Neurology Flashcards

Question

How is Anti-NMDA receptor encephalitis managed?

Answer 1

Treatment of anti-NMDA encephalitis is based of immunosuppression with intravenous steroids, immunoglobulins, rituximab, cyclophosphamide or plasma exchange, alone or in combination. Resection of teratoma is also therapeutic.

Answer 2

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 - erectile dysfunction: often an early feature - postural hypotension - atonic bladder cerebellar signs

Answer 3

Parkinson+cerebellar sign+autonomic dysfunction =Multiple system atrophy Parkinson + vertical gaze palsy= PSP Parkinson + visual hallucinations +deteriorated by antipsychotic =Lewy body dementia

Answer 4

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.

Answer 5

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) hyporeflexia autonomic symptoms: dry mouth, impotence, difficulty micturating ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)

Answer 6

incremental response to repetitive electrical stimulation

Answer 7

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 8

Essential tremor (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs

Answer 9

Features postural tremor: worse if arms outstretched improved by alcohol and rest most common cause of titubation (head tremor)

Answer 10

Management propranolol is first-line primidone is sometimes used

Answer 11

5-HT3 antagonists are antiemetics used mainly in the management of chemotherapy-related nausea. They mainly act in the chemoreceptor trigger zone area of the medulla oblongata. Examples ondansetron palonosetron second-generation 5-HT3 antagonist main advantage is reduced effect on the QT interval Prolonged QT interval Constipation

Answer 12

sensory inattention apraxias astereognosis (tactile agnosia) inferior homonymous quadrantanopia Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation

Answer 13

homonymous hemianopia (with macula sparing) cortical blindness visual agnosia

Answer 14

Wernicke's aphasia: this area 'forms' the speech before 'sending it' to Brocas area. Lesions result in word substituion, neologisms but speech remains fluent superior homonymous quadrantanopia auditory agnosia prosopagnosia (difficulty recognising faces)

Answer 15

expressive (Broca's) aphasia: located on the posterior aspect of the frontal lobe, in the inferior frontal gyrus. Speech is non-fluent, laboured, and halting disinhibition perseveration anosmia inability to generate a list

Answer 16

midline lesions: gait and truncal ataxia hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus

Answer 17

Agonists of the 5-HT3 receptor include ethanol and varenicline

Answer 18

Contralateral hemiparesis and sensory loss, lower extremity > upper

Answer 19

Contralateral hemiparesis and sensory loss, upper extremity > lower Contralateral homonymous hemianopia Aphasia

Answer 20

Contralateral homonymous hemianopia with macular sparing Visual agnosia

Answer 21

Weber's syndrome (branches of the posterior cerebral artery that supply the midbrain) Ipsilateral CN III palsy Contralateral weakness of upper and lower extremity

Answer 22

osterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome) Ipsilateral: facial pain and temperature loss Contralateral: limb/torso pain and temperature loss Ataxia, nystagmus

Answer 23

Anterior inferior cerebellar artery (lateral pontine syndrome) Symptoms are similar to Wallenberg's (see above), but: Ipsilateral: facial paralysis and deafness

Answer 24

Lacunar strokes present with either isolated hemiparesis, hemisensory loss or hemiparesis with limb ataxia strong association with hypertension common sites include the basal ganglia, thalamus and internal capsule

Answer 25

Features vertigo triggered by change in head position (e.g. rolling over in bed or gazing upwards) may be associated with nausea each episode typically lasts 10-20 seconds positive Dix-Hallpike manoeuvre rapidly lower the patient to the supine position with an extended neck a positive test recreates the symptoms of benign paroxysmal positional vertigo rotatory nystagmus

Answer 26

1st line - amitriptyline, duloxetine, gabapentin or pregabalin if the first-line drug treatment does not work try one of the other 3 drugs 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 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

Answer 27

diabetic neuropathy post-herpetic neuralgia trigeminal neuralgia prolapsed intervertebral disc

