Approach to Paraplegia PDF

Approach to Paraplegia Tadele Mesfin (M.D.) AWHSC, DBU December 2024 Outline Definition Basic Anatomy Etiologies and Classification Approach to Patient Common causes of paraplegia Management Principle Complications  Objectives At the end of this clas...

Approach to Paraplegia Tadele Mesfin (M.D.) AWHSC, DBU December 2024 Outline Definition Basic Anatomy Etiologies and Classification Approach to Patient Common causes of paraplegia Management Principle Complications  Objectives At the end of this class, you are expected to know about; Definition of Paraplegia Basic Anatomy of the spinal cord Common Causes of paraplegia Differentiate UMN lesion from LMN lesions How to approach a patient with paraplegia Management Principles  Definition Diseases of the spinal cord are frequently devastating. even a small area lesion may compromise the entire motor output and sensory input of the trunk and limbs. Among the most critical of neurologic emergencies. But may be reversible if recognized and treated early. Para- impairment in motor function of the lower extremities With or with out involvement of sensory system Paraplegia- Complete Paralysis Paraparesis- Partial paralysis Basic Anatomy  Causes Pathologies that cause paraplegia are diverse For better understanding causes can be grouped based on 1, site UMN vs LMN Cerebral vs Spinal 2, Pathophysiology Immuno-allergic, Inflammatory, Demyelinating, Heredofamilial, Toxic Myelopathy, Vascular, Metabolic, Nutritional, Tropical, Paraneoplastic syndromes, Physical agents  Paraplegia UMNLesion LMN Lesion Cerebral Spinal lesions Lesions Non- compressive compressive Extradural Intradural Intramedullary Systemic tract vascular Infectious Degeneration  Compressive SC disorders Extradural Intradural Intramedullary Pott’s Disease Tumors, e.g. Tumors, e.g. Cervical Spondylosis Meningioma, Ependymoma, Epidural Abscess Neurofibroma glioma Malignancy, e.g. Arachidonitis Syringomyelia, Metastasis haematomyeli to vertebrae, a Multiple Myeloma Spinal cord Fluorosis abscess, e.g. Fracture dislocation pyogenic, TB of vertebrae, Paget's disease, Osteoporosis  Non Compressive Vascular Infection Systemic tract degeneration Spinal ATM (post- Toxic, Metabolic infectious) epidural SCD Vit B12 deficiency, Nitrous hematoma Oxide abuse Syphilis Copper deficiency Tubercular myelitis myeloneuropathy Spinal cord Radiation myeloneuropathy Infarction AIDS myelopathy Keraunoparalysis (Electrical Injury) Hepatic myelopathy Tropical Spastic Lathyrism and Konzo Vascular Paraplegia/ HTLV-1 Others malformation Associated Multiple sclerosis Myelopathy e.g. Dural AV ALS fistula Parasites, e.g. Sarcoidosis Schistosomiasis Syringomyelia Hereditary Spastic Paraplegia   Cont’d Cerebral Lesions Rare causes Traumatic Vascular Inflammatory Neoplastic Depressed fracture Superior sagittal Parasagittal of the vault of the Encephalitis sinus thrombosis meningioma skull Thrombosis of Subdural Meningo- unpaired Anterior Hematoma encephalitis Cerebral Artery  Flaccid Paraplegia- LMN causes Anterior Peripheral Neuromuscu Nerve Root Muscles Horn Cells Neuron lar Junction Muscular Peripheral Myasthenia Dystrophi Poliomyelitis Radiculitis neuropathy Gravis es Motor Guillain– Lambert- Polyradiculopa Neuron Barré Eaton thy Disease syndrome syndrome Spinal Periodic paralysis Muscular Tabes Dorsalis (hypo/hyperkalae Atrophy mia) Cauda Equina Classification Upper motor neuron Vs Lower Motor Neuron Lesion  Approach to Patient History Ask about the time, date, and mode of onset, the extent, distribution, and progression of symptoms Motor symptoms including inability to walk or stand (with or without support), difficulty in walking. Sensory symptoms, e.g., loss of sensation or reduction in sensations, radicular pain, sensation of pins and needles, numbness, history of a constriction band of hyperalgesia around the trunk, local pain in the vertebral column  Cont’d Autonomic symptoms, e.g., bowel - bladder dysfunction History of prolonged fever, back pain, or any deformity History of trauma to the head or spine Nutritional and Vaccination history Other associated symptoms, recurrence Comorbid illness (TB, STD, Malignancy, DM, HTN)  Physical Examination Consciousness, posture Mental status – GCS Cranial nerves Vital signs Motor system Pallor Position, Bulk, fasciculations, tone, LGS- power, reflexes, clonus, Lymph node enlargements, Gait (if the patient could walk) Thyromegaly Sensory system, Respiratory system for tuberculosis, Important in identifying sensory bronchogenic carcinoma, and lymphoma level Pain, touch, temperature, Cardiovascular system vibration perception GI tract for hepatosplenomegaly, ascites Sweat level, Neurocutaneous markers, e.g. café-au-lait vibration level spots, palpable neurofibromas Meningeal signs Spine Kyphoscoliosis Gibbus or other deformities Local tenderness Nervous system  localize the segmental level of the lesion The presence of a horizontally defined level below which sensory, motor, and autonomic function is impaired is a hallmark of a lesion of the spinal cord. Upper border of sensory level Level of band-like/girdle-like sensation Localized vertebral tenderness/deformity (if any) Abdominal reflex - Beevor’s