Neurology Flashcards

Question

Visual field defects

Answer 1

- Left homonymous hemianopia means visual field defect to the left, i.e. Lesion of right optic tract - Homonymous quadrantanopias: PITS (lesion in Parietal-Inferior, lesion in Temporal-Superior) - incongruous defects = optic tract lesion; congruous defects = optic radiation lesion or occipital cortex

Answer 2

Neuropathic pain may be defined as pain which arises following damage or disruption of the nervous system. It is often difficult to treat and responds poorly to standard analgesia. Examples include: - diabetic neuropathy - post-herpetic neuralgia - trigeminal neuralgia - prolapsed intervertebral disc

Answer 3

first-line treatment*: - amitriptyline, duloxetine, gabapentin or pregabalin - if the first-line drug treatment does not work try one of the other 3 drugs - tramadol may be used as 'rescue therapy' for exacerbations of neuropathic pain - topical capsaicin may be used for localised neuropathic pain (e.g. post-herpetic neuralgia) - pain management clinics may be useful in patients with resistant problems *please note that for some specific conditions the guidance may vary. For example carbamazepine is used first-line for trigeminal neuralgia

Answer 4

Investigate for alternative diagnoses. - initial investigation - assessment of CN III,IV and VI This is because a common sign of PSP (progressive supranuclear palsy), the most likely alternative diagnosis (and a 'Parkinson's plus' syndrome), commonly manifests with a VERTICAL SUPRANUCLEAR GAZE PALSY. Even if the diagnosis is not PSP, assessment of cranial nerves should be performed in anyone who presents with a fall.

Answer 5

aka Steele-Richardson-Olszewski syndrome a 'Parkinson Plus' syndrome Features - impairment of VERTICAL GAZE (DOWN gaze worse than up gaze - patients may complain of difficultly reading or descending stairs) - parkinsonism - falls - slurring of speech - cognitive impairment Management - poor response to L-dopa

Answer 6

They typically occur in clusters lasting several weeks, with the clusters themselves typically once a year. Cluster headaches are more common in men (3:1) and smokers. ALCOHOL may trigger an attack and there also appears to be a relation to nocturnal SLEEP. Features - pain typical occurs once or twice a day, each EPISODE lasting 15 mins - 2 hours - clusters typically last 4-12 weeks - intense SHARP, stabbing pain around one eye (recurrent attacks 'always' affect same side) - patient is restless and AGITATED during an attack accompanied by REDNESS, LACRIMATION, lid swelling nasal stuffiness miosis and ptosis in a minority

Answer 7

Management - acute: 100% oxygen (80% response rate within 15 minutes), SUBC triptan (75% response rate within 15 minutes) triptans are contraindicated in patients with CAD as they have the potential to cause coronary vasospasm - prophylaxis: verapamil is the drug of choice. There is also some evidence to support a tapering dose of prednisolone NICE recommend seeking specialist advice from a neurologist if a patient develops cluster headaches with respect to neuroimaging

Answer 8

Always replace VITAMIN B12 BEFORE folate - giving folate to a patient deficient in B12 can precipitate SUBACUTE COMBINED DEGENERATION of the cord

Answer 9

Due to vitamin B12 DEFICIENCY - Dorsal + lateral columns affected - Joint position and vibration sense lost first then distal paraesthesia - upper motor neuron signs typically develop in the legs, classically extensor plantars, brisk knee reflexes, absent ankle jerks - if untreated stiffness and weakness persist ---------------------------------------------- Damage to the posterior columns - loss of proprioception, light touch and vibration sense (sensory ataxia and a positive Romberg's test). Damage to lateral columns - spastic weakness and upgoing plantars (UMN signs). Damage to peripheral nerves - absent ankle and knee jerks (LMN signs). - When there is a mix of UMN and LMN signs in a patient, always consider SCDC. ``` ------ Therefore the classic triad of symptoms is: 1. Extensor plantar response 2. Brisk knee jerks 3. Absent ankle jerks ```

Answer 10

A subtype of MND May present with mixed UMN and LMN - no sensory deficits Death of both upper and lower motor neurons in the motor cortex of the brain, the brain stem, and the spinal cord.

Answer 11

Autoimmune disease affecting the neuromuscular junction so will NOT present with UMN signs or sensory loss.

Answer 12

Charcot-Marie-Tooth disease is a hereditary SENSORY and MOTOR PERIPHERAL neuropathy. It results in a predominantly motor loss. UMN signs are NOT present in these patients. However, patients can present with LMN signs in all limbs and REDUCED SENSATION (more pronounced distally). Charcot-Marie-Tooth Disease is the most common hereditary peripheral neuropathy Features: - There may be a history of frequently sprained ankles - Foot drop - High-arched feet (pes cavus) - Hammer toes - Distal muscle weakness - Distal muscle atrophy - Hyporeflexia - Stork leg deformity

Answer 13

CARDIAC monitoring is required due to the pro-arrhythmogenic effects it elicits.

Answer 14

Phenytoin is used in the management of seizures. Mechanism of action: binds to sodium channels INCREASING their REFRACTORY PERIOD Adverse effects: Phenytoin is associated with a large number of adverse effects. These may be divided into acute, chronic, idiosyncratic and teratogenic. Phenytoin is also an INDUCER of the P450 system. Acute - initially: dizziness, diplopia, nystagmus, slurred speech, ataxia - later: confusion, seizures - reduced sensation in a glove-and-stocking distribution - reduced ankle reflex Chronic - common: gingival hyperplasia (secondary to increased expression of platelet derived growth factor, PDGF), hirsutism, coarsening of facial features, drowsiness - megaloblastic anaemia (secondary to altered folate metabolism) - peripheral neuropathy - enhanced vitamin D - metabolism causing osteomalacia - lymphadenopathy - dyskinesia ``` Idiosyncratic - fever - rashes, including severe - reactions such as toxic epidermal necrolysis hepatitis - Dupuytren's contracture* - aplastic anaemia - drug-induced lupus ``` Teratogenic - associated with cleft palate and congenital heart disease Monitoring Phenytoin levels do not need to be monitored routinely but trough levels, immediately before dose should be checked if: - adjustment of phenytoin dose - suspected toxicity - detection of non-adherence to the prescribed medication *although not listed in the BNF

Answer 15

1. Progressive mental impairment and dementia 2. Difficulty walking 3. Impaired bladder control The gait disturbance is often the most noticeable symptom, it can resemble a parkinsonian gait but unlike Parkinson's there is no rigidity or tremor.

Answer 16

Starts with patients having IMPAIRED BALANCE and therefore being prone to many FALLS. On examination, they have a VERTICAL GAZE PALSY. It has a SYMMETRICAL onset and is poorly responsive to levodopa, unlike Parkinson's disease.

Answer 17

Progressive supra nuclear palsy

Answer 18

Corticobasal syndrome begins as a MOVEMENT disorder, with a UNILATERAL ABSENCE of movements and muscle RIGIDITY with a TREMOR. It is a progressive neurological disorder that can also affect cognition.

Answer 19

There are 2 predominant types of multiple system atrophy 1) MSA-P - Predominant Parkinsonian features 2) MSA-C - Predominant Cerebellar features Shy-Drager syndrome is a type of multiple system atrophy. Features - parkinsonism - autonomic disturbance (atonic bladder, postural hypotension, erectile dysfunction) - cerebellar signs

Answer 20

ONLY lower motor signs | UM signs are not present

Answer 21

Only present with UMN signs and not LMN signs.