Answer 28

dopamine receptor agonist

Answer 29

ergot-derived dopamine receptor agonists (bromocriptine, cabergoline) have been associated with pulmonary, retroperitoneal and cardiac fibrosis. The Committee on Safety of Medicines advice that an echocardiogram, ESR, creatinine and chest x-ray should be obtained prior to treatment and patients should be closely monitored

Answer 30

Meniere's disease is a disorder of the inner ear of unknown cause. It is characterised by excessive pressure and progressive dilation of the endolymphatic system. It is more common in middle-aged adults but may be seen at any age. Meniere's disease has a similar prevalence in both men and women.

Answer 31

recurrent episodes of vertigo, tinnitus and hearing loss (sensorineural). Vertigo is usually the prominent symptom a sensation of aural fullness or pressure is now recognised as being common other features include nystagmus and a positive Romberg test episodes last minutes to hours typically symptoms are unilateral but bilateral symptoms may develop after a number of years symptoms typically resolve in 5-10 years majority of patients will be left with a degree of hearing loss

Answer 32

ENT assessment is required to confirm the diagnosis patients should inform the DVLA. The current advice is to cease driving until satisfactory control of symptoms is achieved acute attacks: buccal or intramuscular prochlorperazine. Admission is sometimes required prevention: betahistine and vestibular rehabilitation exercises may be of benefit

Answer 33

alexia (inability to read), without agraphia (inability to write). This occurs because of an infarction of the left posterior cerebral artery which perfuses the splenium of the corpus callosum and left visual (occipital) cortex.

Answer 34

DCM symptoms can include any combination of [1]: 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.

Answer 35

Neurological - altered mental status - ranging from mild confusion to deep coma, fluctuations are common Focal Neurological Deficits: Weakness on one side of the body, aphasia, or visual field defects, depending on the haematoma's location. Headache: Often localised to one side, worsening over time. Seizures: May occur, particularly in acute or expanding hematomas. Behavioural and Cognitive Changes: Memory Loss: Especially in chronic SDH. Personality Changes: Irritability, apathy, or depression. Cognitive Impairment: Difficulty with attention, problem-solving, and other executive functions.

Answer 36

Physical Examination Findings: Papilloedema: Indicates raised intracranial pressure. Pupil Changes: Unilateral dilated pupil, especially on the side of the haematoma, indicating compression of the third cranial nerve. Gait Abnormalities: Including ataxia or weakness in one leg. Hemiparesis or Hemiplegia: Reflecting the mass effect and midline shift.

Answer 37

acute - CT imaging is the first-line investigation and will show a crescentic collection, not limited by suture lines. They will appear hyperdense (bright) in comparison to the brain. Large acute subdural haematomas will push on the brain ('mass effect') and cause midline shift or herniation. Chronic - On CT imaging they similarly are crescentic in shape, not restricted by suture lines and compress the brain ('mass effect'). In contrast to acute subdurals, chronic subdurals are hypodense (dark) compared to the substance of the brain.

Answer 38

Charcot-Marie-Tooth Disease is the most common hereditary peripheral neuropathy. It results in a predominantly motor loss. There is no cure, and management is focused on physical and occupational therapy. Last updated: 7th July 2024

Answer 39

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 40

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.

Answer 41

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 collapse

Answer 42

Stokes-Adams attack, refers to a transient loss of consciousness (syncope) due to a temporary interruption in the blood supply to the brain caused by heart block or certain arrhythmias.

Answer 43

A useful mnemonic to remember the features of Wernicke's encephalopathy is CAN OPEN Confusion Ataxia Nystagmus Ophthamoplegia PEripheral Neuropathy

Answer 44

Spastic paraparesis is a condition that causes weakness and stiffness in the lower limbs. It's a type of central nervous system disease that's often inherited.

