Neuro Conditions Flashcards
1
Q
Bell’s Palsy
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
- Management
- Complications
- Prognosis
A
- Idiopathic lower motor neurone facial nerve palsy
- Idiopathic, 60% preceded by URTI, suggesting viral/post-viral aetiology
- Most cases 20-50yrs
- Prodrome of pre-auricular pain; followed by unilateral facial weakness and droop, max severity = 1-2d; 50% experience facial, neck or ear pain or numbness; hyperacuisis due to stapedius paralysis; loss of taste; tearing or drying of exposed eye
- LMN weakness of facial muscles, affecting ipsilateral muscles of facial expression, doesn’t spare the muscles of the upper part of the face; Bell’s phenomenon = eyeball rolls up but eye remains open when trying to close their eyes; sensation is normal despite reporting numbness; examine ears to check for causes of facial nerve palsy
- Usually unnecessary; EMG may show local axonal conduction block
- Protection of cornea with protective glasses/patches or artificial tears; high dose corticosteroids useful within 72hrs (only if ramsey-hunt syndrome is excluded; surgery - lateral tarsorrhaphy
- Corneal ulcers, eye infection, aberrant reinnervation (blinking may cause contraction of angle of mouth, crocodile tears syndrome causes tearing whilst salivating
- 85-90% recover function within 2-12 weeks with/out treatment
2
Q
Cluster Headache
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx and Sx
- Investigation
A
- Characterised by recurrent, severe headaches on one side of the head, typically the eye, recurring over a period of several weeks
- Unknown aetiology; genetic factor implicated
- More common in med, usually occurs between 20-40yrs
- 2 types = Episodic (periods lasting 7d-1yr, separated by pain free periods lasting a month or longer, clusters last between 2-3wks), chronic (occurring for 1yr without emission/short lived remissions of less than 1month, arising de novo or from episodic ones)
- Pattern of occurrence = bouts lasting 6-12wks; once every 1/2yrs, at the same time every year; typically at night 1-2hrs after falling asleep
- Nature of syx = Pain comes on rapidly over ~10min; pain is intense, sharp and penetrating, centered around the eye/temple/forehead and unilateral, lasting about 45-90min; pain occurs 1x/2x daily; autonomic features = ipsilateral lacrimation, rhinorrhoea, nasal congestion, eye lid swelling, facial swelling, flushing, conjunctival injection, partial Horner’s; pts will pace around
- Triggers = alcohol, exercise and solvents, sleep disruption
- Clinical dx based on hx, neuro exam may be useful
3
Q
Dementia
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx and Sx
- Investigation
A
- Chronic and progressive deterioration of cognitive function due to organic brain disease; irreversible and consciousness isn’t impaired
- Different types:
- Alzheimer’s 50% = degen of cerebral cortex, with cortical atrophy and reduction in ACh production
- Vacular dementia 25% = brain damage due to several incidents of Cerebrovascular disease (strokes/TIAs);
- Lewy body dementias 15% = deposition of abnormal proteins within the brain stem and neocortex
- Frontotemporal dementia = specific dege of frontal and temporal loves
- Prevalence increases with age and 20% prevalence in pts >80yrs old
- Alzheimer’s - insidious onset
- Vascular - stepwise decline
- Lewy Body - fluctuating levels of consciousness, hallucinations, falls and parkinsonian syx
- Frontotemporal - behavioural changes and intellectual changes
- All forms of dementia are associated with progressive loss of memory and cognitive function
- Dx based on hx; ensure hypothyroidism, vit B12/folate deficiency, space-occupying lesion, normal pressure hydrocephalus
4
Q
Encephalitis
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
A
- Inflammation of the brain parenchyma
- Commonly due to viral infection = HSV, VZV, mumps, adenovirus, coxsackie, EBV, HIV, japanese encephalitis; non-viral (rare) = syphillis, staph aureus; immunocompromised pts = CMV, toxoplasmosis, listeria; autoimmune/paraneoplastic associated with certain AB
- UK = 7.4/100,000
- Usually self-limiting and mild, subacute onset, headache, fever, vomiting, neck stiffness, photophobia, behavioural changes, drowsiness, confusion, hx of seizures, focal neuro syx (dysphagia/hemiplegia), detailed travel hx
