Summary

This document contains clinical knowledge about various neurological conditions such as nerve palsy, tremors and seizures. It includes causes, features, and treatments for these conditions.

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Dr Omar’s 80% Essential Clinical Knowledge – Neurology Six Nerve Palsy Fourth nerve palsy Third nerve palsy Causes: Causes Causes Injury Hypertension...

Dr Omar’s 80% Essential Clinical Knowledge – Neurology Six Nerve Palsy Fourth nerve palsy Third nerve palsy Causes: Causes Causes Injury Hypertension Diabetes mellitus Increased ICP Diabetes Vasculitis e.g. temporal Infection TIA arteritis, SLE Stroke Stroke posterior communicating Brain tumor Head trauma artery aneurysm MS cavernous sinus thrombosis Inflammation of the nerve Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia - caused by midbrain strokes amyloid, multiple sclerosis Features: Features Features Double vision Vertical diplopia Eye is deviated 'down and out' Diplopia (with both eyes Subjective tilting of objects Ptosis opened – More when look (torsional diplopia) Pupil may be dilated faraway) The patient may develop a head (sometimes called a 'surgical' Strabismus (eye in affected tilt, which they may or may not third nerve palsy) side drifts toward midline) be aware of Other symptoms: headache – When looking straight ahead, the nausea – vomiting - vison loss affected eye appears to deviate – hearing loss upwards and is rotated outwards Tremors 341 Dr Omar’s 80% Essential Clinical Knowledge – Neurology 342 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Classification of Seizures 343 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Management Most neurologists now start antiepileptics following a second epileptic seizure. NICE guidelines suggest starting antiepileptics after the first seizure if any of the following are present: the patient has a neurological deficit brain imaging shows a structural abnormality the EEG shows unequivocal epileptic activity the patient or their family or carers consider the risk of having a further seizure unacceptable Generalised tonic-clonic seizures Males: sodium valproate * Females: lamotrigine or levetiracetam Girls aged under 10 years and who are unlikely to need treatment when they are old enough to have children or women who are unable to have children may be offered sodium valproate first-line * Focal seizures first line: lamotrigine or levetiracetam second line: carbamazepine, oxcarbazepine or zonisamide Absence seizures (Petit mal) first line: ethosuximide second line: o male: sodium valproate * o female: lamotrigine or levetiracetam carbamazepine may exacerbate absence seizures Myoclonic seizures males: sodium valproate * females: levetiracetam Tonic or atonic seizures males: sodium valproate * females: lamotrigine * From January 2024, valproate must not be started in new patients (male or female) younger than 55 years, unless two specialists independently consider and document that there is no other effective or tolerated treatment, or there are compelling reasons that the reproductive risks do not apply. For the majority of patients, other effective treatment options are available. Seizures: acute management Most seizures are self-limiting and stop spontaneously but prolonged seizures may be potentially life- threatening. For people having a tonic-clonic seizure, note the time, and if it lasts less than 5 minutes: Protect them from injury by: Cushioning their head, for example with a pillow - Removing glasses if they are wearing them - Removing harmful objects from nearby Do not restrain them or put anything in their mouth. When the seizure stops, check their airway and place them in the recovery position. Observe them until they have recovered. Arrange emergency admission (call for an ambulance) if it is their first seizure; a seizure reoccurs shortly after the first one; the person is injured or having trouble breathing after the seizure or is difficult to wake up. 344 Dr Omar’s 80% Essential Clinical Knowledge – Neurology For people having a tonic-clonic seizure lasting more than 5 minutes, or who have more than three seizures in an hour, in addition to the above measures: Treat with one of the following: o Buccal midazolam as first-line treatment in the community. o Rectal diazepam if preferred, or if buccal midazolam is not available. o Intravenous lorazepam if intravenous access is already established o Note that midazolam oromucosal solution is not licensed for children under 3 months or adults over 18 years of age (for infants between 3–6 months of age treatment should be in a hospital setting); and some preparations of rectal diazepam are not licensed for children under 1 year of age. Call an ambulance for urgent hospital admission if seizures