Answer 45

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) cafe-au-lait spots* may be seen

Answer 46

Tuberous sclerosis complex (TSC), also known as tuberous sclerosis, is a rare genetic disease that causes non-cancerous (benign) tumors to grow in the brain and several areas of the body, including the spinal cord, nerves, eyes, lung, heart, kidneys, and skin

Answer 47

Otosclerosis describes the replacement of normal bone by vascular spongy bone. It causes a progressive conductive deafness due to fixation of the stapes at the oval window. Otosclerosis is autosomal dominant and typically affects young adults

Answer 48

Onset is usually at 20-40 years - features include: conductive deafness tinnitus tympanic membrane the majority of patients will have a normal tympanic membrane 10% of patients may have a 'flamingo tinge', caused by hyperaemia positive family history

Answer 49

Management hearing aid stapedectomy

Answer 50

Hemiballism occurs following damage to the subthalamic nucleus. Ballisic movements are involuntary, sudden, jerking movements which occur contralateral to the side of the lesion. The ballisic movements primarily affect the proximal limb musculature whilst the distal muscles may display more choreiform-like movements Symptoms may decrease whilst the patient is asleep. Antidopaminergic agents (e.g. Haloperidol) are the mainstay of treatment lesion most commonly found in the basal ganglia

Answer 51

The diagnosis is clinical although bloods such as ferritin to exclude iron deficiency anaemia may be appropriate

Answer 52

simple measures: walking, stretching, massaging affected limbs treat any iron deficiency dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole) benzodiazepines gabapentin

Answer 53

May occur with or without impairment of consciousness or awareness An aura occurs in most patients typically a rising epigastric sensation also psychic or experiential phenomena, such as dejÃ vu, jamais vu less commonly hallucinations (auditory/gustatory/olfactory) Seizures typically last around one minute automatisms (e.g. lip smacking/grabbing/plucking) are common

Answer 54

Head/leg movements, posturing, post-ictal weakness, Jacksonian march

Answer 55

Paraesthesia

Answer 56

flashers/floaters

Answer 57

Mechanism of action sodium channel blocker Adverse effects Stevens-Johnson syndrome

Answer 58

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.

Answer 59

Causes of Parkinsonism 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 60

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) hyporeflexia autonomic symptoms: dry mouth, impotence, difficulty micturating ophthalmoplegia and ptosis not commonly a feature (unlike in myasthenia gravis)

Answer 61

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 62

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.

Answer 63

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

Answer 64

A collection of cerebrospinal fluid within the spinal cord. ## Footnote Syringobulbia is a similar phenomenon in which there is a fluid-filled cavity within the medulla of the brainstem. This is often an extension of the syringomyelia but in rare cases can be an isolated finding.

Answer 65

a Chiari malformation: strong association trauma tumours idiopathic

Answer 66

loss of sensation to temp but preservation of light touch, proprioception and vibration in the neck shoulders and arms - this is due to the crossing spinothalamic tracts in the anterior commissure of the spinal cord being the first tracts to be affected Spastic weakness Neuropathic pain upgoing plantars autonomic features: Horner's syndrome due to compression of the sympathetic chain

Answer 67

A full MRI spine to exclude a tumour or thered cord A brain MRI to exclude chiari malformation

Answer 68

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 69

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 70

Uhthoff 's phenomenon where neurological symptoms are exacerbated by increases in body temperature is typically associated with multiple sclerosis

Answer 71

paraesthesiae in limbs on neck flexion

Answer 72

Neurological sequalae sensorineural hearing loss (most common) seizures focal neurological deficit infective sepsis intracerebral abscess pressure brain herniation hydrocephalus Patients with meningococcal meningitis are at risk of Waterhouse-Friderichsen syndrome (adrenal insufficiency secondary to adrenal haemorrhage).

