopathic (most common) 2) Nerve compression at cerebellopontine angle by: Tumor Aneurysm Vascular loop of (superior cerebellar artery) 3) Multiple sclerosis (MS)  Clinical picture: Pain is severe, agonizing, Lancinating, like electric shock Recurrent & the patient is free in-between Precipitated by jaw movement (mastication, laughing, toothbrushing)  Treatment: A. Medical treatment: Carbamazepine Pregabalin Gabapentin Amitriptyline NSAID & Acetaminophen B. Surgery: Decompression of the affected nerve 10  Neurology 7. Facial Nerve  Function: Motor function Sensory function Autonomic Reflexes function Muscles of expression Taste sensation of Lacrimal Corneal reflex: of the face: Anterior 2/3 of the submandibular & Aff: trigeminal n. Frontalis tongue sublingual salivary Eff: Facial n. Orbicularis oculi glands Nasalis Glabellar reflex: Retractor anguli Aff: facial n. Orbicularis orris Eff: facial n. Buccinator  Anatomy : 11  Neurology  Differentiation between UMNL & LMNL of the facial Nerve: UMNL LMNL Site ✓ Affects pyramidal tract above the ✓ Affects the facial motor facial nucleus nucleus, or the facial nerve itself Muscles ✓ Paralysis of the muscles of the ✓ Paralysis of all muscles of the lower half of the face on the face (upper & lower halves) of opposite side of the lesion the same side of the lesion (supplied from the opposite pyramidal only) Movement ✓ Paralysis involves the voluntary ✓ Paralysis involves voluntary, movement, BUT: spares the emotional & associated emotional & associated movements Movements (supplied by the extrapyramidal fibers) Association ✓ Paralysis is associated with ✓ Paralysis is associated with hypertonia & hyperreflexia hypotonia & hyporeflexia Hemiplagia ✓ There’s associate hemiplegia on ✓ If there’s hemiplegia ,it’s on the same side of the facial the opposite side of the facial paralysis paralysis ✓ (crossed hemiplegia) Causes 1) Vascular: stroke 1) Pontine lesion(nuclear level) 2) Trauma: head injury 2) Cerebello-pontine angle lesion 3) Inflammatory:postencephalitic 3) Facial canal lesion 4) Demyelinating: Multiple sclerosis 4) Extracranial lesion 5) Neoplastic: Glioma 12  Neurology Bell’s palsy  Definition: ✓ It’s an acute LMN paralysis of the face , due to a non-suppurative inflammation and edema of the facial nerve in the facial canal near the stylomastoid foramen. ✓ It’s usually unilateral ,may be recurrent & sometimes runs in families  Etiology Unknown (many causes have been suggested) 1. Air draft 2. Viral infections 3. Autoimmune  Clinical picture: 1) Pain: - May be painless - If pain occurs (acute pain behind ipsilateral ear) 2) Paralysis: - Complete paralysis of the facial muscles on the affected side of LMN features  Complications: 1) Corneal ulcers 2) Incomplete recovery  Treatment: 1) Physiotherapy 2) Drugs: - Oral corticosteroids (prednisone 1mg/kg/day for 7 days) - Oral antiviral drug (Acyclovir) - Vitamin B complex 3) Eye protection: - Artificial Tears (through the day) - Gel during sleep 4) Surgical treatment (for resistant cases) - Facial nerve grafting - Plastic surgery (for residual asymmetry) 13 Neurology 8. Vestibulo-cochlear nerve  It has 2 divisions: Cochlear Division Vestibular Division Hearing Equilibrium function Irritative: tinnitus Vertigo Lesion Destructive: Deafness Nystagmus Disequilibrium(vestibular Ataxia) at the same side of lesion Pathway of cochlear nerve Pathway of Vestibular nerve 14  Neurology Vertigo  Definition: It’s the sense of rotation of the body in steady surroundings or the reverse  Clinical picture: 1) The body may be felt to rotate or fall while the surroundings is steady or 2) The surroundings themselves appear to rotate around the body 3) It’s aggravated by movement of the head or the body 4) Usually associated with autonomic manifestations in the form of (nausea, vomiting, sweating & pallor) 5) Usually associated with Nystagmus & Ataxia (Vestibular ataxia)  Should be differentiated from dizziness (where no sense of rotation)  Etiology: Labyrinthitis Labyrinthine Meniere’s Disease Peripheral nerve Vestibular neuritis Cerebellopontine angle lesion(e.g Acoustic neuroma) Brain stem & Stroke (Ischemic or hemorrhagic) Cerebellar lesions Encephalitis Multiple sclerosis (MS) Transient Ischemic Attack(TIA) of vertebrobasilar circulation Benign Positional Comes in attacks vertigo(BPV) Related to changes of head position Idiopathic (inner ear disease) Good response to rehabilitation exercises  Treatment: 1) Treatment of the cause 2) Rehabilitation exercises for (BPV): Dix Hallpike test & Epley Maneuver 3) Drugs: https://en.wikipedia.org/wiki/Dix%E2%80%93H - Cinnarizine allpike_test - Betahistine Dihydrochloride https://en.wikipedia.org/wiki/Epley_maneuver 15 Neurology (9) & (10) vagus & Glossopharyngeal nerve  Function: Glossopharyngeal nerve Vagus Nerve Constrictors of pharynx Palate Motor Pharynx larynx Tongue (post 1/3) Visceral sensation Sensory Pharynx (abdomen& thoracic) Tonsils Autonomic Parasympathetic to parotid Parasympathetic to heart, GIT, bronchi  Palatal reflex: Afferent: Trigeminal N. Efferent: Vagus N. Reflexes  Pharyngeal reflex: Afferent: Glossopharyngeal N. Efferent: Vagus N.  Lesion: Bulbar Palsy True Bulbar Palsy Pseudo-bulbar palsy Dysarthria Dysphagia symptoms Dysphonia Nasal regurge Hyporeflexia Hypereflexia Palatal & Absent pharyngeal reflex Nucleus UMNL(above Nucleus) Nerve causes NMJ. Muscle 16  Neurology (11) Accessory Nerve  It’s composed of: 1) Cranial part (runs with vagus & glossopharyngeal nerves)& 2) Spinal part (originates from cervical C1-C5)  Function of spinal accessory nerve: - Pure motor supply to muscles of the neck o Sternomastoid muscle o Trapezius muscle  Lesion of spinal accessory nerve: Sternomastoid paralysis Trapezius paralysis Inability to turn the head to Shoulder drop Unilateral opposite side Inability to raise shoulder Inability to flex the neck Head drop Bilateral Inability to extend the neck (12) Hypoglossal Nerve  Function: Pure motor supply of intrinsic muscles of the tongue  Lesion: UMNL LMNL Deviation to the opposite of the side Deviation to the same side of Unilateral of lesion in the brain (see Fig below) lesion Bilateral Inability to protrude the tongue Inability to protrude the tongue 17 Neurology  Definition: Weakness of one side of the body due to pyramidal tract lesion from its origin in cerebral cortex down to the fifth cervical segment (beginning of origin of brachial plexus).  