Answer 22

Amyotrophic lateral sclerosis (50% of patients) typically LMN signs in arms and UMN signs in legs in familial cases the gene responsible lies on chromosome 21 and codes for superoxide dismutase Primary lateral sclerosis UMN signs only Progressive muscular atrophy LMN signs only affects distal muscles before proximal carries best prognosis Progressive bulbar palsy palsy of the tongue, muscles of chewing/swallowing and facial muscles due to loss of function of brainstem motor nuclei carries worst prognosis (note: MND can initially present with limb weakness, it is progressive so wont resolve in a couple days)

Answer 23

Low CSF can occur due to spontaneous intracranial hypotension (not necessarily post-LP) and are classically WORSE ON STANDING and improve when lying flat Lack of associated features, all the other types of headache can be excluded. Results from a CSF leak. The leak is typically from the thoracic nerve root sleeves. Risk factors include connective tissue disorders such as Marfan's syndrome. Investigations: MRI with gadolinium: typically shows pachymeningeal enhancement Management: usually conservative if this fails an epidural blood patch may be tried

Answer 24

Dopamine receptor agonists are associated with the highest chance of inhibition disorders out of the antiparkinsonian medications

Answer 25

Usually combined with a decarboxylase inhibitor (e.g. carbidopa or benserazide) to PREVENT PERIPHERAL metabolism of levodopa to dopamine - reduced effectiveness with time (usually by 2 years) - unwanted effects: DYSKINESIA (involuntary writhing movements), 'on-off' effect, dry mouth, anorexia, palpitations, postural hypotension, psychosis, drowsiness - no use in neuroleptic induced parkinsonism - it is important not to acutely stop levodopa, for example if a patient is admitted to hospital. If a patient with Parkinson's disease cannot take levodopa orally, they can be given a dopamine agonist patch as rescue medication to prevent acute dystonia

Answer 26

An ACUTE, UNILATERAL, idiopathic, facial nerve PARALYSIS. The aetiology 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: - LOWER motor neurone facial nerve palsy - FOREHEAD AFFECTED - patients may also notice post-auricular PAIN (may precede paralysis), altered TASTE, DRY eyes, HYPERACUSIS Management: - PREDNISOLONE 1mg/kg for 10 days should be prescribed for patients within 72 hours of onset of Bell's palsy. Adding in aciclovir gives no additional benefit - EYE CARE is important - prescription of ARTIFICIAL TEARS and eye lubricants should be considered Prognosis: if untreated around 15% of patients have permanent moderate to severe weakness

Answer 27

This clinical syndrome occurs in patients who have had a SPINAL CORD INJURY at, or above T6 spinal level. Briefly, AFFERENT signals, most commonly triggered by faecal impaction or urinary retention (but many other triggers have been reported) cause a SYMPATHETIC SPINAL REFLEX via thoracolumbar outflow. The usual, centrally mediated, parasympathetic response however is prevented by the cord lesion. The result is an unbalanced physiological response, characterised by EXTREME HTN, FLUSHING and SWEATING above the level of the cord lesion, agitation, and in untreated cases severe consequences of extreme hypertension have been reported, e.g. haemorrhagic stroke. The combination of severe hypertension, flushing and sweating WITHOUT a congruent response in heart rate in the context of spinal cord injury indicates an autonomic dysreflexia and this is an important presentation to be aware of as it is one of the few unique medical emergencies that can occur to spinal cord injured patients. There is often a clear NOXIOUS STIMULUS which has triggered the episode (in this case a catheter change). - The cause of a dysreflexia is a discontinuity between the nociceptors (pain receptor - sensory neurone) on the viscera and the brain stem autonomic centres due to the cord injury. - All the pain receptors on smooth muscle on the abdominal and some pelvic viscera are carried by the sympathetics and the specific nerve which extends to the pelvis is the greater splanchnic nerve (which has its lowest innervation at T6 and so this is the lowest level at which autonomic dysreflexia can still occur). Management of autonomic dysreflexia involves removal/control of the stimulus and treatment of any life-threatening hypertension and/or bradycardia.

Answer 28

Autonomic dysreflexia can only occur if the spinal cord injury occurs above the T6 level

Answer 29

Tuberous sclerosis (TS) is a genetic condition of autosomal dominant inheritance. Like neurofibromatosis, the majority of features seen in TS are NEUROCUTANEOUS Cutaneous features: - DEPIGMENTED 'ash-leaf' SPOTS which fluoresce under UV light - roughened PATCHES of skin over LUMBAR spine (Shagreen patches) - adenoma sebaceum (angiofibromas): BUTTERFLY distribution over nose - fibromata beneath nails (subungual fibromata) - café-au-lait spots may be seen Neurological features: - developmental delay - epilepsy (infantile spasms or partial) - intellectual impairment Also: - retinal hamartomas: dense white areas on retina (phakomata) - rhabdomyomas of the heart - gliomatous changes can occur in the brain lesions - polycystic kidneys, renal angiomyolipomata - lymphangioleiomyomatosis: multiple lung cysts

Answer 30

Acute treatment: - first-line: offer combination therapy with an oral TRIPTAN and an NSAID or an oral TRIPTAN and PARACETAMOL - not effective or not tolerated offer a non-oral preparation of metoclopramide* or prochlorperazine and consider adding a non-oral NSAID or triptan Prophylaxis: prophylaxis should be given if patients are experiencing 2 OR MORE ATTACKS PER MONTH. - Either TOPIRAMATE or PROPRANOLOL Propranolol should be used in preference to topiramate in women of child bearing age as it may be teratogenic and it can reduce the effectiveness of hormonal contraceptives if these measures fail NICE recommend 'a course of up to 10 sessions of acupuncture over 5-8 weeks' or gabapentin NICE recommend: 'Advise people with migraine that riboflavin (400 mg once a day) may be effective in reducing migraine frequency and intensity for some people' for women with predictable menstrual migraine treatment NICE recommend either frovatriptan (2.5 mg twice a day) or zolmitriptan (2.5 mg twice or three times a day) as a type of 'mini-prophylaxis' pizotifen is no longer recommend. Adverse effects such as weight gain & drowsiness are common *caution should be exercised with young patients as acute dystonic reactions may develop

Answer 31

As a general rule 5-HT receptor agonists are used in the acute treatment of migraine whilst 5-HT receptor antagonists are used in prophylaxis.

Answer 32

SUBCUTANEOUS sumatriptan + 100% O2

Answer 33

verapamil.

Answer 34

- SENSORY changes - DEAFNESS or other ear problems - History of skin or oral lesions that could spread perineurally Pain only in the ophthalmic division of the trigeminal nerve (eye socket, forehead, and nose), or bilaterally - Optic neuritis - A family history of multiple sclerosis - Age of onset BEFORE 40 years

Answer 35

- CARBAMAZEPINE is first-line | - failure to respond to treatment or atypical features (e.g. < 50 years old) should prompt referral to neurology

Answer 36

is likely to represent a pseudoseizure

Answer 37

(pseudoseizure) Factors favouring pseudoseizures: - pelvic thrusting - family member with epilepsy - much more common in females - crying after seizure - don't occur when alone - GRADUAL onset - remains conscious during whole-body convulsions - no post-ictal state - can remember what happened Factors favouring true epileptic seizures - tongue biting - raised serum prolactin* Video telemetry is useful for differentiating

Answer 38

Reduced sensation in C8 dermatome - medial side of hand over little finger Weakness in the C8 myotome - flex of the vital interpahalngeal and maetacarpophalangeal joints

Answer 39

Below the vertebrae | - is the only one that does

Answer 40

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. 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, 3-5 Hz 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 drooling of saliva psychiatric features: depression is the most common feature (affects about 40%); dementia, psychosis and sleep disturbances may also occur impaired olfaction REM sleep behaviour disorder fatigue autonomic dysfunction: postural hypotension ``` 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 Diagnosis is usually clinical. 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).

Answer 41

Sensorineural hearing loss Hearing tests are routinely performed to assess this

Answer 42

SENSORINEURAL HEARING LOSS (most common) other neurological: epilepsy, paralysis infective: sepsis, intracerebral abscess pressure: brain herniation, hydrocephalus

Answer 43

autoantibodies to postsynaptic nicotinic acetylcholine receptors at the neuromuscular junction

Answer 44

There are significant implications in the administration of anaesthesia, in particular muscle paralysis.

Answer 45

SUXAMETHONIUM Suxamethonium is a depolarising NMBD - it acts by binding to Ach receptors and activating the receptor, at first causing muscle contraction, then paralysis - as they are desensitised and stop firing. Again, due to a decreased number of available receptors for Sux to bind to, MG patients are typically resistant to depolarising NMBDs and may require significantly higher doses. Therefore they should be used extremely cautiously, if at all, in myasthenic patients.

