Neurology Flashcards
Epilepsy - pregnancy and breast feeding?
Take folic acid
Aim for mono therapy
Sodium valproate - associated with neural tube defects
Carbamazepine -often the least teratogenic
Phenytoin - associated with cleft palate
lamotrigine - congenital malformations may be low. The dose of lamotrigine may need to be increased in pregnancy
Breast feeding - considered safe with anti-epileptics apart from barbiturates
It is advised that pregnant women taking phenytoin are given vitamin K in the last month of pregnancy to prevent clotting disorders in the newborn
what is restless leg syndrome?
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.
clinical features of restless legs syndrome?
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 of restless legs syndromes?
there is a positive family history in 50% of patients with idiopathic RLS
iron deficiency anaemia
uraemia
diabetes mellitus
pregnancy
Treatment of restless leg syndrome?
simple measures: walking, stretching, massaging affected limbs
treat any iron deficiency
dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
benzodiazepines
gabapentin
Features of a third nerve palsy ?
eye is deviated ‘down and out’
ptosis
pupil may be dilated (sometimes called a ‘surgical’ third nerve palsy)
Causes of 3rd nerve palsy?
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
Clinical signs of Uncal herniation?
Signs of uncal herniation may include loss of consciousness, hypertension, bradycardia and Cheyne-Stokes respiration.
what drug is contraindicated in absence seizures?
Carbamazepine
Features of essential Tremor?
Management?
AD inheritance
Features
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor) - usually worse on movement and during stress - relieved by alcohol and sleep
Management
propranolol is first-line
primidone is sometimes used
What is Spastic Paraparesis?
Spastic paraparesis describes a upper motor neuron pattern of weakness in the lower limbs
causes of spastic paraparesis?
demyelination e.g. multiple sclerosis
cord compression: trauma, tumour
parasagittal meningioma
tropical spastic paraparesis
transverse myelitis e.g. HIV
syringomyelia
hereditary spastic paraplegia
osteoarthritis of the cervical spine
where is wernicke’s area found?
Brodmann area 22 in the superior temporal gyrus
treatment for MND?
Riluzole
prevents stimulation of glutamate receptors
used mainly in amyotrophic lateral sclerosis
prolongs life by about 3 months
Respiratory care
non-invasive ventilation (usually BIPAP) is used at night
studies have shown a survival benefit of around 7 months
Nutrition
percutaneous gastrostomy tube (PEG) is the preferred way to support nutrition and has been associated with prolonged survival
Prognosis
poor: 50% of patients die within 3 years
what is the treatment for myoclonic seizures?
males: sodium valproate
females: levetiracetam
what is the treatment for absence seizures?
first line: ethosuximide
second line:
male: sodium valproate
female: lamotrigine or levetiracetam
carbamazepine may exacerbate absence seizures
What is Myotonic Dystrophy?
Myotonic dystrophy (also called dystrophia myotonica) is an inherited myopathy with features developing at around 20-30 years old. It affects skeletal, cardiac and smooth muscle. There are two main types of myotonic dystrophy, DM1 and DM2.
What are the genetics of myotonic dystrophy?
autosomal dominant
a trinucleotide repeat disorder
DM1 is caused by a CTG repeat at the end of the DMPK (Dystrophia Myotonica-Protein Kinase) gene on chromosome 19
DM2 is caused by a repeat expansion of the ZNF9 gene on chromosome 3
What are the key difference between the two types of myotonic dystrophy?
DM1
- DMPK gene on chromosome 19
- Distal weakness more prominent
DM2
- ZNF9 gene on chromosome 3
- Proximal weakness more prominent
- Severe congenital form not seen
Features of myotonic dystrophy?
General features
myotonic facies (long, ‘haggard’ appearance)
frontal balding
bilateral ptosis
cataracts
dysarthria
Other features
myotonia (tonic spasm of muscle)
weakness of arms and legs (distal initially)
mild mental impairment
diabetes mellitus
testicular atrophy
cardiac involvement: heart block, cardiomyopathy
dysphagia
what are the features of Miller Fisher syndrome?
ssociated 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
what antibodies are associated with Miller Fisher Syndrome?
anti-GQ1b antibodies are present in 90% of cases
What is Anti-NMDA receptor encephalitis?
Anti-NMDA receptor encephalitis is a paraneoplastic syndrome, presenting as prominent psychiatric features including agitation, hallucinations, delusions and disordered thinking; seizures, insomnia, dyskinesias and autonomic instability
What is associated with Anti-NMDA receptor encephalitis?