sign Investigations Depend on the possible likely cause 1, Laboratory Investigations CBC, Blood culture, CSF analysis, PCR, Gene xpert, PITC, Viral markers, VDRL, Indian INK, Fungal culture, oligoclonal bands, Autoantibodies 2, Imaging tests Spine MRI Chest radiograph  Management Principles 1, General principles Frequent change of posture to guard against bedsores Care of skin Care for bladder Frequent change of bed-sheets Physiotherapy DVT prophylaxis  Cont’d Symptomatic Treatment 3, Rehabilitation Analgesics and Sedatives Management of complication Muscle relaxants Occupational Therapy Vitamins and mineral Gait retaining supplementation 2, Specific Treatment ATT + Supportive measures in Pott’s Drainage of paraspinal abscess Traumatic spine stabilization Surgical management of some tumors Complication Bed Sores Contractures Urinary tract infection Pneumonia Deep venous thrombosis TB Spondylitis TB of the bones and joints is responsible for ∼10% of extrapulmonary cases In bone and joint disease, pathogenesis is related to reactivation of hematogenous foci or to spread from adjacent paravertebral lymph nodes. Weightbearing joints (the spine in 40% of cases, the hips in 13%, and the knees in 10%) are most commonly affected. Spinal TB (Pott’s disease or tuberculous spondylitis) often involves two or more adjacent vertebral bodies.  Pathogenesis Infection generally begins with inflammation of the anterior aspect of the intervertebral joints; typically, it spreads behind the anterior ligament to involve the adjacent vertebral body. Once two adjacent vertebrae are involved, infection enters the adjoining intervertebral disc space. This tends to occur later in Pott's disease than in bacterial vertebral osteomyelitis and may have the radiographic appearance of relative disc sparing. Eventually, the avascular disc tissue dies; there is vertebral narrowing and subsequent vertebral collapse. Gibbus deformity, a form of structural kyphosis, distorts spinal canal anatomy.  Cont’d Whereas the upper thoracic spine is the most common site of spinal TB in children, the lower thoracic and upper lumbar vertebrae are usually affected in adults. The spinal cord is then at risk of compression, resulting in paraplegia. Occasionally, late-onset paraplegia occurs due to osteophytes and other chronic degenerative changes at a site of prior infection. Formation of a "cold abscess" (soft tissue mass) at the site is common. Noncontiguous spinal disease (eg, disease at more than one level) is uncommon, although in one South African series it was described in 16 of 98 cases  Clinical Feature The most common symptom is local pain, which increases in severity over weeks to months, sometimes in association with muscle spasm and rigidity. The muscle spasm can extend beyond the diseased area. In some cases, a characteristic erect posture and "aldermanic" gait may be observed in which the patient walks with short, deliberate steps to avoid jarring of the spine Constitutional symptoms such as fever and weight loss are present in less than 40 percent of cases.  Diagnosis In endemic areas, the clinical presentation also tends to be relatively late due to limited access to medical care; in these settings, patients have symptoms and signs of cord compression at the time of diagnosis in 40 to 70 percent of cases. Thus, late diagnosis is a major factor in determining the outcome of the disease The diagnosis of Pott’s disease is frequently delayed as a result of its subacute course, especially in regions where the incidence of tuberculosis is relatively low.  Diagnosis CT or MRI reveals the characteristic lesion and suggests its etiology. Aspiration of the abscess or bone biopsy confirms the tuberculous etiology, as cultures are usually positive and histologic findings highly typical. The differential diagnosis includes tumors and other infections. Pyogenic bacterial osteomyelitis, in particular, involves the disk very early and produces rapid sclerosis.  Management Supportive Anti TB - 2RHZE/10RH Surgery — may include decompression, use of hardware for stabilization of spine, abscess drainage, and/or debridement of infected material Indications: 1.Patients with advanced neurological deficits 2.Patients with worsening neurological deficits progressing while on appropriate therapy 3.Patients with kyphosis >40 degrees at the time of presentation  Lathyrism and konzo Two disorders of spastic paraparesis have been described, which occur in association with increased dietary intake of food plants with neurotoxic potential, as occurs in certain geographic regions. Neurolathyrism is associated with prolonged consumption of the grass or chickling pea (Lathyrus sativus.) Konzo is linked to high exposure to cyanogenic compounds in diets containing insufficiently processed bitter cassava (Manihot esculenta)  Cont’d Exposed persons develop a slowly developing spastic paraparesis with cramps, paresthesias, and numbness, accompanied by bladder symptoms and impotence. Some patients have tremors and other involuntary movements in their arms. Pathologic studies have demonstrated a loss of myelinated fibers in the corticospinal and spinocerebellar tracts. The toxin appears to be the neuroexcitatory amino acid, beta-N-oxalylaminoalanine.  