do not respond promptly to treatment. Call an ambulance for urgent hospital admission if seizures do respond to treatment but: o Seizures were prolonged or recurrent before treatment was given, particularly if seizures had developed into status epilepticus. o There is a high risk of recurrence, such as a history of repeated seizures or status epilepticus. o There are difficulties monitoring the person's condition. o This is their first seizure. Arrange for specialist review for consideration of providing the family or carers with buccal midazolam or rectal diazepam (if not already provided), to treat future prolonged or recurrent seizures according to an individually agreed protocol. Convulsive status epilepticus It is a prolonged convulsive seizure lasting for 5 minutes or longer, or recurrent seizures one after the other without recovery in between Epilepsy in children: syndromes A) Infantile spasms (West's syndrome) Infantile spasms, or West syndrome, is a type of childhood epilepsy which typically presents in the first 4 to 8 months of life and is more common in male infants. They are often associated with a serious underlying condition (e.g. TS, encephalitis, birth asphyxia) and carry a poor prognosis Features Characteristic 'salaam' attacks: flexion of the head, trunk and arms followed by extension of the arms Lasts only 1-2 seconds but may be repeated up to 50 times Progressive mental handicap Investigation EEG shows hypsarrhythmia in two-thirds of infants CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis) Management poor prognosis vigabatrin is now considered first-line therapy ACTH is also used 345 Dr Omar’s 80% Essential Clinical Knowledge – Neurology B) Benign Rolandic epilepsy It is a form of childhood epilepsy that typically occurs between the age of 4 and 12 years. Features seizures characteristically occur at night seizures are typically partial (e.g. paraesthesia affecting the face) but secondary generalisation may occur (i.e. parents may only report tonic-clonic movements) the child is otherwise normal EEG characteristically shows centrotemporal spikes The prognosis is excellent, with seizures stopping by adolescence C) Typical (petit mal) absence seizures It is characterized by a brief altered state of consciousness and staring episodes Juvenile Absence Epilepsy Features The child may be unresponsive, then return to activity unaware that anything has happened. The seizures often occur during exercise. This type of epilepsy can be inherited, and one-third third of children with it have a family history or seizures. Seizures typically begin between ages 3 and 6 years. EEG testing shows a 3 Hz generalized spike and waves. Treatment The prognosis for CAE is good, and 60 percent of patients outgrow their seizures. Seizures are often controlled with anti-epileptic epileptic drugs (sodium valproate and ethosuximide are first first-line treatment) D) Lennox-Gastaut Gastaut syndrome Lennox-Gastaut Gastaut syndrome is a severe type of epilepsy characterized by three factors: Multiple seizure types (myoclonic, tonic, atonic, atypical absence, etc.) A distinctive brain-wave wave pattern Mental ental deficiency that can range from slight to profound cognitive impairment The peak age of onset is between the ages of 3 and 5 years. There is a slightly greater prevalence in boys than girls. It is estimated that between 20 and 60 percent of children with this syndrome have a prior history of infantile spasms. Feature The first sign in about one-third of children with Lennox-Gastaut syndrome is a prolonged seizure. The child usually has multiple seizures throughout the day. Tonic seizures (muscle stiffening) are the most common and usually occur during sleep. They usually last about 10 seconds, but they can last up to one minute. An EEG will show a slow spike and wave pattern that is specific to this syndrome Treatment Children with Lennox-Gastaut are treated with multiple epileptic drugs to control their seizures. A vagus nerve stimulator has shown to reduce the frequency and/or intensity of seizures of children with this type of epilepsy. Corpus callosotomy is also effective. 