Ovarian teratomas are detected in up to half of all female adult patients, particularly prevalent in Afro-Caribbean patients
How is Anti-NMDA receptor encephalitis managed?
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.
What is multiple system atrophy?
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
how to differentiate between PSP, MSA and LBD?
Parkinson+cerebellar sign+autonomic dysfunction =Multiple system atrophy
Parkinson + vertical gaze palsy= PSP
Parkinson + visual hallucinations +deteriorated by antipsychotic =Lewy body dementia
What is Lambert-Eaton Syndrome?
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.
What are the features of Lambert-Eaton Syndrome?
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)
what would an EMG show in Lambert-Eaton Syndrome?
incremental response to repetitive electrical stimulation
What is the management of Lambert-Eaton Syndrome?
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
inheritance of essential tremor?
Essential tremor (previously called benign essential tremor) is an autosomal dominant condition which usually affects both upper limbs
Features of essential tremor?
Features
postural tremor: worse if arms outstretched
improved by alcohol and rest
most common cause of titubation (head tremor)
management of essential tremor?
Management
propranolol is first-line
primidone is sometimes used
what are examples of 5-HT3 antagonists?
what are the adverse effects?
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
what would a parietal lobe lesion cause?
sensory inattention
apraxias
astereognosis (tactile agnosia)
inferior homonymous quadrantanopia
Gerstmann’s syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
What would occipital lobe lesion cause?
homonymous hemianopia (with macula sparing)
cortical blindness
visual agnosia
what would a temporal lobe lesion cause?
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)
What would a frontal lobe lesion cause?
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
what would a cerebellum lesion cause?
midline lesions: gait and truncal ataxia
hemisphere lesions: intention tremor, past pointing, dysdiadokinesis, nystagmus
what are examples of 5-HT3 agonist?
Agonists of the 5-HT3 receptor include ethanol and varenicline
was would an anterior cerebral artery lesion cause?
Contralateral hemiparesis and sensory loss, lower extremity > upper
what would a middle cerebral artery lesion cause?
Contralateral hemiparesis and sensory loss, upper extremity > lower
Contralateral homonymous hemianopia
Aphasia
what would a posterior cerebral artery lesion cause?
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
what is Weber’s syndrome?
Weber’s syndrome (branches of the posterior cerebral artery that supply the midbrain)
Ipsilateral CN III palsy
Contralateral weakness of upper and lower extremity
what would a lesion in posterior inferior cerebellar artery cause?
osterior inferior cerebellar artery (lateral medullary syndrome, Wallenberg syndrome)
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
What would an anterior inferior cerebellar artery lesion cause?
Anterior inferior cerebellar artery (lateral pontine syndrome)
Symptoms are similar to Wallenberg’s (see above), but:
Ipsilateral: facial paralysis and deafness
Lacunar strokes?
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
features of BPPV?
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
management of neuropathic pain?
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
causes of neuropathic pain?
diabetic neuropathy
post-herpetic neuralgia
trigeminal neuralgia
prolapsed intervertebral disc
mechanism of action of ropinirole?
dopamine receptor agonist
side effects of ergot-derived dopamine receptor agonists?
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
what is Meniere’’s disease?
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.
what are the features of Ménière’s disease?
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
management of Ménière’s disease?
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
where would the lesion be if a patient is unable to read but able to write?
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.
symptoms of degenerative cervical myelopathy?
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.
clinical features of SDH?
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.
Physical examination findings in SDH?
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.
what would you see on CT of an acute SDH and chronic SDH?
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.
what is Charcot Marie tooth disease
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
what are the clinical features of Charcot Marie Tooth disease?
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
how is degenerative cervical myelopathy managed?
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.
what is cataplexy?
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
what is stokes Adams attack?
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.
features of Wernicke’s encephalopathy
A useful mnemonic to remember the features of Wernicke’s encephalopathy is CAN OPEN
Confusion
Ataxia
Nystagmus
Ophthamoplegia
PEripheral
Neuropathy
what is spastic paraparesis?
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.
what are the cutaneous features of tuberous sclerosis?
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
what is tuberous sclerosis?
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
what is otosclerosis?
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
what are the features of otosclerosis?
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
what is the management of otosclerosis?
Management
hearing aid
stapedectomy
what is Hemibalism?
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
what test is important to do in restless legs?
The diagnosis is clinical although bloods such as ferritin to exclude iron deficiency anaemia may be appropriate
management of restless legs?
simple measures: walking, stretching, massaging affected limbs
treat any iron deficiency
dopamine agonists are first-line treatment (e.g. Pramipexole, ropinirole)
benzodiazepines
gabapentin
temporal lobe focal seizure symptoms?