- Reduce consciousness, deteriorating GCS, seizures, pyrexia, sx of meningism = neck stiffness, photophobia, Kernig’s test +ve; sx of raised ICP = Cushing’s response (HTN, bradycardia, irreg breathing), focal neural sx, mmse may reveal cognitive/psych disturbance
- Bloods -> FBC, U+Es, glucose, viral serology, ABG; MRI/CT -> exclude mass lesion, HSV causes oedema of temporal lobe on MRI; Lumbar puncture -> high lymphocytes/monocytes/protein, glucose normal, viral PCR; EEG -> epileptiform activity; brain biopsy rarely needed
5
Q
Epilepsy
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- History
- Presenting syx
- Presenting Sx
- Investigation
- Management
- Complications
- Prognosis
A
- Tendency to recurrent unprovoked seizures; >2 seizures for epilepsy to dx; seizures = paroxysmal synch cortical electrical discharges, types of seizures =
- focal = localised to specific cortical regions, divided into complex (consciousness affected) and simple (consciousness NOT affected)
- Generalised = affecting whole of the brain, consciousness; types inc. tonic-clonic, absence, myoclonic, atonic, tonic
- Most idiopathic; 1ry epilepsy syndromes; 2ry seizures = tumour, infection (meningitis), inflammation (vasculitis), toxic/metabolic (Na imbalance), drugs (alcohol withdrawal), vascular (haemorrhage), congenital abnormalities, neurodegen diseases, malignant HTN/eclampsia, trauma; look like seizures = syncope, migraine, non-epileptiform seizure disorder; pathophysiology of seizures = imbalance in inhibitory/excitatory currents/NT in brain, precipitants are anything that promotes excitation of the cerebral cortex
- Common, 1% of general popn, typical age onset = children/elderly
- Rapidity of onset, duration of episode, consciousness changed?, tongue biting/incontinence, rhythmic synch limb jerking, post ictal abnormalities (confusion/exhaustion), drug hx
- Focal seizure = frontal lobe focal motor seizure (motor convulsions, Jacksonian march, Todd’s paralysis), temporal lobe seizures (aura, hallucinations), frontal lobe complex partial seizure (loss of consciousness, involuntary actions/disinhibition, rapid recovery); Generalised = Tonic-clonic (vague syx before attack, tonic (generalised muscle spasms) then clonic phase (repetitive synch jerks), faecal/urinary incontinence, tongue biting, post-ictal - impaired consciousness, lethargy, confusion, headache, back pain, stiffness), absence (childhood onset, loss of consciousness but maintains posture, pts stops talking and stares into space for a few seconds, no post-ictal phase), non-convulsive status epilepticus (acute confusional state, often fluctuating, difficult to distinguish from dementia)
- Normal between seizures
- Bloods -> FBC, U+Es, LFT, glucose, Ca, Mg, ABG, toxicology screen, prolactin; EEG -> helps to confirm dx, helps classify epilepsy; CT/MRI -> structural, space occupying or vascular lesions; other due to 2ry causes
- Status epilepticus tx = initiated early, ABC approach, glucose check, IV lorazepam or IV/PR diazepam (repeat again after 10 min if seizure doesn’t terminate) - IV phenytoin considered if seizures recurr after next dose, consider also GA, or treat the cause, check plasma levels of anticonvulsants; Newly dx epilepsy tx = Start anti-convulsants after >2 unprovoked seizures, focal = lamotrigine/carbamazepine, generalised = Na valproate, only ONE drug (others inc. phenytoin, levetiracetam, clobazam, topiramate, gabapentin, vigabatrin); Pt education = avoid triggers, use seizure diaries, women of child-bearing age (!), drug interactions
- Fx from tonic-clonic seizures, behavioural problems, sudden death in epilepsy, of drugs = gingival hypertrophy (phenytoin), neutropaenia and osteoporosis (carbamazepine), Stevens-johnson syndrome (lamotrigine)
- 50% remission at 1yr
6
Q
Guillain-Barre Syndrome
- Definition
- Aetiology/Risk Factors
- Epidemiology
- Presenting Syx
- Presenting Sx
- Investigations
A
- Acute inflammatory demyelinating polyneuropathy
- Inflammatory process where Ab after recent infection react with self-ag on myelin/neurons, with no aetiological trigger; other = post-infection (1-3wks, bacterial, HIV, Herpes), malignancy (lymphoma), post-vaccination
- 1-2/100,000 UK; all age groups