346 Dr Omar’s 80% Essential Clinical Knowledge – Neurology E) Juvenile myoclonic epilepsy (Janz syndrome) It is a hereditary form of epilepsy that begins at puberty Features: The primary type of seizures are myoclonic, especially on awakening. They may occur in clusters, or several times a day for several days in a row. JME is also associated with generalized tonic-clonic and absence seizures. They may be brought on by lack of sleep, early awakening, alcohol and drug use, stress, strong emotion, and menstruation. Seizures can also be brought on by photic stimulation such as flickering lights, TV, video games, or light shining through trees or reflecting off water or snow. The EEG shows interictal fast (4-6 Hz) spike and wave and polyspike and wave discharges. Management JME is usually well controlled with medication. Most patients with JME do not outgrow their seizures and will need to take medication for the rest of their lives. Individuals are encouraged to get enough sleep and avoid alcohol to reduce the likelihood of seizures. Feature of Cerebellar disease Ataxia Dysarthria (Speech problems) Uncoordinated movement Nystagmus Vertigo Reflexes The common reflexes are listed below: Reflex Root Ankle S1-S2 Knee L3-L4 Biceps C5-C6 Triceps C7-C8 347 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Myasthenia gravis Myasthenia gravis is an autoimmune disorder. Myasthenia is more common in women (2:1) The key feature is muscle fatigability - muscles become progressively weaker during periods of activity and slowly improve after periods of rest: Extraocular muscle weakness: diplopia Proximal muscle weakness: face, neck, limb girdle Ptosis Dysphagia Associations Thymomas in 15% Autoimmune disorders: pernicious anemia, autoimmune thyroid disorders, rheumatoid, SLE Thymic hyperplasia in 50-70% Investigations Single fiber electromyography (EMG): high sensitivity (92-100%) CK normal CT thorax to exclude thymoma Autoantibodies: around 85-90% 90% of patients have antibodies to acetylcholine receptors. In the remaining patients, about about 40% are positive for anti-muscle anti-muscle-specific muscle tyrosine kinase antibodies Exacerbating factors The most common exacerbating factor is exertion resulting in fatigability, which is the hallmark feature of myasthenia gravis. Symptoms become more marked during the day The following drugs may exacerbate myasthenia: Penicillamine Quinidine, procainamide Beta-blockers Lithium Phenytoin Antibiotics: gentamicin, macrolides, quinolones, tetracyclines Management Long-acting acetylcholinesterase inhibitors; pyridostigmine is first-line Immunosuppression is usually not started at diagnosis, but the majority of patients eventually require it in addition to long-acting acetylcholinesterase inhibitors: o prednisolone initially o azathioprine, cyclosporine, mycophenolate mofetil may also be used Thymectomy Management of myasthenic crisis Plasmapheresis Intravenous immunoglobulins 348 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Complex regional pain syndrome It describes several neurological and related symptoms which typically occur following surgery or a minor injury. CRPS is 3 times more common in women. There are two types of CRPS: Type I (most common): there is no demonstrable lesion to a major nerve. Type II: there is a lesion to a major nerve. Features Progressive, disproportionate symptoms to the original injury/surgery Allodynia "pain due to a stimulus that does not normally provoke pain." Temperature and skin colour changes Oedema and sweating Motor dysfunction The Budapest Diagnostic Criteria are commonly used in the UK Management Early physiotherapy is important. Neuropathic analgesia in-line with NICE guidelines Specialist management (e.g. Pain team) is required. Botulinum toxin Indications: blepharospasm hemifacial spasm focal spasticity including cerebral palsy patients, hand and wrist disability associated with stroke spasmodic torticollis severe hyperhidrosis of the axillae achalasia 349 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Bell's palsy Bell's palsy may be defined as an acute, unilateral, idiopathic, facial nerve paralysis. The etiology is unknown although the role of the herpes simplex virus has been investigated previously. The peak incidence is 20-40 years, and the condition is more common in pregnant women. Features: (see table) Lower motor neuron facial nerve palsy - forehead affected o in contrast, an upper motor neuron lesion 'spares' the upper face Patients may also notice post-auricular pain (may precede paralysis), altered taste, dry eyes, hyperacusis Management For people presenting within 72 hours of the onset of symptoms, consider prescribing prednisolone. There is no consensus regarding the optimum dosing regimen, but options include: Giving 50 mg daily for 10 days or Giving 60 mg daily for 5 days followed by a daily reduction in dose of 10 mg (for a total treatment time of 10 days) if a reducing dose is preferred. Antiviral treatments alone are not recommended. Antiviral treatment in combination with a corticosteroid may be of small benefit but seek specialist advice if this is being considered. Eye care is important - prescription of artificial tears and eye lubricants should be considered 350 Dr Omar’s 80% Essential Clinical Knowledge – Neurology When should I refer someone with Bell's Palsy? Refer people urgently if clinical features, history and examination suggest facial nerve palsy