Types: 1. Congenital 2. Acquired  Classification: 1. Clinical:  Onset and course o Acute onset and regressive course (vascular, infection and traumatic) o Gradual onset and progressive course (neoplastic) o Relapsing and remitting course (M.S)  Symptoms and signs (vary according the onset) o Acute lesion: the clinical picture passes through two stages → - Stage of flaccidity due to neuronal shock - Stage of spasticity: established hemiplegia o Gradual lesions →The hemiplegia passes directly to the stage of spasticity A. flaccid hemiplegia (shock stage) B. Spastic hemiplegia 18  Neurology A. flaccid hemiplegia (shock stage) B. Spastic hemiplegia  It lasts from 2-6 weeks the shorter  There is pyramidal distribution of the duration the better the prognosis weakness  On the paralyzed side there is  It affects the progravity more than the complete loss of muscle tone and antigravity muscle absence of deep reflexes and Babinski sign become positive  The hands and the feet are weaker than the shoulders and hips  It affects distal more than proximal muscles  During recovery from shock stage the  In upper limb extensors are weaker muscle tone and deep reflexes than flexors reappear and gradually increase  In lower limb flexors are weaker than extensors  There is a spasticity of the paralyzed muscles of clap-knife type  There are exaggerated deep reflexes  Loss of superficial reflexes  Positive Babinski sign  Circumduction gait 19 Neurology 2. Anatomical A. Cerebral : 1) Cortical, subcortical (corona radiate) 2) Internal capsule -Coma if the lesion is extensive - Complete hemiplegia with U.M.N facial -Convulsion if the lesion is irritative and hypoglossal paralysis on the opposite side of the lesion -Contralateral cortical sensory loss if the parietal lobe is involved - Hemihypothesia on the opposite side of the lesion -Aphasia and agraphia if the lesion is dominant hemisphere - Hemianopia may occur, if the fibers of optic radiation in the capsule are -Homonymous hemianopia if the lesion involved involves the parieto-occipital region -No convulsions, aphasia or coma -The paralysis usually involves one limb (monoplegia) especially in vascular lesions B. Brain stem : 1) Crossed hemiplegia -Hemiplegia on the opposite side of the lesion -Cranial nerve paralysis of L.M.N on the same side of lesion 2) Midbrain  Weber's syndrome -Hemiplegia on the opposite of the lesion -3rd cranial nerve paralysis on the same side of the lesion  Benedict's syndrome -Hemiplegia on the opposite of the lesion -3 rd cranial nerve paralysis on the same side of the lesion -Hemiataxia (intention tremors) on the opposite of the lesion due to affection of the Red Nucleus 20  Neurology 3) Pons  Millard Gubler's syndrome - Hemiplegia on the opposite side of the lesion - 6 th and 7th cranial nerves paralysis on the same side of the lesion  Foville's syndrome - Hemiplegia on the opposite side of the lesion - Loss of conjugate deviation of the eyes to the same side of the lesion due to lesion in MLB 4) Medulla  Avellis' syndrome - Hemiplegia on the opposite side of the lesion - 9 th and 10th cranial nerves paralysis on the same side of the lesion  Jackson's syndrome - Hemiplegia on the opposite side of the lesion - 11th and 12th cranial nerves paralysis on the same side of the lesion C. Spinal cord: 1) Brown sequard syndrome  DEF: -The lesion is in one side of the cord and it sustained between C1 and C5 segment is caused by stab wound, disc prolapse, M.S or tumor  Clinical presentation A) At the level of the lesion - Ipsilateral localized L.M.N.L of the muscle supplied by the affected segments - Ipsilateral loss of all sensations in the area supplied by the dorsal roots of the affected segments B) Below the level of the lesion - Ipsilateral hemiplegia - Ipsilateral deep sensory loss - Contralateral superficial sensory loss for pain and temperature - Touch diminishes on both sides 21 Neurology  Causes & Etiology: 1) Vascular, cerebrovascular stroke is the most common cause of hemiplegia 2) Trauma, brain contusion, depressed fracture of skull, 3) Infection, meningitis, encephalitis, brain abscess 4) Demyelination, multiple sclerosis MS 5) Space occupying lesion, brain tumor glioma meningioma 6) Congenital, cerebral palsy 7) Hysterical  Treatment  General management: Care of skin Care of respiration Care of nutrition and fluid balance Care of urinary bladder Control general medical conditions Likes diabetes mellitus, hypertension  Specific treatment:- Treatment of the cause: ✓ Cerebral infarction ✓ Cerebral hemorrhage ✓ Multiple scleroses ✓ CNS infection ✓ CNS tumor NOTES ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… …………….………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… ……………………………………………………………………………………………………………………………………… 22 ………………………………………..  Neurology  What is paraplegia? Paraplegia is paralysis or weakness (paraparesis) of both lower limbs. It may be due to: - Pyramidal (U.M.N.) lesion resulting in spastic paraplegia. - Lower motor neuron (L.M.N.) lesion resulting in flaccid paraplegia. 1- flaccid paraplegia (LMNL) Decreased tone of muscles along with weakness Occur in lower motor neuron disease Decrease tone and reflexes associated with atrophy of muscles flexor or equivocal planter associated with or without fasciculations 23 Neurology 2- Spastic paraplegia (UMNL) Bilateral pyramidal tract lesion Spinal paraplegia (most commonly) Cerebral paraplegia (brain stem or the cerebral parasagittal region) Cerebral paraplegia A. Causes in the Parasagittal Region: (area B. Causes in the Brain Stem: of cortical presentation of L.L.) 1. Traumatic e.g. depressed fracture of the Syringobulbia and midline tumors. vault of the skull, subdural These lesions arise in the midline hematoma. and involve the innermost fibers 2. Vascular e.g. superior sagittal sinus which are those of the lower limbs. thrombosis. 3. Inflammatory e.g. encephalitis, meningio- encephalitis. 4. Neoplastic e.g. parasagittal meningioma. 5. Degenerative e.g. cerebral palsy. 24  Neurology Spinal paraplegia 1. Focal: paraplegia with sensory level. 2. Systemic. 3. Multifocal. Focal spinal paraplegia 1-compression 2- inflamatory 3-vascular 1. Vertebral: Fracture or fracture- Transverse anterior spinal dislocation of the vertebra myelitis artery occlusion Disc prolapse & Spondylosis Myelomeningitis Pott's disease Myeloradiculitis. Neoplastic diseases: Primary or metastatic and Deformity of the vertebral column as kyphoscoliosis. 2. Meningeal Extradural e.g. leukaemic (extramedullary): deposits. Dural e.g. meningioma. Intradural.g. neurofibroma. 3. Cord Syringomyelia (intramedullary): Systemic paraplegia A. Hereditary spastic B. Sub acute combined C. motor neuron disease. paraplegia. degeneration. Multifocal cause: multiple sclerosis. 25 Neurology Focal spinal paraplegia Clinical picture A. At the level of the lesion: 1) Vertebral manifestations: only present if the cause is vertebral. ✓ Localized pain or tenderness. ✓ Localized deformity or swelling. 2) Radicular manifestations: only present in extra-medullary causes. a) Posterior root affection: - Early ,pain in the back referred to the distribution of the affected root and described as girdle pain - Later, there is hypoesthesia or anesthesia in the dermatome supplied by the affected root. b) Anterior root affection: localized L.M.N. weakness in the muscles supplied by the affected root. B. Below the level of the lesion: (cord manifestations): 1) Motor Manifestations: acute or gradual. a) If the cause is acute (inflammation, vascular or traumatic), the paraplegia passes through 2 stages: Stage of flaccidity due to neuronal shock: lasts from 2 to 6 weeks. Stage of spasticity: the full picture of U.M.N.L. will be established including: hypertonia, hyper reflexia, positive Babinski sign & may be clonus. b) If the cause is gradual (e.g. neoplastic): The shock stage is absent Gradual progressive weakness of LL with hypertonia and hyper reflexia. The weakness affects distal, abductors and progravity Spasticity affects (antigravity). (Paraplegia in extension) With further progression of the lesion, the extrapyramidal fibers in the cord will be affected. (Paraplegia in flexion). 