- Progressive; <1month duration of ascending symmetrical limb weakness (lower>upper), ascending paraesthesia; cranial nerve involvement leading to dysphagia, dysarthria, facial weakness; resp muscles affected in severe cases; Miller-Fisher variant (rare) = opthalmoplegia, ataxia, arreflexia
- General motor = hypotonia, flaccid paralysis, arreflexia (from feet to head); Sensory = impairment of sensation in multiple modalities; cranial nerve palsies = facial nerve weakness, abnormality of external ocular movements, pupil constriction affected = botulism; Type II resp failure = due to paralysis of resp muscles; autonomic function = postural BP and arrhythmia assessed
- Lumbar puncture = high protein, normal cell count and glucose; nerve conduction study = reduced velocity; bloods = anti-ganglioside Ab in miller-fisher variant +25% of other cases; spirometry = reduced FVC; ECG = arrhythmias may develop
7
Q
Horner’s syndrome
- Definition
- Aetiology/Risk Factors
- Epidemiology
- Presenting Syx
- Presenting Sx
- Investigations
- Management
- Complications
- Prognosis
A
- Condition resulting from the disruption of the SNs nerves supplying the face resulting in a triad of= ptosis, miosis, anhydrosis (and enopthlamos)
- Caused by disruption of SNS nerves; causes = strokes, MS, apical l,ung tumours, lymphadenopathy, basal skull tumours, carotid artery dissection, neck trauma
- RARE, important sign associated with various diseases
- Inability to open eye fully on affected side, loss of sweating on affected side, facial flushing, orbital pain/headache and others based on cause
- Ptosis, anhydrosis, miosis, enopthalmos
- Directed to finding underlying cause = CXR, CT/MRI, CT angiography
- Mx depends on cause
- depends on cause
- depends on cause
8
Q
Huntington’s disease
- Definition
- Aetiology/Risk Factors
- Epidemiology
- Presenting Syx
- Presenting Sx
- Investigations
A
- Autosomal dominant trinucleotide repeat disease characterised by progressive chorea and dementia, typically in middle age
- Huntingtin gene has triplet expansion of CAG resulting in toxic gain of function; Dominant; earlier age of onset with each generation
- 30-50yrs age of onset
- FHx, insidious onset in middle age, progressive. fidgeting, clumsiness, involuntary, jerky, dyskinetic movements, accompanied by grunting/dysarthria; early cognitive changes = lability, dysphoraia, mental inflexibility, anxiety, develops into dementia; Later stages = rigid, akinetic, bed-bound; Ask about drug Hx (esp. cocaine and anti-psychotics)
- Chorea, dysarthria, slow voluntary saccades, supranuclear gaze restriction, parkinsonism, dystonia, MMSE shows cognitive and emotional deficits
- Genetic analysis - >39CAG, reduced penetrance leads to immediate n# of CAG repeats; imaging = brain MRI/CT may show symmetrical atrophy of the striatum and butterfly dilation of lateral ventricles; bloods = to exclude other pathology
9
Q
Hydrocephalus
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx
- Presenting Sx
- Investigations
A
- Enlargement of the cerebral ventricular system -> subdivided into obstructive and non-obstructive
- Hydrocephalus ex vacuo - apparent enlargement of the ventricles as a compensatory change due to brain atrophy
- Caused by:
- Obstructive: impaired outflow of the CSF from the ventricular system - lesions of 3/4th ventricle or cerebral aqueduct, posterior fossa lesions (tumour) compressing 4th ventricle or cerebral aqueduct stenosis
- Non-obstructive: impaired CSF reabsorption into the subarachnoid villi; tumours, meningitis, normal pressure hydrocephalus (idiopathic chronic ventricular enlargement = gait and cognitive decline)
- Bimodal age distribution; young for congenital malformations and brain tumours; elderly for strokes and tumours
- Obstructive= acute drop in conscious level, diplopia; normal pressure hydrocephalus = Dementia, gait disturbance and urinary incontinence
- Obstructive: Low gcs, papilloedema, 6th nerve palsy (neonates: increased head circumference, sunset sign); normal pressure: cognitive impairment, gait apraxia (shuffling), hyperreflexia
- CT head; CSF (ventricular drain/LP, may indicate pathology, check MC+S, protein and glucose); LP (contraindicated if raised ICP) and therapeutic in normal pressure
10
Q
Meningitis
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx
- Presenting Sx
- Investigations
- Management
- Complications
- Prognosis
A