may be caused by: o An upper motor neurone cause (for example limb paresis, facial paraesthesia, other cranial nerve involvement, postural imbalance). o Cancer (for example, gradual onset of symptoms, persistent facial paralysis for more than 6 months, pain in the distribution of the facial nerve, head or neck lesion suggestive of cancer, history of head and neck cancer, hearing loss on the affected side). o Trauma. o Acute systemic or severe local infection. Refer other people to an appropriate facial nerve specialist (for example, a neurologist, or ear, nose and throat specialist) with urgency determined by clinical judgment, if: o Atypical features are present. o There is no improvement after 3 weeks of treatment. o Existing neurologic findings worsen, or if new neurologic findings develop. o There is incomplete recovery after 3 months of onset of symptoms. o The diagnosis is uncertain. Multiple Sclerosis F>M incidence - two to three times as many women as men Onset is usually between 16 and 50 years with a peak between 20 20-40 years. Clinical features The cardinal feature of multiple sclerosis is the presence of multiple neurological defects which are separated in time and space. 1) Visual Symptoms optic neuritis:: common presenting feature usually unilateral - rarely bilateral optic atrophy Worsening of vision following rise in body temperature 2) Neurogenic bladder dysfunction: urgency is common - incontinence and frequency may also be present. 3) Impotence 4) Sensory deficits: tingling in restricted distribution, e.g. spreading up a leg over a couple of days and resolving over a couple of weeks pins/needles numbness Lhermitte's phenomenon: a sensation like electricity passing down the spine on neck flexion trigeminal neuralgia with facial weakness and sensory loss 5) Upper motor neurone deficit: most commonly a spastic weakness of the legs 6) Cerebellar damage: ataxia – dysarthria - nystagmus 7) Vertigo is common. 8) Dementia in the later stages 351 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Investigations MRI scanning: permits the visualisation of the dissemination of lesions in time and space - will reliably identify plaques in the cortex, brainstem and spinal cord CSF: permits the detection of intrathecal inflammation – pleocytosis (high lymphocytes) - slightly raised protein Visual evoked potentials: permit the demonstration of conduction changes consistent with demyelination are of normal amplitude but show an increased latency in patients who have or have had optic neuritis measurement of visual evoked potentials is a good way of demonstrating a previous, often subclinical, episode of demyelination in order to make the diagnosis of multiple sclerosis Before referring a person suspected of having MS to a neurologist, exclude alternative diagnoses by performing blood tests including: full blood count inflammatory markers for example erythrocyte sedimentation rate, C-reactive protein liver function tests renal function tests calcium, glucose, vitamin B12 thyroid function tests HIV serology Do not diagnose MS on the basis of MRI findings alone Treatment of spasticity with Multiple Sclerosis a) Baclofen – Gabapentin b) Diazepam – Dantrolene – Tizanidine c) Physiotherapy Multiple sclerosis (MS) relapse Corticosteroids are the mainstay of management of the acute relapse. Steroids will reduce the severity and duration of a relapse, probably by reducing oedema, but the progression of disability is not affected. Recognising a relapse: diagnose a relapse of MS if the person: Develops new symptoms or Worsening of existing symptoms. Last for more than 24 hours in the absence of infection or any other cause after a stable period of at least 1 month Assess and offer treatment for relapses of MS, that affect the person's ability to perform their usual tasks, as early as possible and within 14 days of onset of symptoms. Do not routinely diagnose a relapse of MS if symptoms are present for more than 3 months Treatment of a relapse NICE suggest a course of high-dose corticosteroids: offer treatment for relapse of MS with oral methylprednisolone 0.5 g daily for 5 days consider intravenous methylprednisolone 1 g daily for 3-5 days as an alternative for people with MS non-specialists should discuss a person's diagnosis of relapse and whether to offer steroids with a healthcare professional with expertise in MS because not all relapses need treating with steroid 352 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Motor neuron disease Motor neuron disease is a neurological condition of unknown cause which can present with both upper and lower motor neuron signs. It rarely presents before 40 years and various patterns of disease are recognized including amyotrophic lateral sclerosis, progressive muscular atrophy and