Answer 73

adrenal insufficiency secondary to adrenal haemorrhage

Answer 74

Juvenile myoclonic epilepsy (Janz syndrome) typical onset is in the teenage years, more common in girls features: infrequent generalized seizures, often in morning//following sleep deprivation daytime absences sudden, shock-like myoclonic seizure (these may develop before seizures) treatment: usually good response to sodium valproate

Answer 75

a cause of horizontal disconjugate eye movement due to a lesion in the medial longitudinal fasciculus (MLF) controls horizontal eye movements by interconnecting the IIIrd, IVth and VIth cranial nuclei located in the paramedian area of the midbrain and pons

Answer 76

impaired adduction of the eye on the same side as the lesion horizontal nystagmus of the abducting eye on the contralateral side

Answer 77

multiple sclerosis vascular disease

Answer 78

pressure = 60-150 mm (patient recumbent) protein = 0.2-0.4 g/l glucose = > 2/3 blood glucose cells: red cells = 0, white cells < 5/mm³

Answer 79

viral meningitis/encephalitis TB meningitis partially treated bacterial meningitis Lyme disease Behcet's, SLE lymphoma, leukaemia

Answer 80

paraneoplastic syndrome associated with ovarian and breast cancer patients have a cerebellar syndrome

Answer 81

associated with small cell lung carcinoma and neuroblastomas sensory neuropathy - may be painful cerebellar syndrome encephalomyelitis

Answer 82

Vestibular schwannomas (sometimes referred to as acoustic neuromas) account for approximately 5% of intracranial tumours and 90% of cerebellopontine angle tumours.

Answer 83

The classical history of vestibular schwannoma includes 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

Answer 84

neurofibromatosis type 2.

Answer 85

Friedreich's ataxia is the most common of the early-onset hereditary ataxias. It is an autosomal recessive, trinucleotide repeat disorder characterised by a GAA repeat in the X25 gene on chromosome 9 (frataxin). Friedreich's ataxia is unusual amongst trinucleotide repeat disorders in not demonstrating the phenomenon of anticipation.

Answer 86

Neurological features absent ankle jerks/extensor plantars cerebellar ataxia optic atrophy spinocerebellar tract degeneration Other features hypertrophic obstructive cardiomyopathy (90%, most common cause of death) diabetes mellitus (10-20%) high-arched palate

Answer 87

Alcohol may trigger an attack and there also appears to be a relation to nocturnal sleep.

Answer 88

-intense sharp, stabbing pain around one eye pain typical occurs once or twice a day, each episode lasting 15 mins - 2 hours the patient is restless and agitated during an attack due to the severity clusters typically last 4-12 weeks accompanied by redness, lacrimation, lid swelling nasal stuffiness miosis and ptosis in a minority

Answer 89

most patients will have neuroimaging - underlying brain lesions are sometimes found even if the clinical symptoms are typical for cluster headache MRI with gadolinium contrast is the investigation of choice

Answer 90

NICE recommend seeking specialist advice from a neurologist if a patient develops cluster headaches acute 100% oxygen (80% response rate within 15 minutes) subcutaneous triptan (75% response rate within 15 minutes) prophylaxis verapamil is the drug of choice there is also some evidence to support a tapering dose of prednisolone

Answer 91

Von Hippel-Lindau (VHL) syndrome is an autosomal dominant condition predisposing to neoplasia. It is due to an abnormality in the VHL gene located on short arm of chromosome 3

Answer 92

cerebellar haemangiomas: these can cause subarachnoid haemorrhages retinal haemangiomas: vitreous haemorrhage renal cysts (premalignant) phaeochromocytoma extra-renal cysts: epididymal, pancreatic, hepatic endolymphatic sac tumours clear-cell renal cell carcinoma

Answer 93

Hereditary sensorimotor neuropathy (HSMN) is a relatively new term which encompasses Charcot-Marie-Tooth disease (also known as peroneal muscular atrophy). Over 7 types have been characterised - however only 2 are common to clinical practice HSMN type I: primarily due to demyelinating pathology HSMN type II: primarily due to axonal pathology

Answer 94

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 95

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.