- Inflammation of the leptomeningeal coverings of the brain, mostly due to infection
- RF: close communities, basal skull fractures, mastoiditis, sinusitis, inner ear infections, alcoholism, immunodeficiency, splenectomy, sickle cell anaemia, CSF shunts, intracranial surgery. 4 types
- Bacterial:
- Neonates: group B strep, e coli, listeria monocytogenes
- Children: H. influenzae, N meningitidis, strep pneumo
- Adults: N meningitidis, strep pneumo, TB
- Elderly: strep pneumo, listeria monocytogenes
- Viral: Enteroviruses, mumps, HSV, VZV, HIV
- Fungal: cryptococcus (common cause in HIV)
- Others: aseptic meningitis, mollaret’s meningitis (recurrent benign lymphocytic meningitis
- Bacterial:
- 2500 per year nofications
- Severe headaches, photophobia, stiff neck/backache, irritability, drowsiness, vomiting, high pitched crying/fits, reduced consciousness, fever; Good Hx of travel and exposure to: rodents, ticks, mosquitoes, sexual activity
- Photophobia, neck stiffness, kernig’s sign (hips flexed, pain/resistence on passive knee extension) and brudzinski’s sign (neck flexion leads to hip flexion at the same time); sx of infection: fever, tachycardia, hypotension, skin rash, altered mental state
- Bloods (2 blood cultures), imaging (CT scan, exclude raised ICP before LP), LP (MC+S, bacterial = cloudy CSF, high neutrophils/protein, low glucose; TB meningitis = fibrinous CSF, high lymphocytes/protein, low glucose)
- Immediate IV ABx before LP (3rd gen cephalosporins, and benzylpenicillin may be used for initial blind therapy), Dexamethasone IV (shortly before/with 1st dose of ABx, reduced risk of complications), resus (manage in ITU, notify public heallth services)
- Septicaemia, shock, DIC, renal failure, seizures, peripheral gangrene, cerebral oedema, cranial nerve lesions, cerebral venous thrombosis, hydrocephalous, Waterhouse-friderischen syndrome (bilat adrenal haemorrhage)
- Mortality rate from bacterial (10-40%), viral is self limiting
11
Q
Migraine
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
- Management
- Complications
- Prognosis
A
- Severe episodic headache that may have a prodrome of focal neurological syx (aura) and is asociated with systemic disturbance
- Migraine with aura (classical)
- Migraine without aura (common)
- Migraine variants (familial hemiplegic, ophthalmoplegic)
- Poorly understood; early aura of cortical spreading depression is associated with intracranial vasoconstriction leading to localised ischaemia; this is then followed by meningeal and extracranial vasodilation mediated by serotonin, bradykinin and the trigeminovascular system
- Prevalence = 6% M, 15-20% F; usually in adolescence and early adulthood
- 3 main things:
- Headache = pulsatile, duration 4-72hrs, episodic (chronic suggests different aetiology)
- Associated syx = nausea, vomiting, photophobia/phonophobia, aura: flashing lights, spots, blurring, zig-zag lines, blind spots, tingling/numbness in the limbs
- Triggers/RF = stress, exercise, lack of sleep, oral COCP, foods (caffeine, alcohol, cheese, chocolate)
- No specifical physical findings; exclude secondary causes with MMSE, neurological examination, fundoscopy, etc
- Dx based on Hx, investigations may be useful for excluding other dx; bloods, CT/MRI, LP
- Analgesia overuse can cause headaches;
- Acute = NSAIDs, paracetamol, codeine, antiemetis, triptans (5-HT agonists) like sumatriptan
- Prophylaxis = b-blockers, amitriptyline, topiramate, Na valproate, menstrual migraines can be controlled with COCP
- Advice = avoid triggers, rest in a quiet dark room during episodes
- Disruption of daily actvities; can lead to analgesia-overuse headaches in people who use analgesia regularly
- Usually chronic; most cases can be managed well with preventative/early treatment measures
12
Q
Motor neurone disease
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
A
- Progressive neurodegenerative disorder of cortical, brainstem and spinal motor neurons (lower and upper motor neurones)
- Amyotrophic lateral sclerosis -> combined generation of U AND LMN resulting in mix of U/LMN signs
- Progressive muscular atrophy variant -> only LMN sx, better prognosis
- Progressive bulbar palsy variant -> dysarthria, dysphagia, wasted fasciculating tongue, brisk jaw jerk reflex
- Primary lateral sclerosis variant -> UMN pattern of weakness, brisk reflexes, extensor plantar responses, NO LMN sx
