Neurology Flashcards
Causes of Peripheral Neuropathy?
A – Alcohol B – B12 deficiency C – Cancer and Chronic Kidney Disease D – Diabetes and Drugs (e.g. isoniazid, amiodarone and cisplatin) E – Every vasculitis
What is Charcot-Marie-Tooth disease?
inherited disease that affects the peripheral motor and sensory nerves. Autosomal dominant mutation which causes dysfunction to myelin and the axons
Classical features of Charcot-Marie-Tooth disease?
- Distal muscle wasting and weakness causing “inverted champagne bottle legs”
(hands and ankle dorsiflexion weakness) - High foot arches (pes cavus)
- Reduced tendon reflexes and muscle tone
- Peripheral sensory loss
Management of Charcot-Marie-Tooth disease?
SUPPORTIVE MDT
Neurologists and geneticists to make the diagnosis
Physiotherapists to maintain muscle strength and joint range of motion
Occupational therapists to assist with activities of living
Podiatrists to help with foot symptoms and suggest insoles and other orthoses to improve symptoms
Orthopaedic surgeons to correct disabling joint deformities
What indicates NF-2?
Bilateral Acoustic Neuromas
What chromosome is NF-1 gene found on and what protein does it code for?
Chromosome 17 - codes for neurofibromin which is a tumour suppressor protein
What chromosome is NF-2 gene found on and what protein does it code for?
Chromosome 22 - codes for Merlin which is a tumour suppressor protein particularly important in Schwann Cells - therefore SCHWANNOMAS develop
What other tumour is NF-2 associated with?
What are the symptoms?
Acoustic Neuromas - tumours of the auditory nerve innervating the inner ear.
Hearing loss
Tinnitus
Balance problems
NF-1 features?
CRABBING. 2/7 of:
C – Café-au-lait spots (6 or more) measuring ≥ 5mm in children or ≥ 15mm in adults
R – Relative with NF1
A – Axillary or inguinal freckles
BB – Bony dysplasia such as Bowing of a long bone or sphenoid wing dysplasia
I – Iris hamartomas (Lisch nodules) (2 or more) are yellow brown spots on the iris
N – Neurofibromas (2 or more) or 1 plexiform neurofibroma
G – Glioma of the optic nerve
Complications of NF-1?
GLIMMERS
Gastrointestinal stromal tumour (GIST - sarcoma)
Learning problems / Leukaemia
Increased risk of cancer (BREAST/ brain tumours)
Migraines
Malignant peripheral nerve sheath tumours
Epilepsy
Renal artery stenosis - causing Hypertension
Scoliosis / Spinal cord tumours
+ vision loss (secondary to optic glioma)
Cutaneous features of Tuberous Sclerosis?
ASH LEAF SPOTS- depigmented ‘ash-leaf’ spots which fluoresce under UV light
SHAGREEN PATCHES roughened patches of skin over lumbar spine
adenoma sebaceum (angiofibromas): butterfly distribution over nose
fibromata beneath nails (subungual fibromata)
POLIOSIS is an isolated patch of white hair on the head, eyebrows, eyelashes or beard
café-au-lait spots* may be seen
Neuro features of Tuberous Sclerosis
Epilepsy (infantile spasms/ partial)
Developmental delay / intellectual difficulties
Other features of Tuberous Sclerosis
Rhabdomyomas in the heart
Gliomas (tumours of the brain and spinal cord)
Polycystic kidneys
Lymphangioleiomyomatosis (abnormal growth in smooth muscle cells, often affecting the lungs)
Retinal hamartomas
Lambert Eaton syndrome
What condition are features similar too?
What cancer is it associated too?
Myasthenia Gravis
Small cell Lung cancer
Lambert Eaton syndrome
Treatment?
Amifampridine (allows more acetylcholine to be released)
Other options:
Immunosuppressants (e.g. prednisolone or azathioprine)
IV immunoglobulins
Plasmapheresis
Lambert Eaton syndrome
Features?
Proximal muscle weakness hyporeflexia Diplopia autonomic symptoms: dry mouth, impotence, difficulty micturating Ptosis Dysphagia
Lambert Eaton syndrome
What is it?
Antibodies produced by the immune system against voltage-gated calcium channels
Localising features of focal seizures
Temporal?
HEAD
Hallucinations (auditory/gustatory/olfactory),
Epigastric rising/Emotional,
Automatisms (lip smacking/grabbing/plucking),
Deja vu/Dysphasia post-ictal)
Localising features of focal seizures
Parietal?
Paraesthesia
Localising features of focal seizures
frontal?
jacksonian march
Head/leg movements, posturing, post-ictal weakness
Localising features of focal seizures
Occipital?
Floaters and flashes
L-dopa unwanted effects?
Dyskinesia, Drowsiness, Dry mouth
On-off effect
Palpitations, postural hypotension, psychosis
Anorexia
Dopamine receptor agonist examples?
bromocriptine, ropinirole, cabergoline, apomorphine
Dopamine receptor agonist unwanted effects?
impulse control disorders and excessive daytime somnolence
more likely than levodopa to cause hallucinations in older patients.
Signs of benign essential tremor?
Management of benign Essential Tremor?
- Worse on voluntary movement
- improved by alcohol
- symmetrical
- absent in sleep
Propanolol
Primidone
STROKES
Symptoms for ACA stroke?