Cont’d The disease is usually seen in times of Famine No specific Investigation. Diagnosis is based on clinical features and exclusion of other etiologies Management is mainly supportive. Processing food prior to consumption has been shown to decrease the neurotoxic effects. Reading Assignment Guillain–Barré syndrome Transverse Myelitis Epidural Abscess Questions?? Comments? Approach to patient with Coma Tadele Mesfin (M.D.) AWHSC, DBU December 2024 Outline Definition of terms  Pathophysiology of coma  Approach to comatose patients Basic Management Principle  Clinical conditions mimicking coma Objectives By the end of this lecture students should Be able to define coma. Understand the pathophysiology and list causes of coma. Understand basic principles on how to approach comatose patient. Definition Consciousness consists of awareness of self and environment Arousal refers to the physiological state of wakefulness Coma is defined as "unarousable unresponsiveness" Comatose patient is unarousable and unable to respond to external events or inner needs. Coma is among the most common neurologic emergencies encountered general medicine. Pathophysiology Arousal requires the stimulation of the caudal brainstem reticular activating system(RAS) to the cerebral cortices. The cortex controls sensory processing and understanding, which generates awareness Coma results from an impairment of this axis by a process that affects the brain’s arousal center, consciousness center and the tracts that connect them Cont’d Lesions in the brain that are the leading causes of comatose conditions can be divided into three major types of pathology. 1. Widespread abnormalities of the cerebral hemispheres. 2. Reduced activity of the thalamocortical alerting system, the reticular activating system (RAS) 3. Suppression of reticulocerebral function commonly occurs by drugs, toxins, or metabolic derangements. these types of metabolic causes of coma are far more common than structural injuries. Causes The causes of coma can be divided into three broad categories: 1. Those without focal neurologic signs (e.g., metabolic and toxic encephalopathies); 2. Those with prominent focal signs (e.g., stroke, cerebral hemorrhage); and 3. Meningitis syndromes, characterized by fever or stiff neck and an excess of Cont’d Coma Mimics Locked-in syndrome Akinetic mutism Vegetative state Minimally conscious state Abulia Catatonic Severe GBS pharmacologic neuromuscular blockade, critical ill neuropathy Approach to patients with coma Initial assessment The diagnostic workup of the comatose patient must proceed concomitantly with management. It is mandatory to quickly confirm that the patient’s airway is clear. If respiration becomes shallow or struggling or there is threat of aspiration with emesis, tracheal intubation should be immediately performed. Cont’d Concurrently with airway management, the cervical spine must be stabilized. Oxygen saturation must be kept above 94 percent IV access is established. If hypotension occurs, intravenous fluids with or without vasopressor agents must be initiated. Blood drawn for CBC, serum glucose, electrolyte, arterial blood gases; liver biochemical and kidney function tests; and toxicology studies. Cont’d Empiric treatment of reversible causes. Thiamine (100 mg), followed by dextrose 50% (25 g), and naloxone (0.4–1.2 mg) should be given for indicated patients intravenously without delay. Antibiotics including at least vancomycin and a third- generation cephalosporin are typically administered rapidly along with dexamethasone for case of Acute bacterial meningitis History The cause of coma may be immediately evident as in cases of trauma, cardiac arrest, or observed drug ingestion. In the remainder, certain points are useful: 1. The circumstances and rapidity with which neurologic symptoms developed; 2. Antecedent symptoms (confusion, weakness, headache, fever, seizures, dizziness, double vision, or vomiting); 3. The use of medications, drugs, or alcohol; and 4. Chronic liver, kidney, lung, heart, or other medical disease. Cont’d Physical examination General examination Checking vital signs is essential in determining the causes of unconsciousness. Evidence of trauma leads to suspicions of traumatic brain injury Signs of an acute or chronic systemic illness may be related to metabolic coma. Cont’d Neurological examination The initial goal of the neurological examination is to determine whether it is from a structural cause or a metabolic abnormality Level of consciousness GCS Brainstem Reflexes Assessment of brainstem function is essential to the localization of the lesion in coma. Patients with preserved brainstem reflexes typically have a bihemispheric localization to coma. whereas patients with abnormal brainstem reflexes either have a lesion in the brainstem or a herniation syndrome from a cerebral mass lesion impacting the brainstem secondarily.  Investigations Labs CBC, serum electrolytes( including calcium magnesium), LFT, RFT, RBS, ABG, Coagulation profile, CSF analysis, serum/urine toxicology, Urine HCG. Imaging Brain CT, MRI, Others EEG,ECG Management Coma Care ABCDE of life Nutrition and hydration Gastrointestinal management Genitourinary management Hygiene needs and skin care TREATMENT OF UNDERLYING CAUSE Thank you Question?  