bulbar palsy. UMNL vs LMNS Feature UMNL LMNL Site of the lesion Cerebral Hemisphere – Cerebellum – Anterior horn cell – nerve roots – Brainstem – Spinal cord peripheral nerves – Neuromuscular junctions Muscle Weakness Quadriplegia – Hemiplegia – Diplegia – Proximal myopathy – distal Paraplegia neuropathy Muscle Tone Spasticity – Rigidity Hypotonia Fasciculations Absent Present (particularly tongue) Tendon Reflexes Hyperreflexia Hyporeflexia – Areflexia Abdominal Reflexes Absent Present Sensory Loss Cortical Sensation Peripheral sensations EMG Normal Abnormal The diagnosis of motor neuron disease Clinical, but Nerve erve conduction studies will show normal motor conduction and can help exclude neuropathy. Electromyography shows a reduced number of action potentials with increased amplitude. MRI is usually performed to exclude the differential diagnosis of cervical cord compression and myelopathy Management Riluzole Prevents revents stimulation of glutamate receptors Used sed mainly in amyotrophic lateral sclerosis Prolongs life by about 3 months Respiratory care Non-invasive ventilation (usually BIPAP) is used at night Studies have shown a survival benefit of around 7 months Prognosis Poor: 50% of patients die within 3 years 353 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Cavernous sinus thrombosis Causes: A) Primary, or aseptic, cerebral venous thrombosis is rare. It usually occurs in the very young, the very old, or the debilitated. Predisposing factors include anaemia, increased blood coagulability, hypotension, cachexia, dehydration B) Causes of secondary thrombosis include: Most frequently, direct injury through a fracture of the skull or other trauma Spread of infection from a neighbouring site Pregnancy, puerperium Oral contraceptive pill Dehydration Haematological disease, for example polycythaemia Malignancy - metastatic emboli Miscellaneous, including polyarteritis nodosa. Features Fevers and rigors Pain in the eye and forehead; Severe headache especially when lying down. Exophthalmos and occasionally, papilledema Cranial nerve palsies - III, IV, VI Oedema of the periorbital structures and forehead due to blockage of venous drainage symptoms are most usually unilateral but can extend via the circular sinus to become bilateral. Investigation Blood cultures for infection lumbar puncture CT scan MRI scan Carotid angiography. Management: entails consideration of the underlying aetiology: Primary thrombosis - anticoagulants Secondary thrombosis: o Antibiotics if infectious o Anticonvulsants o Surgical drainage of sinuses if these are the source of infection. o Mastoidectomy may be indicated if the cause was an acute mastoiditis. o High dose dexamethasone to reduce cerebral oedema. 354 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Guillain-Barre syndrome: features Guillain-Barre syndrome describes an immune-mediated demyelination of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni). Features 65% of patients experience back/leg pain in the initial stages of the illness. Characteristic feature is progressive, symmetrical weakness of all the limbs; the weakness is classically ascending i.e. the legs are affected first. Reflexes are reduced or absent. Sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs. There may be a history of gastroenteritis. Respiratory muscle weakness Cranial nerve involvement; diplopia - bilateral facial nerve palsy - oropharyngeal weakness Autonomic involvement; urinary retention – diarrhoea Papilloedema: thought to be secondary to reduced CSF resorption Investigations Lumbar puncture;; rise in protein with a normal white blood cell count Nerve conduction studies may be performed o decreased motor nerve conduction velocity (due to demyelination) o prolonged distal motor latency o increased F wave latency Management Supportive Restless legs syndrome Males and females are equally affected and a family history may be present Clinical features Uncontrollable urge to move legs (akathisia). Symptoms initially occur at night but as condition progresses may occur during the day. Symptoms are worse at rest. Paraesthesias e.g. 