Answer 46

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

Answer 47

Myasthenia gravis is an autoimmune disorder resulting in INSUFFICIENT FUNCTIONING ACETYLCHOLINE RECEPTORS 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 anaemia, autoimmune thyroid disorders, rheumatoid, SLE thymic hyperplasia in 50-70% Investigations - single fibre electromyography: high sensitivity (92-100%) - CT thorax to exclude thymoma - CK normal - autoantibodies: around 85-90% of patients have antibodies to acetylcholine receptors. In the remaining patients, about about 40% are positive for anti-muscle-specific tyrosine kinase antibodies - Tensilon test: IV edrophonium reduces muscle weakness temporarily - not commonly used anymore due to the risk of cardiac arrhythmia ``` Management - long-acting ANTICHOLINESTERASE INHIBITORS inhibitors e.g. PYRIDOSTIGMINE immunosuppression: - prednisolone initially - thymectomy ```

Answer 48

Features: - eye is deviated 'DOWN AND OUT' - PTOSIS - pupil may be DILATED (sometimes called a 'surgical' third nerve palsy)

Answer 49

Causes: - diabetes mellitus - vasculitis e.g. temporal arteritis, SLE - false localizing sign* due to uncal herniation through tentorium if raised ICP - posterior communicating artery aneurysm - pupil dilated - often associated pain - cavernous sinus thrombosis - Weber's syndrome: ipsilateral third nerve palsy with contralateral hemiplegia -caused by midbrain strokes - other possible causes: amyloid, multiple sclerosis *this term is usually associated with sixth nerve palsies but it may be used for a variety of neurological presentations

Answer 50

autosomal dominant condition which usually affects both upper limbs Features - postural tremor (associated with a tremor present with sustained muscle tone): worse if arms outstretched - improved by alcohol and rest - most common cause of titubation (head tremor) - it can also affect the vocal cords Management - PROPANOLOL is first-line - primidone is sometimes used

Answer 51

Peripheral neuropathy may be divided into conditions which predominately cause a motor or sensory loss

Answer 52

Guillain-Barre syndrome porphyria lead poisoning hereditary sensorimotor neuropathies (HSMN) - Charcot-Marie-Tooth chronic inflammatory demyelinating polyneuropathy (CIDP) diphtheria