Headache Tadele Mesfin (M.D.) DBU, AWHSC December 2024  Outline Introduction Classification Pathophysiology of headache General approach to headache Primary headaches Objectives At the end of this session, students should be able to: Know the classification of headache Approach to patient with a headache Understand how to diagnose and manage primary headaches Introduction Headache is among the most common reasons patients seek medical attention. Diagnosis and management are based on a careful clinical approach Small number of patients present with life-threatening headaches. The overwhelming majority with benign primary headaches. Failure to recognize a serious headache can have potentially fatal consequences. Classification Headaches can either be primary/idiopathic (eg, migraine, cluster, tension type). or Secondary (resulting from underlying disease, such as tumor or intracranial hemorrhage). Cont’d Pathophysiology Pain usually occurs: when peripheral nociceptors are stimulated in response to tissue injury, visceral distension, or other factors. Or When pain-producing pathways of the peripheral or central nervous system (CNS) are damaged or activated inappropriately. Cont’d Few cranial structures are pain producing; these include: the scalp, meningeal arteries, dural sinuses, falx cereri, and proximal segments of the large pial arteries. The key structures involved in primary headache are the following: The large intracranial vessels and dura mater, and the peripheral terminals of the trigeminal nerve Cont’d The caudal portion of the trigeminal nucleus, which extends into the dorsal horns of the upper cervical spinal Rostral pain-processing regions, such as the ventroposteromedial thalamus and the cortex The pain-modulatory systems in the brain that modulate input from the trigeminal nociceptors, such as the hypothalamus and brainstem Evaluation of Acute, New-onset Headache A careful history and physical examination remain the most important part. In new-onset and severe headache, the probability of finding a potentially serious cause is considerably greater. Patients with recent onset of pain require prompt evaluation and appropriate treatment Warning Symptoms The following historical features are warning signs to the presence of a secondary headache. Sudden onset-That reaches maximal intensity within a few seconds or minutes after the onset of pain warrants aggressive investigation. e.g. SAH, Carotid and vertebral artery dissection, venous sinus thrombosis, angle closure glaucoma and hypertensive emergencies. Cont’d No similar headaches in the past — The absence of similar headaches in the past is another finding that suggests a serious disorder. The "first" or "worst headache of my life“. Altered mental status or seizure-Any change in mental status, personality, or fluctuation in the level of consciousness suggests a potentially serious abnormality. Age over 50— over 50 years of age with new onset or progressively worsening headache are at significantly greater risk of a dangerous cause of their symptom  Cont’d Concomitant infection — Infection in a non-intracranial location may serve as a nidus for the development of meningitis or intracranial abscess. HIV and immunosuppression — HIV and other immunosuppressed patients with headache are at significant risk for intracranial disease. Visual disturbances — Occasionally patients with significant ophthalmologic disease. Medications — should inquire about medication use, particularly anticoagulants, glucocorticoids, and analgesics, Cont’d Additional history—head trauma, toxic exposure, concomitant illness ( like , malignancy, CTDs) - Liver disease, Renal Disease, coagulopathy Illicit drug use  Warning Signs The following findings on physical examination may suggest a life- threatening cause of headache.  Neurologic abnormalities— The patient with any new focal or non-focal neurologic abnormality must be evaluated for serious illness.  single-best clinical predictor of intracranial pathology  Decreased level of consciousness — Obtundation and confusion increase the likelihood of meningitis, encephalitis, subarachnoid hemorrhage (SAH), or other space occupying lesion. Cont’d Meningismus — Meningismus may indicate meningitis or SAH. It can be subtle. Scalp tenderness- temporal arteritis Abnormal vital signs — Fever can reflect CNS infection. It can follow SAH by a few days.  severe hypertension (diastolic bp ≥120 mmHg) can manifest as headache Ophthalmologic findings — Papilledema is indicative of increased ICP. Cont’d LOW -RISK PATIENTS No substantial change in their typical headache pattern No new concerning historical features (eg, seizure, trauma, fever) No focal neurologic symptoms or abnormal neurologic examination findings No high-risk comorbidity Cont’d In high-risk patients' further evaluation is required: Neuroimaging and CSF evaluation Modality depends upon the most likely suspected cause Laboratory tests- CBC, electrolytes, RFT,LFT, coagulation profile, FBS, ESR Red Flags Primary Headaches Tension Type Headache Tension-type headache (TTH) is the most prevalent