26  Neurology 2) Sensory Manifestations: a) Extra medullary: - sensory level: All types of sensations lost below it. - early loss of sensation in the saddle area (S 3, 4, 5) b) Intramedullary: - A jacket sensory loss - A dissociated nature - Sacral spare 3) Sphincteric Manifestations a. In acute lesions: retention of urine followed by precipitancy. b. In gradual lesions: precipitancy (late in extra medullary lesions and early in intramedullary lesions) as the pyramidal fibers controlling the bladder center lie medially in the cord Investigations  Blood routine examination: Total count, differential count, ESR  Blood film  X ray spine (AP and lateral view) may show: ✓ TB spine ✓ Collapse or erosion vertebrae ✓ Herniated intervertebral disc ✓ Metastatic deposits ✓ Fracture or dislocated vertebrae  CSF examination  MRI spine 27 Neurology Management of Paraplegia I. General: ✓ Frequent change of the patient's posture to guard against bedsores. ✓ Care of the skin by frequent washing with alcohol followed by talc powder. ✓ In case of urinary incontinence, frequent change of bed sheets. ✓ Care of the bladder: If there is retention, use a catheter to evacuate the bladder. II. Physiotherapy III. Symptomatic Treatment: Analgesics and sedatives for pain, Muscle relaxants for the spasticity, Vitamins and tonics. IV. Specific Treatment: (treatment of the cause) 1) Antituberculous drugs in Pott's disease. 2) Deep X—ray in case of intramedullary tumors. 3) Surgical eradication in case of extra medullary tumors. 4) Transverse myelitis: Methyl prednisolone 1 gm/day for 7-10 days Plasma pharesis. NOTES …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………….. 28  Neurology  Definition: It's inflammation or degeneration of the peripheral nerves and/or the cranial nerves leading to dysfunction (decreased conductivity) resulting in motor, sensory or autonomic manifestations.  Classifications: - A. Pathological Classification 1) Axonal Neuropathy (poor prognosis) 2) Demyelinating Neuropathy ( Good prognosis) 3) Mixed neuropathy (Axonal & Demyelinating) B. Clinical classification 1) Motor Neuropathy 2) Sensory Neuropathy 3) Mixed (sensory& Motor )Neuropathy 4) Autonomic neuropathy C. Anatomical Classification (see figure) 1) Mononeuropathy = Single Nerve Affection. 2) Polyneuropathy = Symmetrical multiple nerves affection in 4 limbs. 3) Mononeuropathy Multiplex (Multiple Mononeuropathy) = 4) asymmetrical multiple nerves.  Diagnostic tests of peripheral Neuropathy: 1. Nerve conduction studies & Electromyograpgy (NCS & EMG) 29  Neurology Mononeuropathy: Causes Examples of cranial mononeuropathy: 1) Entrapment (ex: Median nerve entrapment at the wrist 1) Facial nerve: Bell's Palsy. (carpal Tunnel syndrome). 2) Cochlear nerve: 2) Trauma (ex: Fracture humerus→ Radial mononeuropathy) Sensorineural Hearing loss. 3) Wrong injection into the nerve→ sciatic mononeuropathy 3) Trigeminal nerve: due to wrong intramuscular injection. Trigeminal neuralgia 4) Infection: Herpes zoster virus mononeuropathy. 4) Abducent nerve :squint 5) Tumor invasion: Pancoast tumor→sympathetic chain 5) Oculomotor nerve: squint →Horner syndrome  Causes of multiple mononeuropathy: 1) DM 2) Vasculitis 3) Malignancy(paraneoplastic) 4) Others: (HIV- sarciodosis- amyloidosis-Leprosy). Polyneuropathy  Clinical manifestations of polyneuropathy: -  A. Motor Manifestations  Weakness: - - LMNL (wasting-Hypotonia-Hyporeflexia). - Bilateral & symmetrical - Lower limbs > Upper limbs - Extensors > Flexors  Motor cranial neuropathy: - Facial Nerve - Oculomotor Nerve - Abducent Nerve - Glossopharyngeal& Vagus Nerves 30  Neurology  B. Sensory Manifestations  1. Irritation stage: - Pain - Paresthesia (pins & needle) - (Lower limbs> upper limbs) - (Distal > Proximal)  2. Destruction stage: - Superficial sensory loss (stocks & Gloves distribution) - Deep sensory loss(vibration & position ) - Sensory Ataxia  C. Autonomic manifestations - It depends on which system is affected more(sympathetic or parasympathetic)  Features of autonomic instability: a) Sweat glands → Anhidrosis b) Stomach → Rapid fullness after meals c) Intestine → Abdominal Bloating d) Rectum → constipation vs. fecal incontinence e) Bladder → Retention vs. urine incontinence f) Blood vessels → orthostatic hypotension g) Heart → Tachycardia vs. bradycardia h) Genitalia→ Erectile dysfunction or failure of ejaculation i) Dizziness j) Sleep disturbance  Causes of polyneuropathy: 1) Hereditary. 2) Immune mediated neuropathy. 3) Infection. 4) Endocrinal 5) Drugs. 6) Toxins 7) Nutritional 8) Neoplastic (paraneoplastic) 31 Neurology 1. Hereditary polyneuropathy (Charcot Marie Tooth peripheral neuropathy) CMT (Peroneal muscle Atrophy)  Criteria of CMT-PN: (Same clinical features of polyneuropathy, with specific features): 1) Child onset 2) Gradual, slowly progressive course 3) Genetics :Autosomal (dominant or recessive) or X-linked inheritance. 4) Mild sensory manifestations 5) Severe wasting with mild weakness 6) Peroneal muscles atrophy (inverted Champaign bottle ) 7) Pes cavus. 2. Infective polyneuropathy viral Bacteria mycobacterium Mumps Diphtheria Leprosy Measels Typhoid tetanus 3. Drugs & Toxins causing polyneuropathy Drugs Toxins INH Heavy metals: Cycloserine lead Phenytoin Mercury Arsenic Alcohol 32  Neurology 4. Nutritional causes Vit B1 Vit B6 Vit B12 Niacin Beri-Beri Peripheral Subacute pellagra neuropathy combined Dermatitis degeneration  Common causes of Vit B deficiency: 1) Malnutrition 2) Loss (vomiting-diarrhea-malabsorption) 3) Alcoholic  Common Targets of Vit B deficiency: 1) Mucous membranes (Tongue & GIT) → ulcers, diarrhea 2) Nerves → peripheral neuropathy 3) Skin & Hair → loss of hair, dermatitis 4) Brain & mental function→ confusion, Encephalopathy, Dementia 5. Endocrinal causes of polyneuropathy 1) Diabetes mellitus 2) Acromegaly 3) Myxedema Diabetes mellitus neuropathy:  Causes: - Diabetic Microangiopathy of vasa nervosa Loss of vitamin B complex in urine Toxic effects of hyperglycemia & Ketone bodies  Clinical forms of diabetic neuropathy: Cranial Neuropathy Entrapment Neuropathy Distal sensory neuropathy Autonomic Neuropathy Distal Motor neuropathy Clinical Picture of diabetic polyneuropathy:- see Proximal Motor neuropathy Above(Clinical manifestations of polyneuropathy) 33 Neurology 6. Immune mediated causes of polyneuropathy A. Guillain Barre Syndrome (AIDP) B. Other autoimmune diseases associated with P.N: - Rheumatoid arthritis - Systemic lupus Erythematosus (SLE) - Polyarteritis Nodosa (PAN) Gullian Barre syndrome (Acute Inflammatory demyelinating polyradiculoneuropathy)  Definition: Acute inflammation (autoimmune) disease of spinal nerves root & cranial nerves myelin sheath.  