- UNKNOWN; free radical damage and glutamate excitotoxicity have been implicated; pathology: progressive motor neurone degeneration and death, gliosis replacing lost neurones; associations: frontaltemporal lobar dementia
- Rare; incidence 2/100,000; mean age of onset: 55yrs; 5-10% have a FHx with autosomal dominant inheritance
- Weakness of limbs, speech disturbance (slurring/reduction in volume), swallowing disturbance (choking on food), behavioural changes (disinhibition, emotional liability)
- Combination of UMN/LMN; LMN = muscle wasting, fasciculations, flaccid weakness, hyporeflexia; UMN = spastic weakness, extensor plantar response, hyperreflexia; sensory examination - normal
- Bloods = mild elevation in CK, ESR, anti-GM1 ganglioside antibodies; EMG; Nerve conduction studies (normal), MRI (exclude cord compression and brainstem lesiions), spirometry (assess respiratory muscle weakness)
13
Q
Multiple Sclerosis
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
A
- Inflammatory demyelinating disease of CNS
- Relapsing remitting MS: commonest form, clinical attacks of demyelinations with complete recovery in between attacks
- Clinically isolated syndrome - single clinical attack of demyelination, which doesn’t count as Ms, but 10-50% progress to develop MS
- 1ry progressive MS = steady accumulation of disability wth no relapsing/remitting pattern
- Marburg variant = severe fulminant variant of MS leading to advanced disability or death within weeks
- Unknown, Autoimmune basis with potential environmental trigger in genetically susceptible individuals; immune mediated damage to myelin sheaths results in impaired axonal conduction; RF - EBV exposure, prenatal vitamin D levels
- Uk prevalence 1/1000, 2x as common in females, age of presentation 20-40
- varies depending on site of inflammation
- Optic neuritis (commonest) = unilateral deterioration of visual acuity and colour perception, pain on eye movement, common 1st syx of MS
- Sensory = pins and needles, numbness, burning
- Motor = limb weakness, spasms, stiffness, heaviness
- Autonomic = urinary urgency, hesitancy, incontinence, impotence
- Psychological = depression, psychosis
- Uhthoff’s sign - worsening of neurological symptoms as the body gets overheated
- Lhermitte’s sign - electrical sensation that runs down the back and into the limbs when the neck is flexed
- x
- Optic neuritis: impaired visual acuity (most common), loss of coloured vision
- Visual field testing: Central scotoma (blind spot in normal visual field) and field defects
- RAPD
- Internuclear ophthalmoplegia: lateral horizontal gaze causes failure of adduction of the contralateral eye; indicates lesion of the contralateral medial longitudinal fasciculus (eye can’t adduct with the other)
- Sensory paraesthesia
- Motor: UMN sx
- Cerebellar: Limb ataxia (intention tremor, past-pointing, dysmetria), dysdiadokinesia, ataxic wide based gait, scanning speech
- Dx is based on the finding of 2 or more CNS lesions with corresponding syx separated in time and space (McDonald criteria); LP = microscopy and CSF electrodes showing unmatched oligoclonal bands); MRI brain, cervical and thoracic spine, plaques can be identified and gadolinium enhancement shows active lesions; evoked potentials: visual, auditory and somatosensory evoked potentials may show delayed conduction velocity
14
Q
Myasthenia gravis
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
A
- An Autoimmune disease affecting the NMJ producing weakness in skeletal muscles
- Impairment of NMJ transmission; most commonly due to Ab against the nAChR; Lambert Eaton syndrome is a paraneoplastic subtype of myasthenia gravis caused by autoAb against pre-synaptic calcium channels, leading to impairment of ACh release; myasthenia gravis is associated with other AI conditions
- Prevalence 8-9/100,000, most common in females at younger ages, equal gender distribution in middle ages
- Syx:
- Muscle weakness that worsens with repetitive use or towards the end of the day (Lambert-eaton syndrome improves with repeated use);
- ocular syx = drooping eyelids, diplopia;
- bulbar syx = facial weakness (myasthenic snarl), disturbed hypernasal speech, difficulty smiling, chewing/swallowing
- May be generalised/bulbar(CN9/10/11/12)/ocular