headache in the general population. Characterized by bilateral tight, band-like discomfort. The pain typically builds slowly, fluctuates in severity, and may persist more or less continuously for many days. Cont’d There are three main subtypes of TTH: Infrequent episodic TTH, with headache episodes less than one day a month Frequent episodic TTH, with headache episodes 1 to 14 days a month Chronic TTH, with headaches 15 or more days a month Pathophysiology The pathophysiology of TTH is incompletely understood. Primary disorder of central nervous system pain modulation peripheral activation or sensitization of myofascial nociceptors in episodic TTH. sensitization of pain pathways in the CNS due to prolonged nociceptive stimuli from pericranial myofascial tissues in chronic TTH Clinical Features The typical presentation of a TTH attack is that of a mild to moderate intensity, bilateral, non-throbbing headache without other associated features. ‘’Dull," "pressure," "head fullness", "head feels large," or, more descriptively, "like a tight cap", "band-like," or a "heavy weight on my head or shoulder Diagnostic criteria Require at least 10 episodes of headache, each lasting 30 minutes to Both of the following: seven days, and  No nausea or vomiting At least two of the following:  No more than one of photophobia or  Bilateral location phonophobia  Pressing or tightening (non- pulsating) quality  Mild or moderate intensity  Not aggravated by routine physical activity such as walking or climbing stair Treatment The pain of TTH can generally be managed with simple analgesics: -Acetaminophen, NSAIDs including ASA - Behavioral therapy Triptans in pure TTH are not helpful For chronic TTH, amitriptyline is the only proven treatment Migraine Headache It is a recurring syndrome of headache associated with other symptoms of neurologic dysfunction in varying admixtures It the second most common cause of headache, The most common headache-related cause of disability in the world Affects women more than men; often familial; onset usually by teens to early 20s, but peak age is 30 to 39 years. Cont’d Migraineurs are particularly sensitive to environmental and sensory stimuli Headache can be initiated or amplified by various triggers. Stress(80%) hormonal Weather(53%) fluctuations (menses) (65%) bright lights sounds Odors lack of or excess Hunger(57%) Neck pain sleep (50%) physical exertion alcohol or other chemicals Pathophysiology Dysfunction of monoaminergic sensory control systems located in the brainstem and hypothalamus Leads to sensitivity to sensory stimuli Activation of cells in the trigeminal nucleus results in the release of vasoactive neuropeptides, CGRPs Involve of the neurotransmitter 5-hydroxytryptamine (5-HT; also known as serotonin There is a role for dopamine in the pathophysiology of migraine Clinical features A migraine attack has three phases: The first phase of a migraine attack for most patients is the premonitory (prodromal) phase consisting of some or all of the following: yawning, tiredness, cognitive dysfunction, mood change, neck discomfort, polyuria, and food cravings Cont’d The headache phase follows: usually pulsatile, lasting 4–72 hours. Pain is typically, but not always, unilateral. Nausea, vomiting, photophobia, and phonophobia are common accompaniments. Pain is aggravated with routine physical activity. Postdrome phase, most commonly feeling tired/weary, unable to concentrating, and experiencing mild neck discomfort Cont’d About 20–25% of migraine patients have a fourth, aura, phase. visual disturbances with flashing lights or zigzag lines moving across the visual field or of other neurologic symptoms Cont’d Treatment Avoidance of known triggers Abortive therapy, including triptans, NSAIDs , alone or in combination IV metoclopramide can be added for abortive therapy Other treatments include ergots or CGRP antagonists (rimegepant, ubrogepant). Cont’d Prophylaxis for frequent or severe migraines, including anticonvulsants (eg, valproate, topiramate), tricyclic antidepressants ([TCAs], eg, amitriptyline), and β-blockers (propranolol; first-line prevention in pregnant patients). Chronic cases may benefit from injection of botulinum toxin. Routine aerobic exercise and good sleep hygiene  Trigeminal Autonomic Cephalalgias ( TACs) A grouping of primary headaches including cluster headache, paroxysmal hemicrania (PH), SUNCT/ SUNA , and hemicrania continua. TACs are characterized by relatively short-lasting attacks of head pain associated with lateralized cranial autonomic symptoms Pain is usually severe and may occur more than once a day. lacrimation, conjunctival injection, aural fullness, or nasal congestion Cluster Headache Cluster headache affects predominantly middle-aged men. Average age of onset is 25 years Patients who smoke have a higher risk of this type of headache The pathophysiology is unclear but may relate to activation of cells in the ipsilateral hypothalamus, triggering the trigeminal autonomic vascular system Clinical features Presents as a brief, excruciating, unilateral, periorbital headache that lasts from 30 minutes to 3 hours, during which time the patient tends to be extremely restless. Patients do not have auras (vs migraine headache). Tends to occur in clusters of time, affecting the same part of the head at the same time of day (commonly during sleep) during a certain season of the year. Cont’d Associated autonomic symptoms include ipsilateral lacrimation, conjunctival injection, Horner syndrome (ptosis, pupillary meiosis, and facial anhidrosis), and nasal stuffiness Treatment Acute therapy: High-flow O2 or sumatriptan injection Prophylactic therapy: Verapamil—first-line treatment, typically prescribed with prednisone (10-day course); alternatives include lithium, valproic acid, and topiramate References Harrison’s Principles of Internal medicine ,21st edition UpToDate 2024 Thank You Questions?  