Etiology : Autoimmune disease following respiratory tract infection ,urinary tract infections & vaccination.  Pathology: Demyelination of spinal roots & cranial nerves.  Clinical picture: A. Febrile stage: respiratory or GIT symptoms B. Latent stage: fever subsides & patient is free for 1-3 weeks C. Paralytic stage: o Weakness of lower motor nature (hypotonia & Areflexia, but no wasting) o Ascending pattern of weakness starting from lower limbs → Trunk muscles→ upper limbs → Respiratory muscles (Dyspnea)→Cranial nerves palsy (Facial, glosspopharyngeal & vagus nerves). o Weakness is proximal more than distal (pelvic muscles & shoulder muscles) o Weakness is associated with areflexia o Mild sensory symptoms: stocks & gloves paresthesia o Autonomic instability (Fluctuating blood pressure heart rate) 34  Neurology  Prognosis: o Good prognosis as recovery is the rule, unless no respiratory distress.  Investigations: A. CSF analysis: - Cytoalbuminous dissociation(increase in protein level with normal cell count) B. Electrophysiological study (Nerve conduction & (normal CSF analysis: https://globalrph.com/medical/co Electromyography): mmon-laboratory-lab-values-csf- - Picture of demyelinating polyradiculoneuropathy analysis/ - Results become positive after 10 days from onset.  Treatment: A. Admission to ICU (monitoring of vital signs) (N.B: no role for steroid B. Plasmapheresis (Plasma Exchange) or in AIDP) C. IVIG (Intravenous Immunoglobulins) D. If there’s respiratory distress → Mechanical ventilation NOTES …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… …………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………….. 35 Neurology  Symptoms of muscle disease Muscle fatigue, exercise intolerance in general Proximal and symmetric weakness Waddling gait; difficulty of rising from sitting, climbing stairs; Gower’s sign Hyperextension of the knee Increased lordosis of the lumbar spine, scoliosis Contractures, tight Achilles tendons Muscle atrophy; pseudohypertrophy of calf Myotonia Tendon reflexes are normal or decreased  Diagnosis of muscle diseases Creatinine kinase levels increased in many myopathies (sign of muscle fiber necrosis) EMG: differentiation between neurogenic and myogenic weakness Muscle biopsy: signs of muscle fiber abnormality, inflammation,immunostaining of muscle constituents Genetic testing  Investigations ✓ Genetics ✓ Muscle Biopsy ✓ NCS / EMG ✓ Lab ( CK)  Types of muscle diseases Hereditary muscle diseases Acquired muscle diseases Muscle dystrophies Inflammatory myopathies Muscle channelopathies Endocrine and toxic myopathies Mitochondrial myopathies Infectious muscle diseases Metabolic myopathies 36  Neurology Muscle dystrophies  Hereditary myopathies, characterized by progressive weakness and muscle atrophy  Genetic defect of proteins constituting the sarcolemma associated cytoskeleton system Duchenne muscular dystrophy Dystrophinopathy First described in 1881- dystrophin gene discovered in the early 1980's Becker’s type: reduced amount of dystrophin; more benign course X-chromosome linked 1 in 3500 live births, occurs in boys, girls are carriers  Cause: deficiency of dystrophin, resulting in progressive loss of muscle fibers  Diagnosis: Lack of immunostaining of dystrophin in muscle biopsy specimen Demonstration of deletion in the dystrophin gene Onset at 3-5 years Initial symptoms: difficulty getting up from deep position and climbing steps , waddling gait Weakness most pronounced in limb-girdle muscles, trunk erectors; craniobulbar muscles are spared Skeletal deformities Inability to walk by 9-11 years Cardiomyopathy Death occurs usually in the 3rd decade, from respiratory insufficiency Gowers’ Maneuver 37 Neurology Myotonic dystrophy 1 Prevalence: 1 in 8000 Autosomal dominant inheritance, with anticipation Multisystemic disease: ✓ Myotonia: hyperexcitability of muscle membrane ✓ Progressive muscular weakness and wasting, most prominent in cranial and distal muscles ✓ Cataracts, frontal balding, testicular atrophy ✓ Cardiac abnormalities, mental retardation  Cause: CTG repeat expansion in a gene on chr. 19 Limb-girdle dystrophies  Causes: 1) Sarcoglycanopathies 2) Calpain deficiency 3) Caveolin deficiency 4) Dysferlin deficiency etc. 5) Sarcoglycanopathies comprise about 10% of autosomal recessive limb-girdle dystrophies 6) α, β, γ, δ sarcoglycans Facioscapulohumeral dystrophy  Prevalence: 1 in 20,000  Autosomal dominant  Age of onset: infancy to middle age  Progressive muscular weakness and atrophy involving the face, scapular, proximal  arm and peroneal muscles  Life span is not significantly affected. 38  Neurology Inflammatory muscle diseases 1) Dermatomyositis 2) Polymyositis 3) Inclusion body myositis 4) Other systemic autoimmun diseases (SLE, Sjögren sy. etc.) Dermatomyositis autoimmune disease affecting the muscles and skin (microangiopathy) Symptoms progress over weeks, months Rash on the face and neck Periorbital oedema Pain and weakness of proximal limb muscles, neck flexors Dysphagia Cardiac abnormalities, interstitial lung disease Often paraneoplastic Polymyositis Cell-mediated immune response against muscle fibers Symptoms are similar to DM, no skin involvement Less often paraneoplastic than DM  Diagnosis / therapy of DM, PM  Blood CK levels are elevated  EMG: myogenic findings  Muscle biopsy: inflammation  DM: perivascular infiltration, mainly in the perimysium  PM: endomysial inflammation  Therapy: immunosuppression, long-term treatment with corticosteroids (1 mg/kg/day) 39 Neurology Endocrine and toxic myopathies Toxic myopathies: Endocrine myopathies Drugs aimed at reducing blood lipid Thyrotoxic myopathy levels: statins Hypothyroidism Corticosteroids – steroid myopathy Hyperparathyroidism Alcohol in a couple of days Adrenal insufficiency Heroin Hypokalemia Disorders of the Neuromuscular Junction Myasthenia gravis  Definition An autoimmune disease due to an antibody mediated attack directed against nicotinic AchR at neuromuscular junction 10% have thymoma 70% have thymic hyperplasia  Epidemiology Incidence 1 : 20 000 in USA Women slightly higher incidence 3 : 2 Majority of the MG are young women in the third decade and middle aged men in 5th and 6th decade Children account for 11% of all patients with MG 40  Neurology  Clinical Features Fluctuated muscle weakness and excessive fatiguability Diplopia and ptosis Bulbar weakness, dysarthria Generalized weakness of the trunk and extremities  Diagnostic algorhitm Electromyography Anti AChR antibodies MR of mediastinum – thymomas, hyperplasia  Treatment Thymectomy Plasma exchange in myasthenic crisis (TPE) Human immune globuline (IvIg) Corticosteroids AChE inhibitors (pyridostigmine, distigmine) 41 Neurology Myasthenic crisis  Respiratory insuficiency – paralysis of respiratory muscles  Assisted ventilation required  Affect 15-20% myasthenic patients  Females : males = 2 : 1  Average age : 55 years  Clinical features Respiratory tract infection, pneumonia ( 38%) Respiratory failure 99% Oropharyngeal or ocular weakness 86% Arms and legs weakness 76%  Complication of crisis Ateletactic pneumonia (40%) Hypotension Cardio-respiratory arrest Pneumothorax  Treatment ICU is required for assisted ventilation Cardiopulmonary monitoring Plasmapheresis ( 5 sessions) or IvIg 2g/kg in five consecutive days Antithrombotic treatment Antibiotics Respiratory rehabilitation  Outcome Duration of intubation : 13 days Duration of hospitalization : 35 days Tracheostomy 40% Mortality 4% 42  Neurology  Definition Incoordination of voluntary motor activity in the absence of motor weakness.  