Answer 53

``` diabetes uraemia leprosy alcoholism vitamin B12 deficiency amyloidosis ```

Answer 54

- secondary to both direct toxic effects and reduced absorption of B vitamins - sensory symptoms typically present prior to motor symptoms

Answer 55

- subacute combined degeneration of spinal cord | - dorsal column usually affected first (joint position, vibration) prior to distal paraesthesia

Answer 56

'face, ear, taste, tear' - face: muscles of facial expression - ear: nerve to stapedius - taste: supplies anterior two-thirds of tongue - tear: parasympathetic fibres to lacrimal glands, also salivary glands

Answer 57

- sarcoidosis - Guillain-Barre syndrome - Lyme disease - bilateral acoustic neuromas (as in neurofibromatosis type 2) - as Bell's palsy is relatively common it accounts for up to 25% of cases of bilateral palsy, but this represents only 1% of total Bell's palsy cases

Answer 58

Lower motor neuron: - Bell's palsy - Ramsay-Hunt syndrome (due to herpes zoster) - acoustic neuroma - parotid tumours - HIV - multiple sclerosis (may also cause UMN palsy) - diabetes mellitus Upper motor neuron - stroke

Answer 59

upper motor neuron lesion 'spares' upper face i.e. forehead lower motor neuron lesion affects all facial muscles

Answer 60

3 branches: 1. greater petrosal nerve 2. nerve to stapedius 3. chorda tympani

Answer 61

Overview can cause cerebral infarction, much lesson common than arterial causes 50% of patients have isolated sagittal sinus thromboses - the remainder have coexistent lateral sinus thromboses and cavernous sinus thromboses Features headache (may be sudden onset) nausea & vomiting Sagittal sinus thrombosis may present with seizures and hemiplegia parasagittal biparietal or bifrontal haemorrhagic infarctions are sometimes seen Cavernous sinus thrombosis other causes of cavernous sinus syndrome: local infection (e.g. sinusitis), neoplasia, trauma periorbital oedema ophthalmoplegia: 6th nerve damage typically occurs before 3rd & 4th trigeminal nerve involvement may lead to hyperaesthesia of upper face and eye pain central retinal vein thrombosis Lateral sinus thrombosis 6th and 7th cranial nerve palsies

Answer 62

Causes finger abduction weakness It affects thumb adduction and finger abduction) and a loss of sensation over the medial epicondyle results in is Klumpke’s paralysis.

Answer 63

damage to T1 loss of intrinsic hand muscles due to traction

Answer 64

A WIDE-based gait with loss of heel to toe walking

Answer 65

Typically occur following cerebellar injury, the causes can be remembered by the mnemonic 'pastries' ``` P - Posterior fossa tumour A - Alcohol S - Multiple sclerosis T - Trauma R - Rare causes I - Inherited (e.g. Friedreich's ataxia) E - Epilepsy treatments S - Stroke ```

Answer 66

Prednisolone | Eye care - artificial tears and eye lubricants

Answer 67

Ankle S1-S2 Knee L3-L4 Biceps C5-C6 Triceps C7-C8

Answer 68

Presents with CAPE-like LOSS of PAIN and TEMPERATURE due to COMPRESSION of the SPINOTHALAMIC tract fibres decussating in the anterior white commissure of the spine

Answer 69

Syringomyelia is a condition whereby fluid filled cavities develop within the spinal cord. Pressure can increase resulting in compression of the spinal cord tracts. (The syrinx can extend to and damage the anterior horn cells, thereby resulting in lower motor neurone features. The spinothalamic tract axons decussate to the other side of the spinal cord via the anterior white commissure, and they are particularly susceptible to damage from the syrinx. Pain and temperature sensation are lost due to spinothalamic tract damage, and one side may be affected more than the other. Classically, the sensation loss is experienced in a shawl-like distribution over the arms, shoulders and upper body. Light touch, vibration and proprioception may also be affected as the syrinx enlarges into the dorsal columns.) Causes include: - a Chiari malformation: strong association - trauma - tumours - idiopathic The classical presentation of a syrinx is a patient who has a ‘CAPE-like’ (neck and arms) loss of SENSATION to but preservation of light touch, proprioception and vibration. Classic examples are of patients who accidentally burn their hands without realising. This is due to the crossing spinothalamic tracts in the ANTERIOR COMMISSURE of the spinal cord being the first tracts to be affected. Other symptoms and signs include SPASTIC WEAKNESS (predominantly of the upper limbs), paraesthesia, neuropathic pain, UPGOING PLANTARS and bowel and bladder dysfunction. Scoliosis will occur over a matter of years if the syrinx is not treated. It may cause a Horner’s syndrome due to compression of the sympathetic chain, but this is rare. Investigation requires a full SPINE MRI with contrast to exclude a tumour or tethered cord. A brain MRI is also needed to exclude a Chiari malformation. Treatment will be directed at treating the cause of the syrinx. In patients with a persistent or symptomatic syrinx, a shunt into the syrinx can be placed.

Answer 70

Wernicke's encephalopathy is a neuropsychiatric disorder caused by THIAMINE DEFICIENCY which is most commonly seen in ALCOHOLICS. Rarer causes include: persistent vomiting, stomach cancer, dietary deficiency. A classic triad of ophthalmoplegia/nystagmus, ataxia and confusion may occur. In Wernicke's encephalopathy petechial haemorrhages occur in a variety of structures in the brain including the mamillary bodies and ventricle walls Features - nystagmus (the most common ocular sign) - ophthalmoplegia - ataxia - confusion, altered GCS - peripheral sensory neuropathy Investigations - decreased red cell transketolase - MRI Treatment is with urgent REPLACEMENT OF THIAMINE

Answer 71

Korsakoff's syndrome may develop as well. This is termed Wernicke-Korsakoff syndrome and is characterised by the addition of antero- and retrograde AMNESIA and CONFABULATION in addition to the symptoms of Wernicke's (nystagmus, opthalmoplegis,ataxia)

Answer 72

Arnold-Chiari malformation describes the downward displacement, or HERNIATION, of the CEREBELLAR TONSILS through the FORAMEN MAGNUM. Malformations may be congenital or acquired through trauma. Features - non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow - headache - syringomyelia

Answer 73

Can affect both MOTOR and SENSORY PERIPHERAL nerves

Answer 74

Inherited myopathy. It is caused by progressive degeneration and weakness of SPECIFIC MUSCLE GROUPS. - Most patients lose the ability to walk by 12 years of age and require ventilatory support by the age of 25. - Sensation is intact in these patients.

Answer 75

Term used for OSTEOARTHRITIS of the SPINE and can result in COMPRESSION of the SPINAL CORD. This is more likely to result in LMN signs at the level of the compression (ie. upper limb if the lesion is below C5) with UMN signs below (in the lower limb). Patients usually complain of neck pain and stiffness.

Answer 76

- Ataxic Gait - due to degeneration of the dorsal columns - Mixed UMN and LMN signs - due to degeneration of lateral motor tracts and peripheral nerves - Subacute history - occurs over months - Often notice sensory symptoms before weakness

Answer 77

Term which encompasses Charcot-Marie-Tooth disease (also known as peroneal muscular atrophy).

Answer 78

- autosomal dominant - due to defect in PMP-22 gene (which codes for myelin) - features often start at puberty - motor symptoms predominate - distal muscle wasting, pes cavus, clawed toes - foot drop, leg weakness often first features

Answer 79

UNILATERAL Parkinsonism + severe AUTONOMIC DISTURBANCE (postural hypotension/erectile dysfunction). - more likely to be multiple system atrophy than Parkinson's disease

Answer 80

The presence of ocular pathology

Answer 81

contains vitamins B and C. It does not vitamin D.

Answer 82

Vitamin B1, also called thiamine, is essential for GLIAL cells of the nervous system, as well as other bodily systems. Deficiency can cause Wernicke's encephalopathy and if left untreated can lead to irreversible Korsakoff's syndrome.

Answer 83

Pernicious anaemia

Answer 84

Tuberous sclerosis | Adenoma sebaceum is a butterfly distribution over nose

Answer 85

Indicates disease near the dorsal column nuclei of the cervical cord. It is seen in MS, subacute combined degeneration of the cord and in cervical stenosis It is: - paraesthesiae in limbs on neck flexion

Answer 86

Results from COMPRESSION of the CAUDA EQUINA and classically includes: - leg weakness - saddle anaesthesia - sphincter disturbance Usually ACUTE syndrome with progressive signs. (does not cause leg stiffness)

Answer 87

Degenerative cervical myelopathy (DCM) has a number of risk factors, which include smoking due to its effects on the intervertebral discs, genetics and occupation - those exposing patients to high axial loading. The presentation of DCM is very variable. Early symptoms are often subtle and can vary in severity day to day, making the disease difficult to detect initially. However as a progressive condition, worsening, deteriorating or new symptoms should be a warning sign. DCM symptoms can include any combination of: - Pain (affecting the neck, upper or lower limbs) - Loss of motor function (loss of digital dexterity, preventing simple tasks such as holding a fork or doing up their shirt buttons, arm or leg weakness/stiffness leading to impaired gait and imbalance - Loss of sensory function causing numbness - Loss of autonomic function (urinary or faecal incontinence and/or impotence) - these can occur and do not necessarily suggest cauda equina syndrome in the absence of other hallmarks of that condition - HOFFMAN'S sign: is a reflex test to assess for cervical myelopathy. It is performed by gently flicking one finger on a patient's hand. A positive test results in reflex twitching of the other fingers on the same hand in response to the flick. The most common symptoms at presentation of DCM are unknown, but in one series 50% of patients were initially incorrectly diagnosed and sometimes treated for carpal tunnel syndrome. An MRI of the cervical spine is the gold standard test where cervical myelopathy is suspected. It may reveal disc degeneration and ligament hypertrophy, with accompanying cord signal change. All patients with degenerative cervical myelopathy should be urgently referred for assessment by SPECIALIST SPINAL SERVICES (neurosurgery or orthopaedic spinal surgery). This is due to the importance of early treatment. The timing of surgery is important, as any existing spinal cord damage can be permanent. Early treatment (within 6 months of diagnosis) offers the best chance of a full recovery but at present, most patients are presenting too late. Currently, DECOMPRESSIVE SURGERY surgery is the only effective treatment. It has been shown to prevent disease progression. Close observation is an option for mild stable disease, but anything progressive or more severe requires surgery to prevent further deterioration. Physiotherapy should only be initiated by specialist services, as manipulation can cause more spinal cord damage.

Answer 88

A - At least 5 attacks fulfilling criteria B-D B - Headache attacks lasting 4-72 hours (untreated or unsuccessfully treated) C - Headache has at least two of the following characteristics: 1. unilateral location 2. pulsating quality (i.e., varying with the heartbeat) 3. moderate or severe pain intensity 4. aggravation by or causing avoidance of routine physical activity (e.g., walking or climbing stairs) D - During headache at least one of the following: 1. nausea and/or vomiting* 2. photophobia and phonophobia E - Not attributed to another disorder (history and examination do not suggest a secondary headache disorder or, if they do, it is ruled out by appropriate investigations or headache attacks do not occur for the first time in close temporal relation to the other disorder)

Answer 89

to compensate for a foot drop

Answer 90

common peroneal nerve lesion should be suspected

Answer 91

due to peripheral neuropathy.

Answer 92

to compensate for a foot drop

Answer 93

common peroneal nerve lesion should be suspected

Answer 94

due to peripheral neuropathy.

Answer 95

All patient must not drive and must inform the DVLA - first unprovoked/isolated seizure: 6 months off if there are no relevant structural abnormalities on brain imaging and no definite epileptiform activity on EEG. If these conditions are not met then this is increased to 12 months - for patients with established epilepsy or those with multiple unprovoked seizures: → may qualify for a driving licence if they have been free from any seizure for 12 months → if there have been no seizures for 5 years (with medication if necessary) a ’til 70 licence is usually restored withdrawawl of epilepsy medication: should not drive whilst anti-epilepsy medication is being withdrawn and for 6 months after the last dose

Answer 96

- simple faint: no restriction - single episode, explained and treated: 4 weeks off - single episode, unexplained: 6 months off - two or more episodes: 12 months off

Answer 97

Multiple system atrophy

Answer 98

Parkinsonism and visual hallucinations

Answer 99

Parkinsonism and ophthalmoplegia

Answer 100

Inferior quadranopia

Answer 101

Superior quadranopia

Answer 102

Topiramate OR | propanolol

Answer 103

Immune mediated DEMYELINATION of the peripheral nervous system often triggered by an infection (classically Campylobacter jejuni) Pathogenesis: - cross reaction of antibodies with gangliosides in the peripheral nervous system - correlation between anti-ganglioside antibody (e.g. anti-GM1) and clinical features has been demonstrated - anti-GM1 antibodies in 25% of patients

Answer 104

Variant of Guillain-Barre syndrome - associated with ophthalmoplegia, areflexia and ataxia. The eye muscles are typically affected first - usually presents as a descending paralysis rather than ascending as seen in other forms of Guillain-Barre syndrome - anti-GQ1b antibodies are present in 90% of cases

Answer 105

Is PROGRESSIVE WEAKNESS of all FOUR limbs. The weakness is classically ASCENDING i.e. the lower extremities are affected first, however it tends to affect proximal muscles earlier than the distal ones. SENSORY symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs. Some patients experience BACK PAIN in the initial stages of the illness. Other features: - there may be a history of gastroenteritis - areflexia - cranial nerve involvement e.g. diplopia - autonomic involvement: e.g. urinary retention, diarrhoea Less common findings - papilloedema: thought to be secondary to reduced CSF resorption (The classical presentation of GBS is rapid, progressive onset of muscle weakness and changes in sensation starting peripherally. Important complications to be aware of, which imply more severe disease, are bulbar symptoms (eg swallowing problems), respiratory difficulties (due to respiratory muscle weakness) and autonomic instability)

Answer 106

LUMBAR PUNCTURE - rise in protein with a normal white blood cell count (albuminocytologic dissociation) Nerve condution studies may be performed

Answer 107

- Onset 4-8 yrs - duration few-30 secs; no warning, quick recovery; often many per day - EEG: 3Hz generalised, symmetrical - sodium valproate, ethosuximide - good prognosis: 90-95% become seizure free in adolescence

Answer 108

Neurofibromatosis - 1

Answer 109

Degenerative cervical myelopathy Should be suspected in elderly patients presenting with limb neurology.

Answer 110

?brain abscess

Answer 111

Cavernous sinus thrombosis usually presents with: - unilateral facial oedema, - photophobia - proptosis - palsies of the cranial nerves which pass through it (III, IV, V, VI).

Answer 112

CNS abscesses may result from a number of causes including, extension of sepsis from MIDDLE EAR or SINUSES, TRAUMA or surgery to the scalp, penetrating HEAD INJURIES and embolic events from endocarditis - The presenting symptoms will depend upon the site of the abscess (those in critical areas e.g.motor cortex) will present earlier. Abscesses have a considerable mass effect in the brain and raised intra cranial pressure is common. - Although FEVER, HEADACHE and FOCAL NEUROLOGY are highly suggestive of a brain abscess the absence of one or more of these does not exclude the diagnosis, fever may be absent and even if present, is usually not the swinging pyrexia seen with abscesses at other sites. - Assessment of the patient includes imaging with CT scanning. - Treatment is usually SURGICAL, a craniotomy is performed and the abscess cavity debrided. The abscess may reform because the head is closed following abscess drainage.

Answer 113

Pathology either within the car viral spinal cord to brain. if brain - more likely to cause unilateral problems

Answer 114

i) review by an anaesthetist - as there is risk of an unsecured airway ii) intubation and ventilation

Answer 115

Motor response: 6. Obeys commands 5. Localises to pain 4. Withdraws from pain 3. Abnormal flexion to pain (decorticate posture) 2. Extending to pain 1. None Verbal response: 5. Orientated 4. Confused 3. Words 2. Sounds 1. None Eye opening: 4. Spontaneous 3. To speech 2. To pain 1. None

Answer 116

If a patient is nil by mouth and needs antiparkinsonian medication, the best option is a DOPAMINE AGONIST TRANSDERMAL PATCH , which will prevent a life-threatening dystonic (person's muscles contract uncontrollably.) withdrawal.

Answer 117

Acoustic neuroma

Answer 118

A combination of VERTIGO, HEARING LOSS, TINNITUS and an ABSENT CORNEAL REFLEX. Features can be predicted by the affected cranial nerves: - cranial nerve VIII: vertigo, unilateral sensorineural hearing loss, unilateral tinnitus - cranial nerve V: absent corneal reflex - cranial nerve VII: facial palsy Bilateral vestibular schwannomas are seen in neurofibromatosis type 2. Patients with a suspected vestibular schwannoma should be referred urgently to ENT. It should be noted though that the tumours are often slow growing, benign and often observed initially. MRI of the cerebellopontine angle is the investigation of choice. Audiometry is also important as only 5% of patients will have a normal audiogram. Management is with either surgery, radiotherapy or observation.

Answer 119

(means visual field defect to the left) | RIGHT optic tract

Answer 120

Usually affects the MEDULLA OBLONGATA and is often a terminal event in an unconscious patient resulting in asystolic cardio-respiratory arrest

Answer 121

Raised ICP can cause a third nerve palsy due to herniation

Answer 122

Acute Sensory level and upper motor neuron signs below the level affected It can occur in patients with multiple sclerosis or Devics disease (neuromyelitis optica). These patients tend to also have features such as optic neuritis.

Answer 123

The sciatic nerve divides into the tibial and common peroneal nerves. Injury often occurs at the neck of the fibula The most characteristic feature of a common peroneal nerve lesion is FOOT DROP. Other features include: - WEAKNESS of foot DORSIFLEXION - weakness of foot EVERSION - weakness of extensor hallucis longus - sensory loss over the dorsum of the foot and the lower lateral part of the leg - wasting of the anterior tibial and peroneal muscles

Answer 124

Most commonly caused by cavernous sinus TUMOUR

Answer 125