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 dejà vu, jamais vu
less commonly hallucinations (auditory/gustatory/olfactory)
Seizures typically last around one minute
automatisms (e.g. lip smacking/grabbing/plucking) are common
frontal lobe focal seizure symptoms?
Head/leg movements, posturing, post-ictal weakness, Jacksonian march
parietal lobe focal seizure symptoms?
Paraesthesia
occipital lobe focal seizure symptoms?
flashers/floaters
mechanism of action of lamotrigine and the adverse effects?
Mechanism of action
sodium channel blocker
Adverse effects
Stevens-Johnson syndrome
what is Lambert-eaton syndrome?
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.
causes of Parkinsonism?
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
what are the features of lamber-eaton syndrome?
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)
management of Lambert eaton syndrome?
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
What is the Arnold Chiari malformation?
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 of Arnold Chiari malformation?
non-communicating hydrocephalus may develop as a result of obstruction of cerebrospinal fluid (CSF) outflow
headache
syringomyelia
What is Syringomyelia?
A collection of cerebrospinal fluid within the spinal cord.
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.
Causes of Syringomyelia?
a Chiari malformation: strong association
trauma
tumours
idiopathic
Features of Syringomyelia?
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
Investigations for Syringomyelia?
A full MRI spine to exclude a tumour or thered cord
A brain MRI to exclude chiari malformation
Treatment for Syringomyelia?
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.
Features of MS?
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
What is Uhthoff’s phenomenon?
Uhthoff ‘s phenomenon where neurological symptoms are exacerbated by increases in body temperature is typically associated with multiple sclerosis
What is Lhermitte’s syndrome?
paraesthesiae in limbs on neck flexion
Complications of meningitis?
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).
What is Waterhouse-Friderichsen syndrome?
adrenal insufficiency secondary to adrenal haemorrhage
Features and management of Juvenile myoclonic epilepsy?
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
what is internuclear opthalmoplegia?
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
Featres of internuclear ophthalmoplegia?
impaired adduction of the eye on the same side as the lesion
horizontal nystagmus of the abducting eye on the contralateral side
Causes of internuclear ophthalmoplegia?
multiple sclerosis
vascular disease
What anti-epileptic causes peripheral neuropathy?
Phenytoin
Normal values of CSF?
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
What conditions are associated with raised CSF lymphocytes?
viral meningitis/encephalitis
TB meningitis
partially treated bacterial meningitis
Lyme disease
Behcet’s, SLE
lymphoma, leukaemia
what is Anti-yo antibody associated with?
paraneoplastic syndrome
associated with ovarian and breast cancer
patients have a cerebellar syndrome
Anti Hu - what is it assoictaed with?
associated with small cell lung carcinoma and neuroblastomas
sensory neuropathy - may be painful
cerebellar syndrome
encephalomyelitis
What is a vestibular schwannoma?
Vestibular schwannomas (sometimes referred to as acoustic neuromas) account for approximately 5% of intracranial tumours and 90% of cerebellopontine angle tumours.
Features of vestibular schwannoma?
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
What are bilateral vestibular schwannomas seen in ?
neurofibromatosis type 2.
What is Friedeich’s ataxia?
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.
features of Friedreich’s ataxia?
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
Friedreich’s ataxia vs ataxic telangiectasia?
what may trigger cluster headache?
Alcohol may trigger an attack and there also appears to be a relation to nocturnal sleep.
Features of cluster headache?
-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
Investigations for cluster headache?
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
what is the management of cluster headaches?
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
What is Von Hippel-Linday syndrome?
How is it inherited?
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
What are the features of Von Hippel Lindau syndrome?
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
What is hereditary sensorimotor neuropathy ?
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
Features of HSMN type 1?
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
What is normal pressure hydrocephalus?
Normal pressure hydrocephalus is a reversible cause of dementia seen in elderly patients. It is thought to be secondary to reduced CSF absorption at the arachnoid villi. These changes may be secondary to head injury, subarachnoid haemorrhage or meningitis.
Symptoms of normal pressure hydrocephalus?
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.
Investigations and management for normal pressure hydrocephalus?
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
First line management for myoclonic seizures?
males: sodium valproate
females: levetiracetam
Common trinucleotide repeat disorders?
CAG - Huntingtons
GAA - Friedrich Ataxia
CTG - myotinic dystrophy
CGG - fragile X syndrome
Huntingtons genetics?
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
Features of Huntingtons?