Anatomical background Vermis 2 cerebellar hemisheres Folloculonodular lobe (Archi, Vestibulocerebellar) Anterior lobe ( Paleocerebellar, Spinocerebellar) Posterior lobe (Neocerebellar, Pontocerebellar  Physiological background Archicerebellum ✓ Equilibrium Paleocerebellum ✓ Muscle tone Neocerebellum ✓ Coordination of movements Cerebellar syndromes Archicerebellum syndrome ▫ Wide-based-swaying truncal ataxia (on standing) Types of ataxias ▫ Drunken gait (on walking) Cerebellar Neocerebellum syndrome (in-coordination) Sensory ▫ Nystagmus Vestibular ▫ Dysarthria Combined ▫ Nodding, titubation Hysterical ▫ Intention kinetic tremors ▫ Deviation and zigzag gait Hypotonia and hyporeflexia. 43 Neurology Cerebellar ataxia ▪ Clinical picture ▪ Clinical tests for co-ordination ▪ Causes (etiology)  Clinical picture ✓ Wide based gait ✓ Incoordination ✓ Hypotonia and hyporeflexia ✓ Positive co-ordination tests for ataxia  Clinical tests  Finger to nose  Finger to finger  Finger to doctor’s finger  Dysdiadokokinesia  Rebound phenomenon  Buttoning and unbuttoning  Heel to knee  Walking on a straight line  Etiology Herido-familial Idiopathic ▫ Friedreich Senile ▫ Marie Symptomatic ▫ Congenital ▫ Infective ▫ Vascular ▫ Toxic ▫ Neoplastic ▫ Demyelinating ▫ Paramalignant 44  Neurology Friedreich ataxia AR, onset in late 1st decade Skeletal deformities ECG changes  C/P: ✓ Archi-cerebellar lesion, Truncal ataxia ✓ Pyramidal tract lesion ✓ Posterior column ✓ PN Marie’s ataxia 2 nd, 3rd decade  C/P ✓ Neocerebellum ✓ Pyramidal ✓ Mental impairment ✓ Ocular nerve palsies ✓ Extrapyramidal syndromes Sensory ataxia Ataxia due to loss of deep proprioceptive sensations at any level from peripheral nerve to the cortical sensory area.  C/P: ✓ Kinetic tremors on eye closure only ✓ Positive Rhomberg’s test ✓ Deep sensory loss ✓ Hypotonia and hyporeflexia 45 Neurology Vestibular ataxia Ataxia due to lesions of the vestibular division of the eighth nerve.  C/P: ✓ Vertigo, tinnitus and deafness ✓ Nystagmus ✓ Impaired tests for vestibular function (caloric test and rotatory chair)  Causes: ✓ Vestibular neuritis ✓ Labyrinthitis ✓ Drugs (ototoxic drugs) ✓ Meniere’s disease NOTES …………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… ………………………………………………………………………………………………………………………………… …………………………………………………………………………………………….. 46 ……………………………………………………………………………………………………………………………………  Neurology  Enumeration 1. Tremors (Static only) 2. Chorea N.B. Convulsions and kinetic cerebellar 3. Athetosis tremors are not included in involuntary 4. Dystonia movements. 5. Tics 6. Myoclonus  Static tremors  Parkinsonism  Essential ◦ It occurs below age 25 years ◦ Mostly familial ◦ Stationary throughout life ◦ Respond to propranolol  Hysterical (psychic)  Metabolic (thyrotoxicosis, liver failure)  Toxic: drug or toxin exposure e.g. alcohol, mercury, cocaine, valproate, B stimulants, Lithium etc.  Senile 47 Neurology Parkinsonism  Definition ◦ It’s a disease resulting from dopamine deficiency in the basal ganglia and substantia nigra leading to static tremors, slowness of movements and rigidity.  Etiology ✓ Primary (idiopathic, degenerative) ✓ Secondary  Vascular  Neoplastic  Toxic  Traumatic  Inflammatory  Clinical picture  Tremors  Regular ,rhythmic, at rest, 4-8 Hz, pill-rolling  Usually begins unilateral.  ↑ with stress , ↓ with sleep and voluntary movements  Rigidity  P>D  Flexed posture, lead pipe or cog-wheel  Hyporeflexia  Bradykinesia (slow movements)  Mask face  Monotonus speech  Decreased arm swinging  Slow shuffling gait (festinant) with forward propulsion.  Manifestations related to the cause. 48  Neurology  Treatment Anticholinergic drugs: ◦ For tremors ◦ Natural and Synthetic ◦ Used with caution by slow titration to avoid SE eg confusion, retention of urine and acute glaucoma. Medical: Levodopa carbidopa ◦ For rigidity and bradykinesia (sinemet) ◦ Less SE, better efficacy ◦ SE: nausea, confusion, chorea Dopamine Agonists: ◦ Bromocriptine and pramipexole Amantadine ◦ Prevents uptake of dopamine by neurons. Hydrochloride: ◦ Pallidectomy, Surgical: ◦ Thalamotomy, ◦ Deep brain stimulation; DBS 49 Neurology Chorea  Def. ◦ Involuntary sudden irregular pseudopurposive movements of any part of the body. ◦ Caudate lesion.  Causes: ✓ Herido-familial: Huntington ✓ 2ry:  Autoimmune : e.g. Rheumatic chorea  Vascular : hemiballismus  Toxic : drugs  Infective : post-encephalitic ✓ Senile chorea Rheumatic chorea  Age: 5-15 years  Sex: females more than males  C/P:  Choreic movements  tongue, face, trunk and extremities.  Milkmaid sign.  Hypotonia  scaphoid-shaped hand.  Pronator drift sign.  Pendular knee jerk.  Emotional lability  Sudden laughter or crying  Clinical varieties 1.Usual type 4.Chorea mollis 2.Chorea gravis 5.Maniacal chorea 3.Hemichorea  Treatment ◦ Bed rest ◦ Steroids ◦ Haloperidol ◦ Tranquilizers 50  Neurology Chorea gravidarum ✓ Primi ✓ 4 th to 5th months ✓ Due to toxemia or stress of pregnancy. Hemiballismus ✓ Unilateral severe violent movement of large amplitude ✓ Due to subthalamic lesion (vascular or MS) Huntington chorea ✓ AD ✓ Middle age, gradual progressive ✓ Caudate nucleus lesion ✓ Triad of chorea, dementia and psychiatric signs. ✓ Genetic counseling is important 51 Neurology Athetosis  Irregular, static, slow, and snake-like movements  Extremities and face  Hypertonia  Causes: ◦ Congenital: Kernicterus, hypoxic brain damage ◦ Acquired : e.g. Post-encephalitic  Treatment: Antichlinergic Dystonia  It’s an involuntary slow twisting-like or torsion-like movement  It involves neck, trunk or proximal muscles of extremities.  Dystonia may be ◦ Generalized: has a genetic basis and starts in childhood in most cases. ◦ Focal:  Blepharospasm  Oromandibular  Torticollis  Writer’s cramp  Treatment of dystonia:  Identification of the cause  Medical treatment:  Anticholinergic drugs  Baclofen  Clonazepam  Levodopa in certain cases  Botox injection 52  Neurology Tics  Involuntary repetitive stereotypic movements (eg. Shrugging of shoulders, Blinking).  It Can be stopped by the patient for a while but can’t be totally avoided  It responds to psychological management. Myoclonus  Irregular,  assymetrical  shock-like contraction of a single muscle or group of muscles.  Some cases are epileptic, while other cases are related to lesions in the cerebral hemispheres, brainstem, or spinal cord.  Causes: 1) Epileptic e.g. Juvenile myoclonic epilepsy. 2) Degenerative e.g. Alzheimer Disease. 3) Metabolic 4) Familial e.g. Lipid storage diseases. 5) Acquired: ✓ Hyponatriemia ✓ Hypocalcemia ✓ Hepatic, renal or hypoxic encephalopathy.  Treatment:  Treat the cause.  