``` pain (on attempt to move eye?) ophthalmoplegia proptosis trigeminal nerve lesion (opthalmic branch) Horner's syndrome. ```

Answer 126

``` Oculomotor nerve Trochlear nerve Ophthalmic nerve Maxillary nerbe Internal carotid artery (and sympathetic plexus) Abducens nerve ```

Answer 127

``` Grade 0 No muscle movement Grade 1 Trace of contraction Grade 2 Movement at the joint with gravity eliminated (e.g. raising knees when on his side) Grade 3 Movement against gravity, but not against added resistance Grade 4 Movement against an external resistance with reduced strength Grade 5 Normal strength ```

Answer 128

Rare but dangerous condition seen in PATIENTS TAKING ANTIPSYCHOTIC MEDICATION. Can also occur with atypical antipsychotics. May also occur with dopaminergic drugs (such as levodopa) for Parkinson's disease, usually when the drug is SUDDENLY STOPPED or DOSE REDUCED. The pathophysiology is unknown but one theory is that the dopamine blockade induced by antipsychotics triggers massive glutamate release and subsequent neurotoxicity and muscle damage. It occurs WITHIN HOURS TO DAYS OF STARTING AN ANTIPSYCHOTIC (antipsychotics are also known as neuroleptics, hence the name) and the typical features are: - pyrexia - muscle rigidity - autonomic lability: typical features include hypertension, tachycardia and tachypnoea - agitated delirium with confusion A RAISED CK is present in most cases. ACUTE KIDNEY INJURY (secondary to rhabdomyolysis) may develop in severe cases. A LEUKOCYTOSIS may also be seen (above the average of WCC) Management: - stop antipsychotic - patients should be transferred to a medical ward if they are on a psychiatric ward and often they are nursed in intensive care units - IV fluids to prevent renal failure - dantrolene* may be useful in selected cases - bromocriptine, dopamine agonist, may also be used *thought to work by decreasing excitation-contraction coupling in skeletal muscle by binding to the ryanodine receptor, and decreasing the release of calcium from the sarcoplasmic reticulum

Answer 129

FINE MOTOR function in BOTH UPPER arms

Answer 130

UMN sign | REFLEX test to investigate CORTICOSPINAL TRACT LESIONS

Answer 131

Differentiates between organic and non-organic LOWER LEG weakness

Answer 132

Overview: caused by LATERAL HEMISECTION of the spinal cord Features: ipsilateral weakness below lesion (corticospinal tract) ipsilateral loss of proprioception and vibration and touch sensation (dorsal column) contralateral loss of pain and temperature sensation (spinothalamic tract)

Answer 133

Overview: - a cause of horizontal disconjugate eye movement - due to a lesion in the medial longitudinal fasciculus (MLF), which connects the IIIrd, IVth and VIth cranial nuclei - a tract that allows conjugate eye movement. Features: - impaired ADduction of the eye on the same side as the lesion - horizontal nystagmus of the abducting eye on the contralateral side Causes: - multiple sclerosis - vascular disease

Answer 134

RAPD is a defect in the direct response to light. It is due to damage in OPTIC NERVE or severe retinal disease. If an optic nerve lesion is present the affected pupil will not constrict to light when light is shone in the that pupil during the swinging flashlight test. However, it will constrict if light is shone in the other eye (consensual response). The most likely cause for this is an OPTIC NEURITIS. Other causes include ischemic optic disease or retinal disease, severe glaucoma causing trauma to optic nerve and direct optic nerve damage (trauma, radiation, tumor).