Answer 96

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 97

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

Answer 98

males: sodium valproate females: levetiracetam

Answer 99

CAG - Huntingtons GAA - Friedrich Ataxia CTG - myotinic dystrophy CGG - fragile X syndrome

Answer 100

Genetics autosomal dominant trinucleotide repeat disorder: repeat expansion of CAG as Huntington's disease is a trinucleotide repeat disorder, the phenomenon of anticipation may be seen, where the disease is presents at an earlier age in successive generations results in degeneration of cholinergic and GABAergic neurons in the striatum of the basal ganglia due to defect in huntingtin gene on chromosome 4

Answer 101

Features typical develop after 35 years of age chorea personality changes (e.g. irritability, apathy, depression) and intellectual impairment dystonia saccadic eye movements

Answer 102

Subacute combined degeneration of the spinal cord is due to vitamin B12 deficiency resulting in impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts.. Recreational nitrous oxide inhalation may also result in vitamin B12 deficiency → subacute combined degeneration of the spinal cord.

Answer 103

giving folate to a patient deficient in B12 can precipitate subacute combined degeneration of the cord Always replace B12 before folare

Answer 104

dorsal column involvement distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms impaired proprioception and vibration sense lateral corticospinal tract involvement muscle weakness, hyperreflexia, and spasticity upper motor neuron signs typically develop in the legs first brisk knee reflexes absent ankle jerks extensor plantars spinocerebellar tract involvement sensory ataxia → gait abnormalities positive Romberg's sign

Answer 105

(lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation Parietal lobe lesions may cause Gerstmann's syndrome

Answer 106

Bleeding into the space between the dura mater and the skull. Often results from acceleration-deceleration trauma or a blow to the side of the head. The majority of epidural haematomas occur in the temporal region where skull fractures cause a **rupture of the middle meningeal artery.** Features features of raised intracranial pressure some patients may exhibit a lucid interval

Answer 107

Bleeding into the outermost meningeal layer. Most commonly occur around the frontal and parietal lobes. Risk factors include old age, alcoholism and anticoagulation. Slower onset of symptoms than a epidural haematoma. There may be fluctuating confusion/consciousness

Answer 108

An intracerebral (or intraparenchymal) haemorrhage is a collection of blood within the substance of the brain. Causes / risk factors include: hypertension, vascular lesion (e.g. aneurysm or arteriovenous malformation), cerebral amyloid angiopathy, trauma, brain tumour or infarct (particularly in stroke patients undergoing thrombolysis). Patients will present similarly to an ischaemic stroke (which is why it is crucial to obtain a CT in head in all stroke patients prior to thrombolysis) or with a decrease in consciousness. CT imaging will show a hyperdensity (bright lesion) within the substance of the brain. Treatment is often conservative under the care of stroke physicians, but large clots in patients with impaired consciousness may warrant surgical evacuation.

Answer 109

the Cushings reflex (hypertension and bradycardia) often occurs late following head injury and is usually a pre terminal event

Answer 110

Astereognosis is defined as the inability to identify objects by touch, despite intact elementary tactile, proprioceptive, and thermal sensation. It occurs due to a lesion in the parietal lobe.

Answer 111

Diagnosis: positive Dix-Hallpike manoeuvre rapidly lower the patient to the supine position with an extended neck a positive test recreates the symptoms of benign paroxysmal positional vertigo rotatory nystagmus Treatment Epley manoeuvre (successful in around 80% of cases)

Answer 112

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 113

Multiple System Atrophy (MSA) is a rare, progressive neurodegenerative disorder that affects movement, balance, and autonomic functions, leading to symptoms like Parkinsonism, cerebellar dysfunction, and autonomic failure, ultimately causing a decline in overall bodily function

Answer 114

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 erectile dysfunction: often an early feature postural hypotension atonic bladder cerebellar signs