- Eye signs: ptosis, complex ophthalmoplegia, check for ocular fatigue by pt sustaining upward gaze for 1 min and watch as ptosis develops
- Ice on eyes test = placing ice packs on closed eyelids for 2 mins can improve NM transmission and reduce ptosis
- Bulbar signs = reading aloud may cause dysarthria or nasal speech
- Limbs = test the power of a muscle before and after repeated use of the muscle
- x
- Bloods: CK, serum ACh receptor Ab, TFTs, anti-voltage gated Ca channel ab (LES)
- Tensilon test: short acting anti-cholinesterase increases Ach levels and causes a rapid and transient improvement in clinical features, risk of bradycardia (generally avoided)
- Nerve conduction study: repetitive stimulation shows decrements of muscle action potential
- EMG
- CT thorax/CXR - visualise thymoma in the mediastinum or lung malignancies
15
Q
Neurofibromatosis
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
A
- Autosomal dominant genetic disorder affecting cells of neural crest origin, resulting in the development of multiple neurocutaneous tumours
- Type 1 Neurofibromatosis -> peripheral spinal neurofibromas, multiple cafe au lait spots, freckling (axillary/inguinal), optic nerve glioma, lisch nodules (on iris), skeletal deformities, phaeochromocytomas, renal artery stenosis
- Type 2 Neurofibromatosis -> schwannomas (bilat vestibular schwannomas), meningiomas, gliomas, cataracts
- Associated with multiple mutations in tumour suppressor genes NF1 and 2
- No gender/racial predilection
- Positive FHx (50% by new mutations); type:
- skin lesions, learning difficulties, headaches, disturbed vision, precocious puberty
- Hearing loss, tinnitus, balance problems, headache, facial pain, facial numbness
- Type:
- 5+ cafe au lait macules of >5mm (prepubertal)/>15mm (postpubertal); neurofibromas, freckling in armpit/groin, lisch nodules (hamartomas on iris), spinal scoliosis
- Few/no skin lesions, sensorineural deafness with facial nerve palsy or cerebellar signs
- Ophthalmological assessment, audiometry, MRI brain and spinal cord (for vestibular schwannomas, meningiomas and nerve root fibromas), skull XR (sphenoid dysplasia in NF1), genetic testing
16
Q
Parkinson’s disease
- Defintion
- Aetiology/Risk Factors
- Epidemiology
- Presenting syx
- Presenting Sx
- Investigation
A
- Neurodegenerative disease of the dopaminergic neurones of the substantia nigra characterised by bradykinesia, rigidity, resting tremor, postural instability
- Pathophysiology = degen of DA neurones projecting from substantia nigra to the striatum; only syx after >70% loss of DA neurones;
- Sporadic/idiopathic PD: most common, aetiology unknown, may be related to env toxins and oxidative stress
- 2ry PD: neuroleptic therapy, vascular insults, MPTP toxin from illicit drug contamination, post-encephalitis, repeated head injury
- Very common, 1-2% of >60yrs, mean age at 57
- Insidious onset, resting tremor (hands), stiffness and slowness of movements, difficulty initiating movement, frequent falls, micrographia, insomnia, mental slowness
- x
- Tremor = pill rolling rest, 4-6Hz, decreased on action, usually assymmetrical;
- rigidity = lead pipe rigidity of muscle tone, superimposed tremor (cogwheel rigidity), rigidity can be enhanced by distraction;
- Gait = stooped, shuffling, small-stepping gait, reduced arm swing, difficulty initiating walking
- postural instability = falls easily with little pressure
- Other features = frontalis overactivation, hypomimic face, soft monotonous voice, immpaired olfaction, tendency to drool, mild impairment of up-gaze
- Psychiatric = depression, cognitive problems and dementia
- Clinical dx, Levodopa trial (timed walking and clinical assessment), bloods (serum caeruloplasmin - rule out wilson’s disease as a cause of PD), CT/MRI brain (exclude other causes of gait decline); DA transporter scintigraphy (reduction in striatum and putamen
17
Q
Spinal Cord compression
- Definition
- Aetiology/RF
- Epidimiology
- Presenting syx
- Presenting sx
- Investigations
A
- Injury to the spinal cord with neurological syx dependent on the site and extent of the injury
- Most cases: trauma/tumours; trauma: compression by direct cord contusion, compression by bone fragments, haematoma, acute disk prolapse; tumours more frequently metastases; other causes: spinal abscess, TB
- RF = Trauma, OP, metabolic bone disease, vertebral disc disease