'crawling' or 'throbbing' sensations Movements during sleep may be noted by the partner - periodic limb movements of sleeps (PLMS) Causes and associations Positive family history in 50% of patients with idiopathic RLS Iron deficiency anaemia Uraemia Diabetes mellitus Pregnancy The diagnosis is clinical although bloods such as ferritin to exclude iron deficiency anemia. Management Simple measures: walking, stretching, massaging affected limbs. Treat any iron deficiency. First-line treatment; dopamine agonists (e.g. Pramipexole, ropinirole) Benzodiazepines Gabapentin 355 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Neuropathic pain Examples include: Diabetic neuropathy Post-herpetic neuralgia Trigeminal neuralgia Prolapsed intervertebral disc NICE updated their guidance on the management of neuropathic pain in 2013: First-line treatment: amitriptyline, duloxetine, gabapentin or pregabalin o If the first-line drug treatment does not work, try one of the other 3 drugs o In contrast to standard analgesics, drugs for neuropathic pain are typically used as monotherapy, i.e. if not working then drugs should be switched, not added Topical capsaicin may be used for localized neuropathic pain (e.g. post-herpetic neuralgia) Pain management clinics may be useful in patients with resistant problems For people awaiting referral after initial treatments have failed, consider prescribing a short course of tramadol for pain relief. Prescribe tramadol cautiously, bearing in mind the potential for misuse Peripheral neuropathy Peripheral neuropathy may be divided into conditions which predominately cause a motor or sensory loss Predominately motor loss Guillain-Barre syndrome Porphyria Lead poisoning Hereditary ereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth Charcot Chronic hronic inflammatory demyelinating polyneuropathy (CIDP) Diphtheria Predominately sensory loss Diabetes Uraemia Leprosy Alcoholism Vitamin B12 deficiency Amyloidosis Drugs causing peripheral neuropathy Amiodarone Isoniazid Vincristine Nitrofurantoin Metronidazole 356 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Idiopathic intracranial hypertension Idiopathic intracranial hypertension (also known as pseudotumor cerebri and formerly benign intracranial hypertension) is a condition classically seen in young, overweight females. Risk factors Obesity Female sex Pregnancy Drugs; combined oral contraceptive pill – steroids – tetracyclines - vitamin A - lithium Features Headache Blurred vision Papilloedema (usually present) Enlarged blind spot Sixth nerve palsy may be present Management Weight loss Diuretics e.g. acetazolamide Topiramate is also used, and has the added benefit of causing weight loss in most patients Repeated lumbar puncture Surgery: optic nerve sheath decompression and fenestration may be needed to prevent damage to the optic nerve. A lumboperitoneal or ventriculoperitoneal shunt may also be performed to reduce intracranial pressure. Normal pressure hydrocephalus Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. A classical triad of features is seen Urinary incontinence Dementia and bradyphrenia Gait abnormality (may be similar to Parkinson's disease) Symptoms typically develop over a few months. Imaging Hydrocephalus with ventriculomegaly in the absence of, or out of proportion to, sulcal enlargement Management Ventriculoperitoneal shunting Around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages 357 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Parkinson's disease Parkinson's disease is a progressive neurodegenerative condition caused by degeneration of dopaminergic neurons in the substantia nigra. This results in a classic triad of features: bradykinesia, tremor and rigidity. Epidemiology Around twice common in men Mean age of diagnosis is 65 years Features The symptoms of Parkinson's disease are characteristically asymmetrical. Bradykinesia Poverty of movement also seen, sometimes referred to as hypokinesia Short, shuffling steps with reduced arm swinging Difficulty in initiating movement Tremor Most marked at rest Worse when stressed or tired, improves with voluntary movement Typically, 'pill-rolling', i.e. in the thumb and index finger Rigidity Lead pipe Cogwheel: due to superimposed tremor Other characteristic features Mask-like facies Flexed posture Micrographia; abnormally small handwriting or handwriting that becomes progressively smaller Drooling of saliva Psychiatric sychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis and sleep disturbances may also occur Impaired olfaction REM sleep behavior disorder Fatigue Autonomic dysfunction: postural hypotension Diagnosis 1. Is usually clinical. 2. However, if there is difficulty differentiating between essential tremor and Parkinson's disease NICE recommend considering 123I-FP-CIT single photon emission computed tomography (SPECT). Management A) Non-pharmacological management: Exercise - physical activity has been shown to be very important in reducing motor and non-motor symptoms; consider referring to physio especially if balance or motor problems. Consider referral to Occupational Therapy if difficulty with activities of daily living & to Speech & Language therapy if problems with communication, swallowing or saliva. 