Answer 135

- Parkinson's disease - drug-induced e.g. antipsychotics, metoclopramide - 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

Answer 136

DOMPERIDONE does not cross the blood-brain barrier and therefore does not cause extra-pyramidal side-effects. (Cyclizine is an antihistamine which, like prochlorperazine, may exacerbate Parkinson's disease.)

Answer 137

- doesn't affect external ocular muscles - no cerebellar signs - abdominal reflexes are usually preserved and sphincter dysfunction if present is a late feature

Answer 138

Subarachnoid haemorrhage

Answer 139

- Vomiting more than once with no other cause. - New neurological deficit (motor or sensory). - Reduction in conscious level (as measured by the Glasgow coma score). - Valsalva (associated with coughing or sneezing) or positional headaches. - Progressive headache with a fever. NICE suggest that if two or more of the ‘red-flag’ criteria are present then an urgent CT scan should be performed

Answer 140

- compromised immunity, caused, for example, by HIV or immunosuppressive drugs - age under 20 years and a history of malignancy - a history of malignancy known to metastasis to the brain - vomiting without other obvious cause - worsening headache with fever - sudden-onset headache reaching maximum intensity within 5 minutes - 'thunderclap' - new-onset neurological deficit - new-onset cognitive dysfunction - change in personality - impaired level of consciousness - recent (typically within the past 3 months) head trauma - headache triggered by cough, valsalva (trying to breathe out with nose and mouth blocked), sneeze or exercise - orthostatic headache (headache that changes with posture) - symptoms suggestive of giant cell arteritis or acute narrow-angle glaucoma - a substantial change in the characteristics of their headache

Answer 141

Damage at wrist - 'claw hand' - hyperextension of the metacarpophalangeal joints and flexion at the distal and proximal interphalangeal joints of the 4th and 5th digits - wasting and paralysis of intrinsic hand muscles (except lateral two lumbricals) - wasting and paralysis of hypothenar muscles - sensory loss to the medial 1 1/2 fingers (palmar and dorsal aspects) Damage at elbow - as above (however, ulnar paradox - clawing is more severe in distal lesions) - radial deviation of wrist

Answer 142

sodium valproate second line: lamotrigine, carbamazepine

Answer 143

sodium valproate or ethosuximide sodium valproate particularly effective if co-existent tonic-clonic seizures in primary generalised epilepsy (NOTE: *carbamazepine may exacerbate absence seizures)

Answer 144

sodium valproate second line: clonazepam, lamotrigine (NOTE: carbamazepine may exacerbate myoclonic seizures)

Answer 145

first line: carbamazepine or lamotrigine | second line: levetiracetam, oxcarbazepine or sodium valproate

Answer 146

Sodium valproate is considered the first line treatment for patients with generalised seizures with carbamazepine used for focal seizures.

Answer 147

HIGH DOSE STEROIDS (e.g. oral or IV methylprednisolone) may be given for 5 days to shorten the length of an acute relapse. It should be noted that steroids shorten the duration of a relapse and do not alter the degree of recovery (i.e. whether a patient returns to baseline function)

Answer 148

- BETA-INTERFERON has been shown to reduce the relapse rate. Certain criteria have to be met before it is used: relapsing-remitting disease + 2 relapses in past 2 years + able to walk 100m unaided secondary progressive disease + 2 relapses in past 2 years + able to walk 10m (aided or unaided) reduces number of relapses and MRI changes, however doesn't reduce overall disability Other drugs used in the management of multiple sclerosis include: - glatiramer acetate: immunomodulating drug - acts as an 'immune decoy' - natalizumab: a recombinant monoclonal antibody that antagonises Alpha-4 Beta-1-integrin found on the surface of leucocytes, thus inhibiting migration of leucocytes across the endothelium across the blood-brain barrier - fingolimod: sphingosine 1-phosphate receptor modulator, prevents lymphocytes from leaving lymph nodes. An oral formulation is available

Answer 149

once other problems (e.g. anaemia, thyroid or depression) have been excluded NICE recommend a trial of amantadine other options include mindfulness training and CBT

Answer 150

baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine physiotherapy is important cannabis and botox are undergoing evalulation

Answer 151

- may take the form of urgency, incontinence, overflow etc - guidelines stress the importance of getting an ultrasound first to assess bladder emptying - anticholinergics may worsen symptoms in some patients - if significant residual volume → intermittent self-catheterisation - if no significant residual volume → anticholinergics may improve urinary frequency

Answer 152

gabapentin is first-line

Answer 153

- non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow - headache - syringomyelia

Answer 154

is a condition classically seen in young, overweight females. ``` Features: headache blurred vision papilloedema (usually present) enlarged blind spot sixth nerve palsy may be present ``` ``` Risk factors: obesity female sex pregnancy drugs*: oral contraceptive pill, steroids, tetracycline antibodies, vitamin A, lithium, isotretinoin, levothyroxine, cimetidine ``` 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 *if intracranial hypertension is thought to occur secondary to a known causes (e.g. Medication) then it is of course not idiopathic

Answer 155

- L5 radiculopathy - sciatic nerve lesion - common peroneal nerve lesion - superficial or deep peroneal nerve lesion - other possible includes central nerve lesions (e.g. stroke) but other features are usually present A common peroneal nerve lesion is the most common cause. This is often secondary to compression at the neck of the fibula. This may be caused by certain positions such as leg crossing, squatting or kneeling. Prolonged confinement, recent weight loss, Baker's cysts and plaster casts to the lower leg are also known to be precipitating factors.

Answer 156

- smoking - previous infectious mononucleosis - glandular fever - genetics - hypovitaminosis D

Answer 157

Lesion at brachial trunks C8-T1 Classically there is weakness of the hand intrinsic muscles. Involvement of T1 may cause a Horner's syndrome - as the nerves leave and join the sympathetic chain at T1 It occurs as a result of traction injuries or during delivery.

Answer 158

Lesion at brachial trunks Brachial Trunks C5-6

Answer 159

speech non-fluent, COMPREHENSION NORMAL, repetition good

Answer 160

left middle cerebral artery

Answer 161

Cerebral function usually remains normal in a patient with meningitis while in encephalitis, however, abnormalities in brain function are a differentiating feature, including altered mental status.

Answer 162

Features: fever, headache, psychiatric symptoms, seizures, vomiting focal features e.g. aphasia peripheral lesions (e.g. cold sores) have no relation to presence of HSV encephalitis Pathophysiology: HSV-1 responsible for 95% of cases in adults typically affects temporal and inferior frontal lobes Investigation: - CSF: lymphocytosis, elevated protein - PCR for HSV - CT: medial temporal and inferior frontal changes (e.g. petechial haemorrhages) - normal in one-third of patients - MRI is better - EEG pattern: lateralised periodic discharges at 2 Hz

Answer 163

Tongue fasciculations

Answer 164

CHOCOLATE ``` Chocolate Hangovers Orgasms Cheese Caffeine The oral contraceptive pill Lie-ins Alcohol Travel Exercise ```

Answer 165

raised CK Leukocytosis Patients may have high potassium, not usually low, due to the muscle damage seen.

Answer 166

superior temporal gyrus patients being able to produce fluent speech, but comprehension and repetition is impaired.