Contralateral hemiparesis and sensory loss, lower extremity > upper
STROKES
Symptoms for MCA stroke?
Contralateral hemiparesis and sensory loss, upper extremity > lower
Contralateral homonymous hemianopia
Aphasia
STROKES
Symptoms for PCA stroke?
Contralateral homonymous hemianopia with macular sparing
Visual agnosia
STROKES
What symptoms should be assessed when assessing an anterior circulation infarct?
PACI vs TACI
- unilateral hemiparesis and/or hemisensory loss of the face, arm & leg
- homonymous hemianopia
- higher cognitive dysfunction e.g. dysphasia
(Total anterior circulation (ACA+MCA) = all 3)
STROKE
Lacunar infarct symptoms?
presents with 1 of the following:
- unilateral weakness (and/or sensory deficit) of face and arm, arm and leg or all three.
- pure sensory stroke.
- limb ataxia
STRONG ASSOCIATION WITH HYPERTENSION
Lateral Medullary syndrome involves what artery?
Posterior inferior cerebellar artery
Associated effects to Lateral Medullary Syndrome?
Ipsilateral: facial pain and temperature loss
Contralateral: limb/torso pain and temperature loss
Ataxia, nystagmus
lateral pontine syndrome involves what artery?
Anterior inferior cerebellar artery
Associated effects to Lateral Pontine Syndrome?
Ipsilateral: facial paralysis and deafness
Stroke in opthalamic artery is associated with what?
Amaurosis Fugax
Ramsey Hunt Syndrome
what is it?
features?
unilateral lower motor neurone facial nerve palsy caused by varicella zoster virus (VCV)
- Vesicular rash on pinna/ around the ear and sometimes on the anterior 2/3 of the tongue
What should be prescribed for LMN palsys as well as prednisolone?
Lubricating eye drops
Reflex test for Cervical Myelopathy?
Hoffman’s sign:
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.
Valproate adverse effects?
Valproate Appetite and weight gain Liver failure Pancreatitis Reversible hair loss Oedema Ataxia Tremor + teratogenicity + thrombocytopenia Encephalopathy (hyperammonemic) - L - cartinine may be used as treatment if this develops
P450 inhibitor
gastrointestinal: nausea
hyponatraemia
Cavernous sinus thrombosis
What is it a complication of?
Features?
Sinusitis
- unilateral facial oedema
- photophobia
- proptosis (Exophthalmos)
- palsies of the cranial nerves which pass through it (III, IV, V, VI).
Brain abscess features?
1) Headache
2) Fever
3) Focal Neurology (e.g. CN3/ CN6 palsy secondary to raised ICP)
Management of Brain abscess?
Surgery (craniotomy)
IV 3rd-generation cephalosporin + metronidazole
most common complication of bacterial meningitis?
Sensorineural hearing loss
Antibiotic prophylaxis of meningitis contacts?
ciprofloxacin is now preferred over rifampicin
MENINGITIS MANAGEMENT
Initial empirical therapy aged < 3 months?
Intravenous cefotaxime + amoxicillin (or ampicillin)
MENINGITIS MANAGEMENT
Initial empirical therapy aged 3 months- 50 years?
Intravenous cefotaxime (or ceftriaxone)
MENINGITIS MANAGEMENT
Initial empirical therapy aged > 50 years
Intravenous cefotaxime (or ceftriaxone) + amoxicillin (or ampicillin)
MENINGITIS MANAGEMENT
Tx for Meningococcal meningitis?
Intravenous benzylpenicillin or cefotaxime (or ceftriaxone)
MENINGITIS MANAGEMENT
Tx for Pneuomococcal meningitis/ caused by Haemophilus influenzae?
Intravenous cefotaxime (or ceftriaxone)
MENINGITIS MANAGEMENT
Tx for meningitis caused by Listeria?
Intravenous amoxicillin (or ampicillin) + gentamicin
Syringomyelia classic presentation?
cape-like loss of pain and temperature sensation due to compression of the spinothalamic tract fibres
Treatment of Raised intracranial pressure?
investigate and treat the underlying cause
head elevation to 30º
IV mannitol may be used as an osmotic diuretic
controlled hyperventilation
removal of CSF, different techniques include:
drain from intraventricular monitor (see above) repeated lumbar puncture (e.g. idiopathic intracranial hypertension) ventriculoperitoneal shunt (for hydrocephalus)
Raised ICP features?
Cushing’s triad
widening pulse pressure
bradycardia
irregular breathing
Causes of raised ICP?
HIT HIM
head injuries
idiopathic intracranial hypertension
tumours
hydrocephalus
infection
meningitis
Normal Intracranial pressure?
the normal ICP is 7-15 mmHg in adults in the supine position
DCM symptoms?
Pain in neck, upper and lower limbs loss of dexterity e.g buttons, holding cutlery incontinence numbness Hoffmans sign
Gold standard dx for DCM?
Treatment?
MRI of cervical spine
Surgery needed within 6 months
Who classically gets Intracranial Hypertension
Risk factor drugs?
Young overweight females
COCP, Tetracyclines, Steroids, Lithium
Features of Intracranial hypertension?
Headache with blurred vision and papilloedema - enlarged blind spot
Management of Intracranial Hypertension?
Weight loss
Acetazolamide