Features typical develop after 35 years of age
chorea
personality changes (e.g. irritability, apathy, depression) and intellectual impairment
dystonia
saccadic eye movements
What is subacute combined degeneration of the spinal cord?
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.
what may precipitate subacute combined degeneration of the spinal cord?
giving folate to a patient deficient in B12 can precipitate subacute combined degeneration of the cord
Always replace B12 before folare
Features of subacute combined degeneration of the spinal cord?
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
What is Gerstmann’s syndrome?
(lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
Parietal lobe lesions may cause Gerstmann’s syndrome
Features of extra dural haematoma?
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
Features of subdural haematoma?
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
Features of intracerebral haematoma?
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.
What is the cushing reflex?
the Cushings reflex (hypertension and bradycardia) often occurs late following head injury and is usually a pre terminal event
What is Asteregnosis?
where would a lesion be in the brain for patient to have asteregnosis?
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.
Diagnostic manoevure for BPPV?
Manoeuvre for treatment of BPPV?
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)
what factors would and would not contribute to headache?
Management of headache post lumbar puncture?
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
What is mutiple system atrophy?
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
What are the features of multiple system atrophy?
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
Mechanism of action of baclofen?
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)
Symptoms of brachial neuritis?
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.
Causes of Hingival hyperplasia?
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)
What are the features of narcolepsy?
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
What HLA is Narcolepsy associated with ?
HLADR2
A low level of what neuropeptide is associated with narcolepsy?
it is associated with low levels of orexin (hypocretin), a protein which is responsible for controlling appetite and sleep patterns
Managment of narcolepsy?
daytime stimulants (e.g. modafinil) and nighttime sodium oxybate
what does a painful third nerve palsy suggest?
Posterior communicating artery aneurysm
what medication has been shown to prolong life in MND?
prevents stimulation of glutamate receptors
used mainly in amyotrophic lateral sclerosis
prolongs life by about 3 months
Indications for disease mdifying drugs in MS?
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)
What are the drug options reducing the risk of relapse in MS?
Natalizumab
Ocrelizumab
Fingolimod
Beta interferon
Glatiramer acetate
What is Natalizumab?
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
How do you manage spasticity in MS?
baclofen and gabapentin are first-line. Other options include diazepam, dantrolene and tizanidine
physiotherapy is important
cannabis and botox are undergoing evaluation
How do you manage fatigue in MS?
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
How do you manage bladder dysfunction in MS?
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
What kind of drugs of are triptas?
specific serotonin (5-HT1) receptor agonists
How do you manage Oscillopsia in MS?
Oscillopsia (visual fields appear to oscillate)
gabapentin is first-line
why do patients who have surgery myelopathy require follow up?
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.
vertical gaze palsy
Horizontal gaze palsy
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)
What is Parinaud syndrome?
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
Which antibodies is associated with ocular opsoclonus-myoclonus in patients with breast cancer?
Anti Ri
also known as anti-neuronal nuclear antibody type 2 or ANNA-2
What is Opsoclonus-myoclonus syndrome ?
Opsoclonus-myoclonus syndrome is characterised by rapid, irregular, multidirectional eye movements (opsoclonus), involuntary muscle jerking (myoclonus), and ataxia.
What is the difference between cervical spondylitic myelopathy and degenerative cervical myelopathy?
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.
Features of cervical spondylitic myelopathy ?
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
you get a lower motor neuron lesion at the level of the compression and UML below. cervical
What is cervical spondylitic myelopathy?
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
What is Vigabatrin?
What are the side effects?
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
What is acute angle closure glaucoma?
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
what are the adverse effects of Topiramate?
reduced appetite and weight loss
dizziness
paraesthesia
lethargy and poor concentration
rare but important: acute myopia and secondary angle-closure glaucoma
Features of acute closed angle glaucoma?
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
Drug causes of peripheral neuropathy?
amiodarone
isoniazid
vincristine
nitrofurantoin
metronidazole
Management of medication overuse headache?
simple analgesics and triptans should be withdrawn abruptly (may initially worsen headaches)
opioid analgesics should be gradually withdrawn
Transient global amnesia vs transient epileptic amnesa?
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.
Features of Progressive supranuclear palsy?
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
Mechanism of action of ondasetron?
5HT3 antagonists
what is Erb Duchenne paralysis?
Erb-Duchenne paralysis
damage to C5,6 roots
winged scapula
may be caused by a breech presentation
What is Klumpke’s paralysis?
damage to T1
loss of intrinsic hand muscles
due to traction
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.