Answer 115

Baclofen is used to treat muscle spasticity in conditions such as multiple sclerosis, cerebral palsy and spinal cord injuries. Mechanism of action agonist of GABA receptors acts in the central nervous system (brain and spinal cord)

Answer 116

Brachial neuritis is characterized by acute onset of unilateral (occasionally bilateral) severe pain, followed by shoulder and scapular weakness several days later. Sensory changes are usually minimal. There may be subsequent rapid wasting of the arm muscles in accordance to which nerve is involved. Precipitating factors include recent trauma, infection, surgery, or even vaccination. Rarely it may be hereditary. The prognosis is usually good except when the phrenic nerve is involved since this can result in significant breathlessness.

Answer 117

Drug causes of gingival hyperplasia phenytoin ciclosporin calcium channel blockers (especially nifedipine) Other causes of gingival hyperplasia include acute myeloid leukaemia (myelomonocytic and monocytic types)

Answer 118

typical onset in teenage years hypersomnolence cataplexy (sudden loss of muscle tone often triggered by emotion) sleep paralysis vivid hallucinations on going to sleep or waking up

Answer 119

it is associated with low levels of orexin (hypocretin), a protein which is responsible for controlling appetite and sleep patterns

Answer 120

daytime stimulants (e.g. modafinil) and nighttime sodium oxybate

Answer 121

Posterior communicating artery aneurysm

Answer 122

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

Answer 123

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)

Answer 124

Natalizumab Ocrelizumab Fingolimod Beta interferon Glatiramer acetate

Answer 125

a recombinant monoclonal antibody that antagonises alpha-4 beta-1-integrin found on the surface of leucocytes inhibit migration of leucocytes across the endothelium across the blood-brain barrier

Answer 126

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

Answer 127

Fatigue 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 128

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 129

specific serotonin (5-HT1) receptor agonists

Answer 130

Oscillopsia (visual fields appear to oscillate) gabapentin is first-line

Answer 131

Postoperatively, patients with cervical myelopathy require ongoing follow-up as pathology can 'recur' at adjacent spinal levels, which were not treated by the initial decompressive surgery. This is called adjacent segment disease. Furthermore, surgery can change spinal dynamics increasing the likelihood of other levels being affected. Patients sometimes develop mal-alignment of the spine, including kyphosis and spondylolisthesis, and this can also affect the spinal cord. All patients with recurrent symptoms should be evaluated urgently by specialist spinal services.

Answer 132

Midbrain: vertical gaze palsy Pons: horizontal gaze palsy CN 3,4 nuclei in Midbrain hence vertical (SO4, R3) CN 6 in pons hence horizontal (LR6)

Answer 133

Parinaud's syndrome, also known as dorsal midbrain syndrome, is a neurological condition characterized by difficulty looking upwards, convergence-retraction nystagmus, and pupillary light-near dissociation, caused by damage to the dorsal midbrain Rostral interstitial nucleus of medial longitudinal fasciculus lies at the dorsal midbrain and control vertical gaze. They project to the vestibular nuclei. It results in the following symptoms: Upward gaze palsy, often manifesting as diplopia Pupillary light-near dissociation (Pseudo-Argyll Robertson pupils) Convergence-retraction nystagmus It's aetiology include: Brain tumours in the midbrain or pineal gland (pinealoma) Multiple sclerosis Midbrain stroke

Answer 134

Anti Ri also known as anti-neuronal nuclear antibody type 2 or ANNA-2

Answer 135

Opsoclonus-myoclonus syndrome is characterised by rapid, irregular, multidirectional eye movements (opsoclonus), involuntary muscle jerking (myoclonus), and ataxia.