358 Dr Omar’s 80% Essential Clinical Knowledge – Neurology B) Drug treatment of motor symptoms: All drugs are for the symptomatic benefit, and none influence the long-term progression. Initiation and alteration of all drugs should be done under specialist supervision. First-line drugs: o Levodopa - is better for motor symptoms with fewer adverse effects but higher long-term motor complications. o Dopamine agonists (non-ergot derived e.g. pramipexole, ropinirole) - less good for motor symptoms but fewer motor complications, but higher adverse effects. o MAO-B inhibitors - less good for motor symptoms but fewer motor complications, but higher adverse effects). Adjuvant therapy: consider adding dopamine agonist, MAO-B inhibitor or COMT inhibitor to levodopa if dyskinesia or motor fluctuations despite optimal levodopa therapy. Impulse control disorders (e.g. hypersexuality, gambling, binge eating) can occur with any dopaminergic therapy, but particularly dopamine agonists; warn patients and family about this potential complication as can be distressing. o If it occurs seek specialist advice, but we should not alter/stop medications without advice - medications usually need to be slowly reduced due to the risk of dopamine withdrawal. C) Non-motor symptom treatment (review potential causative/contributory drugs in all cases): Daytime sleepiness, particularly associated with dopamine agonists; consider modafinil. Rapid eye movement sleep disorder - consider clonazepam or melatonin melatonin. Orthostatic hypotension: meds review important; consider midodrine or fludrocortisone. Depression - can be difficult to diagnose as features may be wrongly attributed to the PD; consider CBT; the best evidence is for TCAs but use may be limited by side effects (cognitive impairment and falls) so SSRIs may be more appropriate. Psychosis: don’t treat if well tolerated; consider quetiapine or clozapine (specialist only). Dementia: consider cholinesterase inhibitor or memantine. Drooling: refer to speech & language therapy or consider glycopyrronium bromide. Parkinsonism Causes of Parkinsonism Parkinson's disease progressive supranuclear palsy multiple system atrophy Wilson's disease post-encephalitis dementia pugilistica (secondary to chronic head trauma e.g. boxing) toxins: carbon monoxide, MPTP Drug-induced e.g. antipsychotics, metoclopramide Drug-induced parkinsonism has slightly different features to Parkinson's disease: motor symptoms are generally rapid onset and bilateral rigidity and rest tremor are uncommon 359 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Transient ischemic attack Clinical features The clinical features are like those of a stroke, i.e. sudden onset, focal neurological deficit but, rather than persisting, the features resolve, typically within 1 hour. Possible features include Unilateral weakness or sensory loss. Aphasia or dysarthria Ataxia, vertigo, or loss of balance Visual problems o sudden transient loss of vision in one eye (amaurosis fugax) o diplopia o homonymous hemianopia Assessment and referral The ABCD2 prognostic score is no longer recommended by NICE Referral: if the patient has had more than 1 TIA ('crescendo TIA') or has a suspected cardioembolic source or severe carotid stenosis: o discuss the need for admission or observation urgently with a stroke specialist If the patient has had a suspected TIA in the last 7 days: days: o arrange urgent assessment (within 24 hours) by a specialist stroke physician if the patient has had a suspected TIA which occurred more than a week previously previously: o refer for specialist assessment as soon as possible within 7 days Management,, NICE recommend: Immediate antithrombotic therapy: aspirin 300 mg immediately, unless a) The he patient has a bleeding disorder or is taking an anticoagulant (needs immediate admission for imaging to exclude a hemorrhage) hemorrhage b) The patient is already taking low-dose aspirin regularly: continue the current dose of aspirin until reviewed by a specialist c) Aspirin is contraindicated: discuss management urgently with the specialist team Advise the person not to drive until they have been seen by a specialist. Investigations Neuroimaging NICE recommend that CT brains should not be done 'unless there is clinical suspicion of an alternative diagnosis that CT could detect' MRI is preferred to determine the territory of ischaemia, or to detect haemorrhage or alternative pathologies; it should be done on the same day as specialist assessment if possible Carotid imaging Atherosclerosis in the carotid artery may be a source of emboli in some patients All patients should therefore have an urgent carotid doppler unless they are not a candidate for carotid endarterectomy 360 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Long term management (secondary prevention) Antithrombotic therapy Clopidogrel is recommended first-line (as for patients who've had a stroke) Aspirin + dipyridamole should be given to patients who cannot tolerate