Clonazepam 53 Neurology CEREBRAL ISCHEMIC STROKE  Blood supply of the brain The blood reaches the brain through two systems of blood vessels: 1- The carotid system. 2- The vertebral system. A- The carotid system - Each internal carotid artery enters the cranial cavity through the carotid foramen and canal to the cavernous sinus. - It is divided into 2 main terminal branches: 1- The middle cerebral artery - It runs on the lateral surface of the cerebral hemispheres in the lateral sulcus to supply the lateral aspect of the anterior 3/5 of the cerebral hemisphere - It gives the following branches: 1- Capsular branch 2- Cortical branches to supply the dorsal half of internal capsule including : (Lenticulostriate artery). - Frontal branch: supplying the lower part of motor area (Face, UL, and trunk), and motor speech areas. - Parietal branch: supplying the lower part of the sensory area , the angular , supramarginal, the upper fibers of the optic radiation. - Temporal branches: supplying the auditory areas and the lower fibers of the optic radiation. 54  Neurology 2- The anterior cerebral artery - It runs medially on the medial surface of the cerebral hemisphere then around the corpus callosum as far as parieto-occipital sulcus. - It supplies the medial aspect of the anterior 3/5 of the cerebral hemisphere and the upper edge of lateral surface. - It gives the following branches : 1 - Capsular branch 2- Cortical branches (Heubner artery) : involving the : supplying the anterior half of the anterior - Frontal branch: supplying the prefrontal limb of internal capsule. area. - Paracentral branch: Supplying the motor and sensory areas of the lower limb and paracentral lobule (cortical bladder center) - Callosal branch: supplying c the corpus callosum. B- The vertebral system : ✓ Each vertebral artery passes upwards through the vertebral foramina to enter the cranial cavity through the foramen Magnum and runs upwards on each side of the medulla. ✓ Both arteries meet at the lower border of the pons to form midline single artery; the basilar artery. ✓ The basilar artery runs upwards on the ventral surface of the pons where it gives small branches known as paramedian arteries to the brain stem and then divides into two terminal branches ; the posterior cerebral arteries. ✓ The posterior cerebral artery supplies the whole occipital lobe and the posterior part of the temporal lobe posterior 2/5 of the cerebral hemispheres. ✓ It gives the following branches: 1) Posterior communicating artery 2) Thalamo-geniculate artery: to supply the ventral half of the posterior limb of internal capsule, the thalamus, and Each vertebral artery gives 3 geniculate body. cerebellar branches on its side 3) Cortical branches to the occipital lobe. 55 Neurology  Physiology of Cerebral Circulation The brain receives about 15% of the total cardiac output, although it represents only about 2% of the total body weight. Its metabolism is a very high one, and derived from the oxidation of glucose. Since the brain has no store for oxygen or glucose, an adequate blood flow is essential for its function. The normal cerebral blood flow (C.B.F) is about 55 ml /100 gm brain tissue/minute. CEREBRAL STROKE  Definition : Cerebral stroke is: Rapidly developing Clinical symptoms and/or signs of focal loss of brain function Lasting more than 24 hours or leading to death with no apparent cause other than that of vascular origin.  Epidemiology The third most common cause of death after coronary heart disease andcancer. The most common life-threatening neurological disease. Incidence : 100 – 300 / 100 000 populations. Age : About 75% of cases are above 65 years old. Sex : males are slightly more, but in young or very elderly, both sexes are equal. Types of stroke A- Ischemia stroke (cerebral infarction). About 80% of all strokes: Due to inadequate blood supply to a part of the brain. B- Hemorrhagic stroke (intracerebral or subarachnoid) About 20% of all strokes: Due to spontaneous hemorrhage into or over the brain. 56  Neurology  Risk Factors: About 80% of stroke patients have at least one vascular risk factor. A- Non-modifiable risk factors B- Modifiable risk factors C- Modifiable risk by medical treatment factors by changing lifestyle Age: Old age is the most Hypertension: Cigarette smoking. important non-modifiable risk (The most important Hyperlipidemia. factor. modifiable risk factor.) Physical inactivity. Sex : Male. Diabetes mellitus. Obesity. Race : African-American race. Heart disease. Stressful life. Past history of stroke. Transient ischemic attacks. Positive family history of stroke. Raised hematocrit.  Causes of stroke 1- Large artery athero-embolism (45%): o Extracranial (40%) e.g. aorta, carotid or vertebral arteries. o Intracranial (5%) e.g. ICA, MCA, ACA, VA, BA or PCA. A- Arterial disease (75%) 2- Small artery disease (25%) : e.g. Microatheroma, Lipohyalinosis. 3- Other arteriopathies (5%): e.g. Dissection, Arteritis. Embolism from the heart e.g. with: - Atrial fibrillation (AF). - Mitral stenosis. - SBE - Atrial myxoma. B- Cardioembolic causes (20%) - Prosthetic valves. - Mitral valve prolapses. - Congenital heart disease. - Cardiomyopathy. Causing a prothrombotic state e.g. Polycythemia. C- Hematological disorders (5%) Essential thrombocythemia. Leukemia. Sickle cell disease. Hypercoagulability states etc. 57 Neurology  Clinical Picture  A- Focal loss of neurological function: These symptoms and signs are determined by the site and extent of the brain damage caused by the artery involved: In the carotid system: Unilateral signs predominate e.g. hemiplegia, hemi- hypoesthesia, hemianopia and aphasia (if the left hemisphere is involved). In the vertebro-basilar system: hemiplegia may also be found, but more commonly there are bilateral signs, motor and sensory in combination with disturbance of cranial nerves and cerebellum. N.B: The commonest involved artery is the middle cerebral artery and its central branches which supply the internal capsule. The resulting disability is hemiplegia due to destruction of the pyramidal fibers. It might be associated with hemianaesthesia and hemianopia.  B- Negative in quality. The focal neurological symptoms and signs are negative in quality (i.e. loss of function) rather than positive e.g : Muscle paralysis rather than convulsions, blindness rather than visual  C- Rapidly developing. The onset of the focal neurological symptoms and/or signs is sudden or acute.  D- Maximal at onset. 58  Neurology  Investigations  A- Radiological 1- An urgent brain CT scan: a - To exclude non-vascular causes of the suspected stroke e.g. tumor or subdural hematoma. b- To differentiate cerebral infarction (hypodense-blackish lesion) from intracerebral hemorrhage (hyperdense-whitish lesion) c - To suspect the likely cause of the ischemic stroke (from the site and size of the infarction). CT-brain after 48 hours of onset: 1- Low density. 2- Homogenous. 3- Has cortical base. 4- It lies in the anatomical distribution of cerebral blood vessel. 