Seizure and Epilepsy Tadele Mesfin (M.D.) DBU, AWHSC December 2024. Outline  Definition  Classification  Seizure Mimics  Approach to Patient  Status epilepticus  References  Objectives Define seizure and epilepsy Understand the classification of seizure Know presenting features of seizure disorders Know how to approach a patient presented with seizure 109  Introduction Seizure Is a sudden behavior change caused by electrical hyper-synchronization of neuronal networks in the cerebral cortex. Can be provoked or unprovoked Epilepsy: Refers to a clinical phenomenon rather than a single disease entity that is defined as a disorder of the brain characterized by an enduring predisposition to epileptic seizures  ILAE …..2014 definition Epilepsy is a disease of the brain defined by any of the following conditions: At least two unprovoked (or reflex) seizures occurring >24hrs apart………..Why? One unprovoked (or reflex) seizure and a probability of further seizures similar to the general recurrence risk of 2 unprovoked seizures, occurring over the next ten years /at least 60%/ Diagnosis of an epilepsy syndrome Epidemiology ~5–10% of the population will have at least one seizure, with the highest incidence occurring in early childhood and late adulthood The incidence of epilepsy is ~0.3–0.5% in different populations throughout the world, and the prevalence of epilepsy has been estimated at 5–30 persons per 1000  CLASSIFICATION OF SEIZURES Why do we classify seizures? 1. For proper diagnostic approach 2. Identifying particular etiologies, triggers 3. Selecting the appropriate therapy, 4. Providing potentially vital information regarding the prognosis 5. To inform the risk of comorbidities and mortality  Cont’d The International League Against Epilepsy, is based on the; 1, Clinical features of seizures and 2, Associated electroencephalographic findings Etiology or cellular substrate are not considered in this classification system (for now) 116 Focal Onset Seizures Focal seizures arise from a neuronal network either discretely localized within one brain region or more broadly distributed but still within a cerebral hemisphere Classified based on Awareness (intact or impaired) and Nature of the onset (motor or nonmotor). Focal seizures can also evolve into generalized seizures.  Focal Seizures with Intact Awareness Focal seizures can have motor manifestations (such as tonic, clonic, or myoclonic movements) or nonmotor manifestations (such as sensory, autonomic, or emotional symptoms) without impairment of awareness. Three additional features of focal motor seizures are worth noting. Jacksonian march- represents the spread of seizure activity over a progressively larger region of motor cortex. Todd’s paralysis epilepsia partialis continua  Focal Seizures with Impaired Awareness The patient is unable to respond appropriately to visual or verbal commands during the seizure and has impaired recollection or awareness of the ictal phase  The seizures; 1. Frequently begin with an aura 2. Are usually accompanied by automatisms: 3. Postictal confusion ( seconds to hours) 4. Anterograde amnesia and transient postictal neurologic deficit following the seizure (caused by postictal inhibition of the cortical regions most involved in the seizure itself)  GENERALIZED ONSET SEIZURES Generalized seizures arise at some point in the brain but immediately and rapidly engage neuronal networks in both cerebral hemispheres. Absence Seizures (Typical vs Atypical) Generalized, Tonic-Clonic Seizures Atonic Seizures Myoclonic Seizures  Generalized Absence Seizures Typical absence seizures are characterized by a sudden blank stare with motor arrest, usually lasting less than 15 seconds. The individual is usually unresponsive and unaware. The seizure ends as abruptly as it starts, and the patient returns immediately to a baseline level of function with no postictal confusion but may have missed conversation and seems confused as a result. Generalized Absence Seizures Typical Atypical Absence Absence Seizures Seizures  Longer duration and less abrupt in onset and cessation  Sudden, brief lapses of consciousness without loss of  more obvious motor signs postural control   Usually associated with diffuse or Usually accompanied by subtle, bilateral motor signs multifocal structural abnormalities of the brain  generalized 2.5- to 4-Hz spike- and-wave activity with a normal interictal background  Less responsive to anticonvulsants  Generalized Tonic-Clonic Seizures The main seizure type in ∼10% of