neuro Flashcards
Lambert-Eaton myasthenic syndrome
- associated with small cell lung cancer (also breast and ovarian)
- antibody directed against pre-synaptic voltage gated calcium channel in the peripheral nervous system
- can also occur independently as autoimmune disorder
improves with reinforcement unlike MG
Anti-Hu
- associated with small cell lung carcinoma and neuroblastomas
- sensory neuropathy - may be painful
- cerebellar syndrome
- encephalomyelitis
Anti-Yo
- associated with ovarian and breast cancer
* cerebellar syndrome
Anti-GAD antibody
- associated with breast, colorectal and small cell lung carcinoma
- stiff person’s syndrome or diffuse hypertonia
Anti-Ri
- associated with breast and small cell lung carcinoma
* ocular opsoclonus-myoclonus
Purkinje cell antibody
- peripheral neuropathy in breast cancer
restless leg syndrome causes and associations
+ Mx
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
Trigeminal neuralgia
first line treatment carbamazepine
CADASIL
cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy
rare cause of multi-infarct dementia
patients often present with migraine
Syringomyelia
cape like loss of pain and temperature sensation (spinothalamic tract decussating at the anterior white commisure of the spine
increase in protein in CSF
Guillain-Barre syndrome
tuberculous, fungal and bacterial meningitis
Froin’s syndrome*
viral encephalitis
*describes an increase in CSF protein below a spinal canal blockage (e.g. tumour, disc, infection)
rapid onset dementia and myoclonbus
creutzfeld jakob disease
drug induced parkinsons treatment for tremor
Benzhexol (anti muscarinic) is now more commonly referred to as trihexyphenidyl. It is now mainly used for drug-induced parkinsonism rather than idiopathic Parkinson’s disease
down or upbeat nystagmus
Upbeat nystagmus
cerebellar vermis lesions
Downbeat nystagmus - foramen magnum lesions
Arnold-Chiari malformation
Palatal myoclonus
olivary nucleus degeneration
Palatal myoclonus is a specific feature of hypertrophic olivary degeneration. This is caused by a lesion in the triangle of Guillain and Mollaret (triangle linking the inferior olivary nucleus, red nucleus and the contralateral dentate nucleus). An MRI brain is the gold standard imaging for this lesion.
Miller Fisher syndrome
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
Migraine
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)
SAH when to LP
after 12 hours
tuberous sclerosis features
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
hyperacusis
sensitivity to noise - stapedius muscle after facial nerve injury
after a stroke
thrombolyse within 4.5 hours IF haemorrhage has been excluded
Mx of lambert eaton
Management
treatment of underlying cancer
immunosuppression, for example with prednisolone and/or azathioprine
3,4-diaminopyridine is currently being trialled**
intravenous immunoglobulin therapy and plasma exchange may be beneficial
Multiple sclerosis acute episode treatment
IV methylprednisolone
Disease modifying MS medications
Beta-interferon has been shown to reduce the relapse rate by up to 30%. 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
MS Symptomatic treatmentes
Fatigue - CBT and amantidine
Spasticity - baclofen and gabapentin
Bladder dysfunction
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
Oscillopsia (visual fields apper to oscillate)
gabapentin is first-line
Temporal lobe lesion
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)
Occipital lobe lesions
homonymous hemianopia (with macula sparing)
cortical blindness
visual agnosia
Parietal lobe lesions
sensory inattention apraxias astereognosis (tactile agnosia) inferior homonymous quadrantanopia Gerstmann's syndrome (lesion of dominant parietal): alexia, acalculia, finger agnosia and right-left disorientation
Frontal lobe lesion
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
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
Guillain barre syndrome monitoring and Rx
monitor resp function with FVC
Management
plasma exchange
IV immunoglobulins (IVIG): as effective as plasma exchange. No benefit in combining both treatments. IVIG may be easier to administer and tends to have fewer side-effects
steroids and immunosuppressants have not been shown to be beneficial
FVC regularly to monitor respiratory function
Chorea causes and common in
Chorea is caused by damage to the basal ganglia, especially the caudate nucleus.
Causes of chorea Huntington's disease, Wilson's disease, ataxic telangiectasia SLE, anti-phospholipid syndrome rheumatic fever: Sydenham's chorea drugs: oral contraceptive pill, L-dopa, antipsychotics neuroacanthocytosis pregnancy: chorea gravidarum thyrotoxicosis polycythaemia rubra vera carbon monoxide poisoning cerebrovascular disease
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.
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.
after 14 days ischaemic stroke
clopidogrel and statin
clopidogrel can be swapped for aspirin/dipyridamole
myasthenic crisis can be ppt by which drugs
The following drugs may exacerbate myasthenia:
penicillamine
quinidine, procainamide
beta-blockers
lithium
phenytoin
antibiotics: gentamicin, macrolides, quinolones, tetracyclines
motor neuron disease drug
Riluzole
prevents stimulation of glutamate receptors
used mainly in amyotrophic lateral sclerosis
prolongs life by about 3 months
subthalamic region lesion
hemiballism 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
loss of muscle tone after laughing
cataplexy