Answer 167

Due to a lesion of the superior temporal gyrus. It is typically supplied by the inferior division of the left MCA This area 'forms' the speech before 'sending it' to Broca's area. Lesions result in sentences that make NO SENSE, word substitution and neologisms but speech remains fluent Comprehension is impaired

Answer 168

Due to a lesion of the inferior frontal gyrus. It is typically supplied by the superior division of the left MCA Speech is non-fluent, laboured, and halting Comprehension is normal

Answer 169

Classically due to a stroke affecting the arcuate fasiculus - the connection between Wernicke's and Broca's area Speech is fluent but repetition is poor. Aware of the errors they are making Comprehension is normal

Answer 170

Large lesion affecting all 3 of the above areas resulting in severe expressive and receptive aphasia

Answer 171

Topiramate or propanolol

Answer 172

Amitriptyline, Duloxetine, gabapentin or pregabalin if the first line drug treatment doesn't work try one of the other 3 drugs

Answer 173

Topical capsaicin

Answer 174

Unilateral cerebellar lesions cause ipsilateral signs. Useful and well-known mnemonic to remember symptoms of cerebellar disease is DANISH: D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear 'Drunk' A - Ataxia (limb, truncal) N - Nystamus (horizontal = ipsilateral hemisphere) I - Intention tremour S - Slurred staccato speech, Scanning dysarthria H - Hypotonia Causes - Friedreich's ataxia, ataxic telangiectasia - neoplastic: cerebellar haemangioma - stroke - alcohol - multiple sclerosis - hypothyroidism - drugs: phenytoin, lead poisoning - paraneoplastic e.g. secondary to lung cancer

Answer 175

Patient's with multiple sclerosis (MS) may present with non-specific features, for example around 75% of patients have significant lethargy. Diagnosis can be made on the basis of two or more relapses and either objective clinical evidence of two or more lesions or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse. Visual - optic neuritis: common presenting feature - optic atrophy - Uhthoff's phenomenon: worsening of vision following rise in body temperature - internuclear ophthalmoplegia Sensory - pins/needles - numbness - trigeminal neuralgia - Lhermitte's syndrome: paraesthesiae in limbs on neck flexion Motor - spastic weakness: most commonly seen in the legs Cerebellar - ataxia: more often seen during an acute relapse than as a presenting symptom - tremor Others - urinary incontinence - sexual dysfunction - intellectual deterioration

Answer 176

a Colle's fracture, which is a fracture of the distal radius that sometimes involves the ulna. The median nerve is most commonly affected. As the median nerve supplies the motor supply for the radial half of the fingers in the hand, it explains why he has lost the ability to abduct his thumb.

Answer 177

Thrombolysis with alteplase should only be given if: - it is administered within 4.5 hours of onset of stroke symptoms (unless as part of a clinical trial) - haemorrhage has been definitively excluded (i.e. Imaging has been performed)

Answer 178

Unilateral cerebellar lesions cause ipsilateral signs. Useful and well-known mnemonic to remember symptoms of cerebellar disease is DANISH: D - Dysdiadochokinesia, Dysmetria (past-pointing), patients may appear 'Drunk' A - Ataxia (limb, truncal) N - Nystamus (horizontal = ipsilateral hemisphere) I - Intention tremour S - Slurred staccato speech, Scanning dysarthria H - Hypotonia Causes - Friedreich's ataxia, ataxic telangiectasia - neoplastic: cerebellar haemangioma - stroke - alcohol - multiple sclerosis - hypothyroidism - drugs: phenytoin, lead poisoning - paraneoplastic e.g. secondary to lung cancer

Answer 179

Patient's with multiple sclerosis (MS) may present with non-specific features, for example around 75% of patients have significant lethargy. Diagnosis can be made on the basis of two or more relapses and either objective clinical evidence of two or more lesions or objective clinical evidence of one lesion together with reasonable historical evidence of a previous relapse. Visual - optic neuritis: common presenting feature - optic atrophy - Uhthoff's phenomenon: worsening of vision following rise in body temperature - internuclear ophthalmoplegia Sensory - pins/needles - numbness - trigeminal neuralgia - Lhermitte's syndrome: paraesthesiae in limbs on neck flexion Motor - spastic weakness: most commonly seen in the legs Cerebellar - ataxia: more often seen during an acute relapse than as a presenting symptom - tremor Others - urinary incontinence - sexual dysfunction - intellectual deterioration

Answer 180

a Colle's fracture, which is a fracture of the distal radius that sometimes involves the ulna. The median nerve is most commonly affected. As the median nerve supplies the motor supply for the radial half of the fingers in the hand, it explains why he has lost the ability to abduct his thumb.

Answer 181

Thrombolysis with alteplase should only be given if: - it is administered within 4.5 hours of onset of stroke symptoms (unless as part of a clinical trial) - haemorrhage has been definitively excluded (i.e. Imaging has been performed)

Answer 182

Guillain–Barré syndrome (GBS)

Answer 183

confusion, ataxia, ophthalmoplegia This is thought to be due to a deficiency in thiamine and often seen in alcoholics.

Answer 184

Acetazolamide

Answer 185

The '4Ds' can be useful in remembering causes of postural hypotension with compensatory tachycardia. - Deconditioning. - Dysfunctional heart: aortic stenosis. - Dehydration: disease (acute illness, adrenal insufficiency), dialysis, drugs (diuretics, narcotics). - Drugs: anti-anginals, anti-parkinsonian medications (levodopa), antidepressants, antipsychotics, anti–benign prostatic hyperplasia drugs (tamsulosin). Parkinson's can lead to postural hypotension due to autonomic failure, but no compensatory tachycardia on standing.

Answer 186

The tremor seen in Parkinson's disease is often asymmetric , occurs with rest, and improves with voluntary activity

Answer 187

Riluzole - prevents stimulation of glutamate receptors - used mainly in amyotrophic lateral sclerosis - prolongs life by about 3 months

Answer 188

sensory symptoms tend to be mild (e.g. distal paraesthesia) with very few sensory signs (or none)

Answer 189

NORMAL motor conduction on nerve conduction studies

Answer 190

In patients with focal seizures A focal weakness, typically of the frontal lobe (motor strip), may occur following a focal-onset seizure - this is called Todd's paresis.

Answer 191

seizures may be provoked by hyperventilation or stress

Answer 192

Absence seizures (petit mal) are a form of generalised epilepsy that is mostly seen in children. The typical age of onset of 3-10 years old and girls are affected twice as commonly as boys Features - absences last a few seconds and are associated with a quick recovery - seizures may be provoked by hyperventilation or stress - the child is usually unaware of the seizure - they may occur many times a day - EEG: bilateral, symmetrical 3Hz spike and wave pattern Management - sodium valproate and ethosuximide are first-line treatment - good prognosis - 90-95% become seizure free in adolescence

Answer 193

if patients have migraine with aura then the COC is absolutely contraindicated due to an increased risk of stroke

Answer 194

Pregnant women

Answer 195

middle finger +palm of hand

Answer 196

lower motor neurone facial nerve palsy the movement of the forehead/ eyebrow-raising is therefore affected

Answer 197

A classical triad of features is seen - urinary incontinence - dementia and bradyphrenia - gait abnormality (may be similar to Parkinson's disease) It is thought around 60% of patients will have all 3 features at the time of diagnosis. Symptoms typically develop over a few months.

Answer 198

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. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis. Imaging - hydrocephalus with an enlarged fourth ventricle - in addition to the ventriculomegaly there is typically an absence of substantial sulcal atrophy Management - ventriculoperitoneal shunting - around 10% of patients who have shunts experience significant complications such as seizures, infection and intracerebral haemorrhages

Answer 199

Association with small cell lung cancer and to a lesser extent breast and ovarian cancer.

Answer 200

Lambert-Eaton myasthenic syndrome is seen in association with small cell lung cancer and to a lesser extent breast and ovarian cancer. It may also occur independently as an autoimmune disorder. Lambert-Eaton myasthenic syndrome is caused by an antibody directed against presynaptic voltage-gated calcium channel in the peripheral nervous system. Features: - repeated muscle contractions lead to increased muscle strength (in contrast to myasthenia gravis) - -in reality, this is seen in only 50% of patients and following prolonged muscle use muscle strength will eventually decrease - limb-girdle weakness (affects lower limbs first)- ( can manifest as a waddling gait) - hyporeflexia - autonomic symptoms: dry mouth, impotence, difficulty micturating - ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis) EMG incremental response to repetitive electrical stimulation Management treatment of underlying cancer immunosuppression, for example with prednisolone and/or azathioprine 3,4-diaminopyridine is currently being trialled works by blocking potassium channel efflux in the nerve terminal so that the action potential duration is increased. Calcium channels can then be open for a longer time and allow greater acetylcholine release to the stimulate muscle at the end plate intravenous immunoglobulin therapy and plasma exchange may be beneficial

Answer 201

Decompressive surgery should be by specialist spinal services (neurosurgery or orthopaedic spinal surgery).