all persons with epilepsy The most common seizure type resulting from metabolic derangements The seizure usually begins abruptly without warning, aura is not common 125 Tonic phase (10 to 20 seconds Clonic phase (30 to 90 seconds) Sudden loss of consciousness Loss of posture with high risk of self Brief, violent, generalized flexor injury depending on activity contractions alternating with Brief flexion of arms, eyes deviated progressively longer muscle upward relaxation Extension of back, neck, arms, and legs -cyanosis Involuntary crying out from -possible cheek or tongue biting contraction of respiratory muscles -foamy salivation Shallow respiration, cyanosis may -possible loss of bowel or bladder occur control Ends with tremors that gradually slow -ends with deep inspiration, sustained and merge with clonic phase muscle relaxation 126  Cont’d Postictal phase (Minutes to several hours) Headache, mild confusion Muscles sore Fatigue, patient may sleep and awake refreshed Other features Fast heart rate Elevated blood pressure Respiratory and metabolic acidosis Dilated pupils Risk of aspiration pneumonia  Atonic Seizures Characterized by sudden loss of postural muscle tone lasting 1–2 s. Consciousness is briefly impaired, but there is usually no postictal confusion. A very brief seizure may cause only a quick head drop or nodding movement, Whereas a longer seizure will cause the patient to collapse 128  Myoclonic Seizures Myoclonus is a sudden and brief muscle contraction that may involve one part of the body or the entire body. Pathologic myoclonus is most commonly seen in association with Metabolic disorders, Degenerative CNS diseases, Anoxic brain injury 129  Epilepsy Syndromes An epilepsy syndrome represents a complex of clinical features, signs, and symptoms that together define a distinctive, recognizable clinical seizure disorder. Some syndromes are highly correlated with a single specific etiology (eg, severe loss of function SCN1A variants in Dravet syndrome). In contrast, others may be due to a broad range of causes (eg, infantile epileptic spasms syndrome or Lennox-Gastaut syndrome).  Epilepsy Syndromes JUVENILE MYOCLONIC LENNOX-GASTAUT EPILEPSY SYNDROME  Characterized by bilateral defined by the following triad: myoclonic jerks that may be single 1) multiple seizure types or repetitive. 2) EEG showing slow ( 5 minutes Elevation in prolactin Frequent Rare Elevation in ck Frequent Rare  Management Principles Ensure that the patient has a seizure disorder and not a condition that mimics epilepsy Confirm weather the seizure is provoked or unprovoked First time seizure or Recurrent seizure If first time unprovoked seizure, assess risk of recurrence 148  Cont’d Acute symptomatic seizure Focused on correction of the acute provoking illness and preventing its recurrence Some indications for antiseizure medications Severe electrolyte disturbances with dehydration Seizures due to meningitis or encephalitis Severe traumatic brain injury requiring intensive care 149  Cont’d First unprovoked seizure Immediate AED initiation Vs Deferring till second seizure Seizure recurrence and long-term outcomes Factors influencing the decision The risk for recurrent seizures The relative risk reduction with initiation The risks of not treating The risks of chronic antiseizure medication therapy 150  Cont’d Risk factors associated with recurrent seizures include the following: Abnormal neurologic examination (focal findings, ID) Prior brain insult such as a stroke or trauma, EEG with epileptiform abnormalities, A significant brain imaging abnormality A nocturnal seizure. 151  Cont’d Initiation of anti-seizure medication Seizure classification is an important element in designing the treatment plan Start with 10-20 % of the maintenance dose of single drug Gradually escalate the dose till seizure controlled or adverse effects develop Add additional drug if not controlled with the first drug of choice Follow-up and monitoring Utility of serum drug levels Monitoring for specific drugs Monitoring for adverse events 152  Cont’d Treatment of underlying conditions that cause or contribute to the seizures, Avoidance of precipitating factors, Suppression of recurrent seizures by prophylactic therapy with antiepileptic medications or surgery, and Addressing a variety of psychological and social issues When to Discontinue Therapy The following patient profile yields the greatest chance of remaining seizure free after drug withdrawal: Complete medical control of seizures for 1–5 years; Single seizure type Normal neurologic examination No family history of epilepsy;  Normal EEG. 154  Pregnancy and epilepsy Seizure frequency during pregnancy will remain unchanged in ~50% of women, increase in ~30%, and decrease in ~20%. Always consider eclampsia in new onset seizure during pregnancy and post partum period Incidence of fetal abnormalities is 5-6% (CVS & MSS) Valproic acid is Category X drug; Except Topiramate, newer drugs are safer than valproate 155  Cont’d Pregnant women should be maintained on effective AED therapy. Put on monotherapy at the lowest effective dose, especially during the first trimester Give folate (1-4mg/day) Treat all pregnant mothers with oral vitamin