Answer 136

Cervical Spondylotic Myelopathy (CSM) and Degenerative Cervical Myelopathy (DCM) are often used interchangeably, however, there is a subtle difference between them. CSM specifically refers to spinal cord compression caused by age-related changes in the bones and tissues of the neck. These changes include osteophytes formation, disc herniation or ligamentous hypertrophy that result in narrowing of the spinal canal leading to myelopathy. On the other hand, DCM is an umbrella term that includes CSM but also encompasses other degenerative conditions that cause spinal cord compression in the cervical region such as ossification of posterior longitudinal ligament (OPLL). Therefore, all cases of CSM are considered DCM but not all cases of DCM are necessarily due to spondylosis.

Answer 137

Features a variety of motor weakness, sensory loss and bladder/bowel dysfunction may be seen neck pain wide-based, ataxic or spastic gait upper motor neuron weakness in the lower legs - increased reflexes, increased tone and upgoing plantars bladder dysfunction e.g. urgency, retention ## Footnote you get a lower motor neuron lesion at the level of the compression and UML below. cervical

Answer 138

Cervical spondylosis is a degenerative condition affecting the cervical spine, essentially osteoarthritis of the cervical vertebral bodies. If the spinal canal is narrowed due to this process it can press on the spinal cord resulting in neurological dysfunction. Myelopathy is thought to occur in around 5-10% of patients who have cervical spondylosis

Answer 139

An Anti-epileptic medication Inhibits the enzyme GABA transaminase, increasing levels of gamma-aminobutyric acid (GABA) in the brain and thereby reducing seizure activity. Side effects - visual fiels defects

Answer 140

Raised intraocular pressure In acute angle-closure glaucoma (AACG) there is a rise in IOP secondary to an impairment of aqueous outflow. Factors predisposing to AACG include: hypermetropia (long-sightedness) pupillary dilatation lens growth associated with age

Answer 141

reduced appetite and weight loss dizziness paraesthesia lethargy and poor concentration rare but important: acute myopia and secondary angle-closure glaucoma

Answer 142

severe pain: may be ocular or headache decreased visual acuity symptoms worse with mydriasis (e.g. watching TV in a dark room) hard, red-eye haloes around lights semi-dilated non-reacting pupil corneal oedema results in dull or hazy cornea systemic upset may be seen, such as nausea and vomiting and even abdominal pain

Answer 143

amiodarone isoniazid vincristine nitrofurantoin metronidazole

Answer 144

simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches) opioid analgesics should be gradually withdrawn

Answer 145

Transient global amnesia is a clinical syndrome of uncertain aetiology, characterised by a discrete episode lasting for a few hours (always less than 24 hours) of anterograde amnesia, retrograde amnesia, repetitive questioning with an absence of other cognitive or neurological impairments. Diagnostic criteria (in addition to the above features) are as follows; reliable witness to episode, an absence of head trauma or loss of consciousness at the onset, preserved personal identity and absence of epileptic features. Epilepsy can present with discreet episodes of amnesia. This syndrome is called transient epileptic amnesia. Features that suggest epilepsy are; shorter duration (should be less than 1 hour), multiple attacks, onset on waking from sleep and accompanying epileptic features - e.g. motor automatism, stereotyped behaviours, limb shaking.

Answer 146

Overview aka Steele-Richardson-Olszewski syndrome a 'Parkinson Plus' syndrome Features postural instability and falls patients tend to have a stiff, broad-based gait impairment of vertical gaze (down gaze worse than up gaze - patients may complain of difficultly reading or descending stairs) parkinsonism bradykinesia is prominent cognitive impairment primarily frontal lobe dysfunction

Answer 147

5HT3 antagonists

Answer 148

Erb-Duchenne paralysis damage to C5,6 roots winged scapula may be caused by a breech presentation

Answer 149

damage to T1 loss of intrinsic hand muscles due to traction ## Footnote This paralysis or palsy is normally associated with a traumatic birth injury, or the mechanism described above of acute traction of the arm upwards. The injury results in reduced power of the intrinsic hand muscles and over time can result in a 'claw-like' appearance.

Neurology Flashcards

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