5- non-enhancing lesion CT-Brain may be normal in the following conditions: a. Immediate after onset (1st 48 hours), while weighted-image MRI- brain shows the infarction b. Very small infarction (< ½ cm). c. Infarction in the posterior fossa. 2- Other Duplex carotid ultrasound: When a carotid ischemic event is suspected. Echocardiography: When a cardio-embolic source is suspected. More specialized investigations: When the cause of stroke remains uncertain e.g.: MRI and MRA of the brain, angiography etc.  B- Laboratory Investigations Full blood count and ESR. PT, INR and PTT. blood glucose. Lipid profile Renal and liver function tests. 59 Neurology  Prognosis Early predictors of stroke outcome: - Level of consciousness (The best single predictor). Extent of brain lesion (e.g site, size by CT or MRI). Age of the patient. Premorbid state. Death: - In 1st month (ischemic: 10%, hemorrhagic: 50%) total → 20%. ✓ Causes of death: - 1- In the first few days (Due to the brain lesion itself): either - Large infarction → brain edema → midline shift or herniation. - Direct disruption of vital centers in the brain stem. 2- After the 1st week: - - Due to indirect complications; mainly bronchopneumonia, pulmonary embolism or coexistent heart disease. 3- After one month: - - Recurrent stroke or associated heart disease.  Treatment Patients with an acute stroke should be admitted to hospital for emergency evaluation and treatment preferably in a stroke unit. Modern therapy for ischemic stroke includes: A- Acute treatment: to reduce morbidity and mortality. B- Rehabilitation: to reduce disability and dependence. C- Prevention: to reduce stroke recurrence. Acute Treatment A- Thrombolytic therapy B- Care of respiration C- Care of circulation D- Prevention of complications 60  Neurology A- Thrombolytic therapy:  1- Thrombolytic drugs To recanalize the occluded artery and re-perfuse the ischemic brain tissue. Dose: 0.9 mg/kg recombinant tissue plasminogen activator (actylase) is given I.V. over 60 minutes. Indicated for patients with all of the followings: 1-Acute ischemic stroke with clearly detected time of onset. 2- Within 3 hours from onset of symptoms. 3- A baseline CT excluding ICH and SAH.  2- Intra-Arterial Mechanical Thrombectomy: - Thrombectomy (ie. retrieval of the occluding thrombus via a catheter) devices can be useful in achieving recanalization alone or in combination with pharmacological fibrinolysis in carefully selected patients. - Stent retrievers are better than coil retrievers. Indications: proximal large artery occlusion in anterior cerebral circulation. B- Care of respiration: ✓ To protect against airway obstruction, hypoventilation and aspiration. Supplemental oxygen and ventilator assistance may be needed. A feeding tube is placed if there is evidence oropharyngeal dysfunction. Suction of nasal and pharyngeal secretions may be needed. Endotracheal intubation may be needed. 61 Neurology C- Care of circulation: Cardiac monitoring is recommended in the first 24-48 hours to guard against myocardial ischemia and cardiac arrhythmias. Moderate elevation of BP with stroke onset is often reactive and spontaneously falls. If systolic BP was >220 mmHg or diastolic BP was >120 mmHg, urgent but slow lowering of BP is indicated. Rapid lowering of BP and hypotension will decrease cerebral blood flow leading to reduction of cerebral perfusion and worsening of outcome. D- Prevention of complications 1) Brain edema: Mannitol 20% solution 0.25 – I gm / kg. 2) Pneumonia: Prevention of aspiration and chest exercise. 3) Seizures: Anticonvulsant medications. 4) D.V.T:Low doses of unfractionated or low molecular weight heparin. 5) Pressure sores: Skin cleaning, frequent positional changes, and special air mattress. 6) Urinary tract infection : Indwelling catheters are used only when absolutely indicated and for the shortest possible time. 7) Electrolyte disturbances and nutritional deficiency: Adequate I.V. fluids, tube feeding. 8) Depression: Antidepressants. 62  Neurology Rehabilitation It should start as soon as the diagnosis of stroke is established and includes: Physiotherapy. Speech therapy. Occupational therapy. Prevention All patients who have survived should receive secondary prevention which includes:  A- Aggressive control of risk factors: e.g. - Treatment of hypertension - Control of diabetes. - Lipid lowering. - Avoid smoking.  B- Antiplatelet therapy: For thromboembolic ischemic strokes. e.g. Aspirin 75-150 nig daily, clopidogrel 75 mg daily.  C- Anticoagulation; For cardio-embolic ischemic strokes. - Heparin is given I.V. and maintained for some days while oral anticoagulant therapy (warfarin) is instituted. - The oral anticoagulant may be continued for 6 months. The dose should be controlled by the international normalized value (INR) (2-3 times).  D- Carotid endarterectomy: For patients with severe carotid stenosis (>70%). 63  Neurology CEREBRAL TRANSIENT ISCHEMIC ATTACK  Definition ✓ An acute focal loss of brain function with symptoms lasting less than 24 hours due to inadequate cerebral blood supply. ✓ Most TIAs last less than 15 minutes, but occasionally as long as 24 hours (followed by clinical complete recovery). ✓ Episodes that last longer than 1 hour will usually continue to be stroke. ✓ TIA is an important predictor of stroke risk. ✓ Two cerebral TIAS in 24 hours are considered a medical emergency  Pathogenesis TIAs usually occur due to either: Sudden and temporary thrombotic or embolic occlusion of a cerebral artery. Low flow distal to an already occluded or highly stenosed artery. So, any cause of ischemic stroke if less severe and temporary may cause a TIA.  Clinical Picture The symptoms differ according to whether the carotid or vertebrobasilar system is involved Ipsilateral transient visual loss, contralateral motor or sensory Carotid TIAs dysfunction, aphasia. Vertebrobasilar TIAs Alternating hemiparesis or hemisensory symptoms, dysartheria , dysphagia, diplopia, vertigo, homonymous hemianopia.  Investigations  Duplex carotid : (when a carotid ischemic event is suspected).  Echocardiography (when a cardioembolic event is suspected).  Transcranial Doppler : (to study intracranial vessels).  Cerebral angiography : (to detect occlusions or stenosis of cerebral vessels).  CT or MRI of the brain : (to detect small infarcts)  Treatment  Anticoagulants: I.V. as heparin, or S.C. as low molecular weight heparin or oral as warfarin.  Antiplatelets: as aspirin or clopidogrel.  Endarterectomy and angioplasty: for severe carotid stenosis.  Control of risk factors. 64  Neurology HYPERTENSIVE ENCEPHALOPATHY  Pathophysiology  This is an acute commonly reversible disorder of cerebral function due to severe or malignant hypertension (diastolic blood pressure above 140 mm hg).  Normally rise in blood pressure causes reactive vasoconstriction of the cerebral arteries to protect the brain against flooding with blood.  In hypertensive encephalopathy, the failure of this autoregulation leading to severe cerebral vasodilatation and the clinical manifestations of encephlopathy.  Clinical pictures 1) Manifestaions of increased intracranial tension as headache, vomiting, blurring of vision, confusion, and maybe coma and convulsions. 