clopidogrel Evidence supports dual antiplatelet therapy (DAPT) in acute phase after minor ischaemic stroke or high-risk TIA for 21 days (National Stroke Guideline 2023): Clopidogrel + aspirin (both 300mg loading dose then 75mg OD), or Ticagrelor (180mg loading dose then 90mg BD) + aspirin (dose above) Lipid modification drug treatment Unless contraindicated, treatment with a high-intensity statin (such as atorvastatin 80 mg daily) will be offered at diagnosis of stroke or TIA by secondary care. The aim of statin therapy is to reduce non- HDL cholesterol by more than 40%. Fibrates, bile acid sequestrants, nicotinic acid, and omega-3 fatty acid compounds should not be prescribed to people with ischaemic stroke or TIA for secondary vascular prevention. Stroke Assessment The FAST-screening tool (Facial weakness, Arm weakness, Speech problems, Time to call 999 999) is widely known by the general public following a publicity campaign. It has a positive predictive value of 78%. A variant of FAST called the ROSIER score is useful for medical professionals. It is a validated tool recommended by the Royal College of Physicians. ROSIER score Exclude hypoglycaemia first, then assess the following: Assessment Scoring Loss of consciousness or syncope - 1 point Seizure activity - 1 point New, acute onset of: asymmetric facial weakness + 1 point asymmetric arm weakness + 1 point asymmetric leg weakness + 1 point speech disturbance + 1 point visual field defect + 1 point A stroke is likely if > 0. Investigations A non-contrast CT head scan is the first line radiological investigation for suspected stroke; one of the key questions to answer is whether there is an ischaemic stroke or haemorrhagic stroke. Management Selected points relating to the management of acute stroke include: blood glucose, hydration, oxygen saturation and temperature should be maintained within normal limits blood pressure should not be lowered in the acute phase unless there are complications e.g. Hypertensive encephalopathy Aspirin 300mg orally or rectally should be given as soon as possible if a hemorrhagic stroke has been excluded 361 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Thrombolysis for acute ischemic stroke It recommends that patients with an acute ischaemic stroke, regardless of age or stroke severity, who were last known to be well more than 4.5 hours earlier, should be considered for thrombolysis with alteplase if: treatment can be started between 4.5 and 9 hours of known onset, or within 9 hours of the midpoint of sleep when they have woken with symptoms, AND they have evidence from CT/MR perfusion (core-perfusion mismatch) or MRI (DWI-FLAIR mismatch) of the potential to salvage brain tissue This should be irrespective of whether they have a large artery occlusion and require mechanical thrombectomy. Blood pressure should be lowered to 185/110 mmHg before thrombolysis. Contraindications to thrombolysis: Absolute Relative Previous intracranial haemorrhage Pregnancy Seizure at onset of stroke Concurrent anticoagulation Intracranial neoplasm (INR >1.7) Suspected subarachnoid haemorrhage Haemorrhagic diathesis Stroke or traumatic brain injury in preceding 3 months Active diabetic haemorrhagic Lumbar puncture in preceding 7 days retinopathy Gastrointestinal haemorrhage in preceding 3 weeks Suspected intracardiac Active bleeding thrombus Oesophageal varices Major surgery / trauma in Uncontrolled hypertension >200/120mmHg the preceding 2 weeks Secondary prevention Same as TIA 362 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Syringomyelia Syringomyelia (‘syrinx’ for short) describes a collection of cerebrospinal fluid within the spinal cord. 363 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Causes Chiari malformation: strong association Trauma Tumors Idiopathic Features ‘Cape-like’ (neck, shoulders and arms) loss of sensation to temperature but the preservation of light touch, proprioception, and vibration Classic examples are of patients who accidentally burn their hands without realizing. Spastic weakness (predominantly of the lower limbs) Neuropathic pain Upgoing plantars Horner’s syndrome due to compression of the sympathetic chain, but this is rare. Bowel and bladder dysfunction Scoliosis will occur over a matter of years if the syrinx is not treated. 364 Dr Omar’s 80% Essential Clinical Knowledge – Neurology Investigations Full spine MRI with contrast to exclude a tumour or tethered cord. Brain MRI is also needed to exclude a Chiari malformation. Treatment Directed at treating the cause of the syrinx. In patients with a persistent or symptomatic syrinx, a shunt into the syrinx can be placed. Brown-Sequard Syndrome 365 Dr Omar’s 80% Essential Clinical Knowledge – Neurology 366

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