Answer 202

Seen in patients taking antipsychotic medication hyperthermia muscle rigidity autonomic instability altered mental status

Answer 203

Overview - supplies superior oblique (depresses eye, moves inward) Features - vertical diplopia - -classically noticed when reading a book or going downstairs - subjective tilting of objects (torsional diplopia) - the patient may develop a head tilt, which they may or may not be aware of - when looking straight ahead, the affected eye appears to deviate upwards and is rotated outwards

Answer 204

Cerebellar VERMIS lesions cause gait ataxia Cerebellar HEMISPHERE lesions cause peripheral ('finger-nose ataxia')

Answer 205

Common peroneal nerve lesion

Answer 206

Sudden onset severe OCCIPITAL headache , like being 'kicked in the back of the head' The pain is worse when bends neck.

Answer 207

MRI of the cervical spine

Answer 208

- Refer to epilepsy clinic only Anti-epileptic drug treatment should only be started before specialist review in exceptional circumstances including: 1. Seizure activity observed on EEG 2. Presence of a neurological deficit 3. Presence of a structural brain abnormality 4. Patient, parent or carer considers the risk of a further seizure to be unacceptable

Answer 209

More likely due to multisystem atrophy rather than idiopathic Parkinson's. Idiopathic Parkinsons disease can cause autonomic instability, but this is usually a very late development. If it rapidly follows a diagnosis of Parkinson's disease (for example, two weeks, as in the question), it is more likely due to multisystem atrophy rather than idiopathic Parkinson's. 5% of patients with idiopathic Parkinson's disease have postural hypotension, compared to 75% of patients with multisystem atrophy, making the latter more likely.

Answer 210

Neurofibromatosis type 2 | vestibular schwannomas (previously known as acoustic neuromas

Answer 211

duplex ultrasound Would hear a carotid bruit

Answer 212

typically benign tumours that develop from the dura mater of the meninges They are extra-axial lesions, meaning they do not arise from the brain parenchyma. They do not invade the brain substance, but rather cause symptoms by compression.

Answer 213

Restless legs syndrome (RLS) is a syndrome of spontaneous, continuous lower limb movements that may be associated with paraesthesia. It is extremely common, affecting between 2-10% of the general population. 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 - there is a 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 anaemia may be appropriate Management - simple measures: walking, stretching, massaging affected limbs - treat any iron deficiency - dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole) - benzodiazepines - gabapentin

Answer 214

pituitary tumour- lower quadrant defect | craniopharyngioma- upper quadrant defect

Answer 215

Cataplexy describes the sudden and transient loss of muscular tone caused by strong emotion (e.g. laughter, being frightened). Around two-thirds of patients with narcolepsy have cataplexy. Features range from buckling knees to collaps

Answer 216

worsens after exercise | Weakness in Lambert Eaton improves after exercise

Answer 217

ACh receptor antibodies

Answer 218

``` Refeeding syndrome describes the metabolic abnormalities which occur on feeding a person following a period of starvation. The metabolic consequences include: Hypophosphataemia Hypokalaemia Hypomagnesaemia Abnormal fluid balance ``` These abnormalities can lead to organ failure. High risk for re-feeding problems If one or more of the following: - BMI < 16 kg/m2 - Unintentional weight loss >15% over 3-6 months - Little nutritional intake > 10 days - Hypokalaemia, Hypophosphataemia or hypomagnesaemia prior to feeding (unless high) If two or more of the following: BMI < 18.5 kg/m2 - Unintentional weight loss > 10% over 3-6 months - Little nutritional intake > 5 days - History of: alcohol abuse, drug therapy including insulin, chemotherapy, diuretics and antacids Prescription - Start at up to 10 kcal/kg/day increasing to full needs over 4-7 days - Start immediately before and during feeding: oral thiamine 200-300mg/day, vitamin B co strong 1 tds and supplements - Give K+ (2-4 mmol/kg/day), phosphate (0.3-0.6 mmol/kg/day), magnesium (0.2-0.4 mmol/kg/day)

Answer 219

Verapamil | Sumatriptan is used as an ACUTE rescue therapy (along with high-flow oxygen)

Answer 220

Guillain Barre Syndrome

Answer 221

It often occurs as a complication secondary to chronic hypertension. Patients often present with reduces Glasgow coma score, quadriplegia, miosis, and absent horizontal eye movements.

Answer 222

Prolactin - in psuedoseizures Elevated serum prolactin 10 to 20 minutes after an episode can be used to differentiate a general tonic-clonic/partial seizure from a non-epileptic pseudo seizure.

Answer 223

radial nerve

Answer 224

The Recognition of Stroke in the Emergency Room (ROSIER) scale is effective in the initial differentiation of acute stroke from stroke mimics.

Answer 225

objectively measure the impairment caused by a stroke.

Answer 226

Jacksonian movement (clonic movements travelling proximally) Indicates frontal lobe epilepsy

Answer 227

spinothalamic sensory loss (pain and temperature)

Answer 228

posterior communicating artery aneurysm

Answer 229

Measures disability or dependence in 10 different activities of daily living in stroke patients. This index can be used to assess the functional status and to monitor their improvement with ongoing rehabilitation.

Answer 230

CAN OPEN ``` Confusion Ataxia Nystagmus Ophthalmoplegia PEripheral Neuropathy ```

Answer 231

- plasmapheresis - filter blood | - intravenous immunoglobulins

Answer 232

Stevens-Johnson syndrome

Answer 233

Pseudoseizures

Answer 234

third nerve palsy

Answer 235

Radicular pathology such as disc herniation. The patient feels pain in the back when the leg is raised between 30-60 degrees.

Answer 236

4 features 1. rigidity 2. hyperthermia 3. autonomic instability (hypotension, tachycardia) 4. altered mental status (confusion)

Answer 237

Acute withdrawal of LEVODOPA can precipitate neuroleptic malignant syndrome As a result, levodopa is a critical medication, and must be given on time and not missed with acute admissions. Giving METOCLOPAMIDE or HALOPERIDOL, rather than withholding them, can precipitate neuroleptic malignant syndrome due to their dopaminergic antagonism.

Answer 238

Headache following lumbar puncture (LP) occurs in approximately one-third of patients. The pathophysiology of is unclear but may relate to a 'leak' of CSF following dural puncture. Post-LP headaches are more common in young females with a low body mass index Typical features - usually develops within 24-48 hours following LP but may occur up to one week later - may last several days - worsens with upright position - improves with recumbent position Factors which may contribute to headache - Increased needle size - Direction of bevel - Not replacing the stylet - Increased number of LP attempts Factors which do not contribute to headache - Increased volume of CSF removed - Bed rest following procedure - Increased fluid intake post procedure - Opening pressure of CSF - Position of patient Management: - supportive initially (analgesia, rest) - if pain continues for more than 72 hours then specific - treatment is indicated, to prevent subdural haematoma treatment options include: blood patch, epidural saline and intravenous caffeine

Answer 239

1. Buccal midazolam/ IV lorazepam 2. IV lorazepam 3. IV phenytoin (phenobarbital if already on regular phenytoin) 4. Rapid sequence induction of anaesthesia using thiopental sodium

Answer 240

Common peroneal nerve lesion

Answer 241

a physiological nervous system response to increased intracranial pressure (ICP) that results in hypertension and bradycardia

Answer 242

extradural haematoma

Answer 243

Brown- Séquard Syndrome

Answer 244

BNF recommend dose for rectal diazepam, repeated once after 10-15 minutes if necessary BNF recommend dose for rectal diazepam, repeated once after 10-15 minutes if necessary ``` Neonate 1.25 - 2.5 mg Child 1 month - 1 year 5 mg Child 2 years - 11 years 5 - 10 mg Child 12 years - 17 years 10 mg Adult 10 - 20 mg (max. 30 mg) Elderly 10 mg (max. 15 mg) ```