2) Focal manifestations: as aphasia, hemiparesis, fundus exudates, bilateral papilledema, and hypertensive retinopathy  Prognosis ✓ The condition is reversible as usually there is no infarction.  Treatment - Control of hypertension - Symptomatic treatment e.g anticonvulsant drugs in cases of convulsions. 65 Neurology Vascular Occlusive Syndromes I- Internal carotid artery (ICA) occlusion  Onset: it may be preceded by recurrent transient attacks of one or more of the following: 1) Headache. 2) Blindness (Amaurosis Fugax). 3) Hemiparesis or hemihyposthesia. 4) Aphasia. 5) Convulsions. 6) Mentality changes.  These symptoms : indicate ICA insufficiency (Carotid TIAs) which may terminate in complete occlusion of the artery manifested by: 1) Ipsilateral blindness. 2) Contralateral hemiplegia. 3) Contralateral hemihyposthesia. 4) Contralateral homonymous hemianopia. 5) Aphasia with or without agraphia in left-sided lesions. 6) The internal carotid pulse may be diminished or lost & an audible bruit. over the vessel may be heard. 66  Neurology II- Middle cerebral artery (MCA) occlusion (The most commonly affected vessel)  A- Main Artery Occlusion: 1) Coma at the onset. 2) Contralateral hemiplegia affecting UL more than LL. 3) Contralateral hemihyposthesia with cortical sensory loss in UL. 4) Contralateral homonymous hemianopia (without macular sparing). 5) Aphasia & agraphia in left sided lesions.  B- Capsular branch occlusion (Lenticulostriate artery) 1) Contralateral complete hemiplegia affecting the upper & lower limbs to the same extent. 2) Contralateral hemihyposthesia of subcortical type. 3) Contralateral hemianopia may occur. 4) No loss of consciousness or aphasia.  C- Cortical branches occlusion a) Frontal vessel occlusion 1) Facio-brachial monoplegia 2) Motor aphasia and agraphia in left sided lesions. b) Parietal Vessel Occlusion: 1) Cortical sensory loss in the upper limb. 2) Lower quadrantic homonymous hemianopia. 3) Sensory aphasia (alexia) & apraxia in left sided lesions. c) Temporal Vessel Occlusion: 1) Upper quadrantic homonymous hemianopia. 2) Sensory aphasia (Auditory Agnosia). 67 Neurology III- Anterior Cerebral Artery (ACA) Occlusion  A- Main Artery Occlusion: 1) Contralateral hemiparesis affecting LL more than UL. 2) Contralateral cortical sensory loss in the LL. 3) Urine incontinence. 4) Mentality & personality changes. 5) Forced grasp reflex.  B- Capsular Branch Occlusion (Heubner artery) - Facio-brachial monoparesis which is proximal more than distal, i.e. involving the shoulder more than the hand.  C- Cortical Occlusion: a) Frontal vessel occlusion: -Mental and personality changes - Forced grasp reflex b) Paracentral vessel occlusion: - Monoplegia in the lower limb. - Cortical sensory loss in the lower limb. - Urinary incontinence. C) Callosal vessel occlusion: Apraxia of the left side. 68  Neurology IV- Posterior Cerebral Artery Occlusion  A- Main Vessel Occlusion: 1) Contralateral homonymous hemianopia with macular sparing as the macula ha double blood supply. 2) Visual agnosia in left sided lesions. 3) Thalamic Syndrome; in cases where the occlusion is proximal to the origin of the thalamogeniculate artery.  B- Ganglionic branch (Thalamogeniculate artery) oclusion (Thalamic syndrome) 1) Thalamic Pain i.e., constant burning severe pain in the hemianaesthetic side. 2) Complete contralateral hemi-anaesthesia to all types of sensations with partial recovery of superficial sensations; however, vibration sense is permanently lost. 3) Reflex dystrophy of the shoulder girdle & arm, secondary to the pain. 4) Choreo-athetosis or hemiballismus due to ischemia of the basal ganglia and/or subthalamus.  C- Cortical branches occlusion 1) Contralateral homonymous hemianopia with macular sparing. 2) Visual agnosia in left sided lesions. 69 Neurology V- Vertebro- basilar artery occlusion  A- Main vessel occlusion a) Partial occlusion (insufficiency): manifested by recurrent transient attacks of the following (Vertebro-Basilar TIAs). 1) Syncope. 2) Diplopia. 3) Ophthalmoplegia. 4) Vertigo or tinnitus. 5) Bulbar symptoms (dysphagia, dysarthria, nasal regurgitation, & hoarseness of voice). 6) Hemiparesis, hemi anaesthesia or paresthesias. 7) Ataxia. b) Complete occlusion (usually fatal): - Deep coma. - Complete quadriplegia with decerebrate rigidity. - Bulbar paralysis. - Respiratory embarrassment.  b- Cerebellar arteries occlusion i. Superior cerebellar artery occlusion: 1-Ipsilateral cerebellar ataxia 2- Ipsilateral Horner's syndrome. 3- Ipsilateral deafness. 4- Contralateral hemihyposthesia. ii. Anterior inferior cerebellar artery 1) Ipsilateral cerebellar ataxia. 2) Ipsilateral Horner's syndrome 3) Ipsilateral deafness. 4) Ipsilateral 5th, 6th & 7th cranial nerve paralysis. 5) Contralateral hemihyposthesia 70  Neurology iii. Posterior inferior cerebellar artery ✓ This artery arises as a branch of the vertebral artery but may rarely originate from the proximal part of the basilar artery. ✓ It runs upwards on the lateral aspect of the medulla supplying its upper lateral part which contains: 1) Some of the reticular formation nuclei. 2) Part of the vestibular nucleus. 3) Spinal sensory nucleus of the 5th CN for pain & temperature sensations of the same side of the face. 4) Sympathetic fibers to the eye & face. 5) The nuclei of 9th, 10th & 11th CNs. 6) Lateral lemniscus carrying pain & temperature sensations from the opposite side of the body. ✓ The artery finally terminates & supplies the inferior part of the cerebellum. ✓ Occlusion of the artery will lead to Wallenberg Syndrome (Lateral medullary syndrome) 1) Acute onset associated with syncope, hiccup, vomiting, vertigo & pain over the face. 2) Ipsilateral cerebellar ataxia (nystagmus, dysarthria, incoordination). 3) Ipsilateral Horner's syndrome. 4) Ipsilateral palato-pharyngeo-laryngeal paralysis & weakness of the sternomastoid & trapezius. 5) Ipsilateral loss of pain & temperature sensations over the face. 6) Contralateral loss of pain and temperature sensations over the body.  C- Brain stem (Paramedian) Branches Occlusion: 1) Mid- brain: - Weber's syndrome. - Benedict's syndrome - Claud Syndrome (midbrain & cerebro-thalamic connections lesion): Ipsilateral III nerve palsy with contralateral ataxia (cerebro-thalamic connections lesion). 2) Pons: - Millard-Gubler's syndrome. - Foville's syndrome. 3) Medulla: - Avellis Syndrome. - Jackson's Syndrome 71 Neurology  Causes 1) Hypertension. 2) Vascular malformation as aneurysms or AVM. 3) Bleeding disorders (thrombocytopenic purpura, liver disease, complication of anti-coagulant etc.). 4) Hemorrhagic infarction. 5) Trauma. 6) Cerebral arterial amyloidosis.  Pathophysiology ✓ The commonest cause of spontaneous intracerebral hemorrhage (ICH) is due to chronic hypertension. ✓ The extravasated blood forms a mass (hematoma) that disrupts and compresses the adjacent brain tissue. ✓ If the hemorrhage is large: 1- The midline structures are displaced to the opposite side. 2- Compression on the reticular activating system and vital centers in brainstem, leading to coma and even death.