K The infant should receive intramuscular vitamin K at birth 156  Status Epilepticus Refers to continuous seizures or repetitive, discrete seizures with impaired consciousness in the interictal period  Status epilepticus has numerous subtypes, including Generalized convulsive status epilepticus (GCSE) (e.g., persistent, generalized electrographic seizures, coma, and tonic-clonic movements) and Nonconvulsive status epilepticus (e.g., persistent absence seizures or focal seizures with confusion or partially impaired consciousness, and minimal motor abnormalities)  Management The first steps in the management of a patient in GCSE are to attend to any: ABCDs Manage or Rule out Causes (hypoglycemia, Ethanol, Eclampsia, Poisoning….) Anticonvulsant therapy should then begin without delay Search for underlying cause  Other Management Issues EPILEPSY COMORBIDITIES 1. Psychiatric Problems 2. Psychosocial Issues 3. Driving 4. Employment  Mortality of Epilepsy Risk of death 2-3x greater than expected in a matched population without epilepsy. Most of the increased mortality is due to the underlying etiology of epilepsy (e.g., tumors or strokes)  A significant number of patients die from accidents, status epilepticus, and a syndrome known as sudden unexpected death in epilepsy (SUDEP), which usually affects young people with convulsive seizures and tends to occur at night). Questions? Thanks!! Cerebrovascular Accident Tadele Mesfin (M.D.) AWHSC, DBU December 2024 Outline Objectives Introduction Definition and classification Risk factors Clinical presentation Diagnosis Management Learning Objectives Understand the definition and classification of Stroke Know the different mechanisms of acute stroke Approach to a patient with stroke Basic understanding of Stroke mimics Execute appropriate management strategies for acute stroke Definition A stroke, or cerebrovascular accident, is defined as an abrupt onset of a neurologic deficit that is attributable to a focal vascular cause. Stroke refers to any damage to the brain or the spinal cord caused by an abnormality of the blood supply. The definition of stroke is clinical, and laboratory studies including brain imaging are used to support the diagnosis.  Epidemiology Stroke is the second leading cause of death worldwide. Global prevalence of stroke in 2019 was 101.5 million. Ischemic stroke 77.2 million, hemorrhagic 20.7 million and subarachnoid hemorrhage 8.4 million According to ET-CDC report stroke is the 5th common cause of death Cont’d Diagnosis and treatment of stroke patients require a basic understanding of the anatomy, physiology and pathology of the major structures involved: 1. The brain and spinal cord 2. The heart and blood vessels that supply blood to these structures 3. The blood itself Basic Vascular Anatomy The brain is a relatively small organ, accounting for only 2% of adult body weight Despite its relatively small size, the highly metabolic active organ uses; about one-quarter of the body’s energy supply and ~ 20% of the cardiac output when the body is resting These requirements for oxygen and glucose is fully dependent on the rate and the amount of blood that flows into the brain: CBF Cont’d Cont’d Cont’d Cont’d Cont’d Cont’d Classification  Cont’d A stroke can be ischemic (62%) or hemorrhagic (38%). Cerebral ischemia is caused by a reduction in blood flow that lasts longer than several seconds. There are three main causes of brain ischemia: Thrombosis Embolism Systemic hypoperfusion Cont’d  If the cessation of flow lasts for more than a few minutes, infarction or death of brain tissue results.  When blood flow is quickly restored, brain tissue can recover fully and the patient’s symptoms are only transient: this is called a transient ischemic attack (TIA). Cont’d Pathophysiology of ischemic stroke Focal cerebral infarction occurs via two distinct pathways (1) A necrotic pathway in which cellular cytoskeletal breakdown is rapid, due principally to energy failure of the cell; and (2) An apoptotic pathway in which cells become programmed to die Cont’d PARP, poly-A ribose polymerase. iNOS, inducible nitric oxide synthase;  Cont’d Causes of Ischemic stroke Risk factors for Ischemic stroke Transient Ischemic Attacks TIAs are episodes of stroke symptoms that last only briefly; the traditional definition of duration is 120 mm Hg DBP) or low BP…. WHY??? Assessment of blood glucose levels and treatment to keep levels between 140 and 180 Monitor for fever and treat it with antipyretics Monitor for signs and symptoms of ↑ intracranial pressure (ICP), and herniation.  Cont’d Prevention and treatment of poststroke complications, such as aspiration pneumonia, urinary tract infection (UTI), and deep vein thrombosis (DVT). Preventive and Long-Term Treatment Hypertension management Diabetes management Blood lipids management with a statin Aspirin or clopidogrel Diet, exercise For cardioembolic strokes, anticoagulation.  Hemorrhagic Stroke ABC‘s of life(airway, breathing, and circulation) Admission to the ICU BP reduction Elevated BP increases ICP and can cause further bleeding. However, hypotension can lower cerebral blood flow, worsening the neurologic deficits. BP reduction must be gradual with target BP