- Common, trauma, occurs across all age groups, malignancy/disc disease is more common in elderly
- Hx of trauma/malignancy, pain, weakness, sensory loss, disturbance of bowel and bladder function
- large central lumbar disc prolapse may cause: bilateral sciatica, saddle anaesthesia, urinary retention
- Diaphragmatic breathing, reduced anal tone, HYPOREFLEXIA, priapism (persistent/painful erection), spinal shock (low BP without tachycardia); sensory loss at level of lesion; motor: weakness or paralysis, downward plantars in acute phase, UMN signs below the level of the lesion, LMN signs at the level of the lesion; Brown-sequard syndrome seen with hemisection of the spinal cord
- Radiology: lat radiographs of spine to look for loss of alignment, features etc, MRI/CT; Bloods: FBC, U+E, Calcium, ESR, Ig electrophoresis (multiple myeloma); urine for Bence Jones proteins in Multiple Myeloma
18
Q
Stroke
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx
- Presenting sx
- Investigation
- Management
- Complications
- Prognosis
A
- Rapid permanent neurological deficit from cerebrovascular insult; also defined clinically as focal/global impairment of CNS function developing rapidly and lastiing >24hrs; subdivided on location (ant vs post circulation), pathological process (infarction vs haemorrhage)
- x
- Infarction (80%): thrombosis = can occur in small vessels (lacunar infarcts), larger vessels (middle cerebral artery) and in prothrombotic states (dehydration, thrombophilia); emboli: carotid dissection/atherosclerosis, AF, venous and pass through venous septal defect into cerebral circulation; Hypotension: if bP lower that autoregulatory range required for cerebral blood flow then infarction can occur in watershed zones; others: vasculitis, cocaine
- Haemorrhage (10%): HTN, charcot bouchard microaneurysm rupture, amyloid angiopathy, arteriovenous malformations, less common: trauma, tumours, vasculitis
- Common, 2/1000, 3rd most common cause of death in industrialised countries; usual age 70+
- Sudden onset, weakness, sensory/visual/cognitive impairment, impaired coordination, impaired consciousness, head/neck pain (carotid/vertebral artery), time of onset (mx <4.5hrs), hx of AF/MI/valvular heart disease/carotid artery stenosis/recent neck trauma/pain
- Underlying cause; Infarction:
- Posterior circulation:
- post cerebral = hemianopia; ant inf cerebellar = vertigo, ipsilateral ataxia/deafness/facial weakness;
- post inf cerebellar (affected in lat medullary syndrome) = vertigo, ipsilateral ataxia/horner’s/hemisensory loss, dysarthria, contralat spinothalamic sensory loss;
- basilar artery = cranial nerve pathoogy and impaired consciousness;
- multiple lacunar infarcts = vascular dementia, urinary incontinence, gait apraxia, shuffling gait, normal/excessive arm swing;
- intracerebral = headache, meningism, focal neuro signs, N/V, sx of raised ICP, seizures
- anterior circulation:
- ant cerebral = lower limb weakness, confusion;
- middle cerebral = facial weakness, hemiparesis, hemisensory loss, apraxia, hemineglect, receptive or expressive dysphasia, quadrantopia
- lacunar infarcts affecting:
- internal capsule/pons = pure sensory/motor/both deficit;
- thalamus = loss of consciousness, hemisensory deficit;
- _basal gangli_a = hemichorea, hemiballismus, parkinsonism
- Posterior circulation:
- Bloods for clotting profile; ECG for arrhythmias; EchoCG for cardiac thrombus, endocarditis and other cardiac sources of embolism; carotid doppler US for carotid disease; CT head scan for haemorrhages; MRI brain for infarction sensitive imaging; CT cerebral angiogram for dissections/stenosis
- x
- Hyperacute = <4.5hrs onset, exclude haemorrhage using head CT, thrombolysis may then be considered
- Acute ischaemic = Aspirin+clopidogrel for further thrombosis, heparin if high risk of emboli recurrence/stroke progression; swallow assessment (NGT for feeding?), GCS monitoring, thromboprophylaxis
- 2ry prevention = aspirin and dipyridamole, warfarin anticoagulation (AF), control RF = HTN, hyperlipidaemia, CAD
- Surgical treatment = carotid endarterectomy
- Cerebral oedema, immobility, infections, DVT, Cardiovascular events, death
- 10% mortality in 1st month, up to 50% survive and are dependent on others; 10% recurrenc in 1 yr; haemorrhagic prognosis worse than ischaemic
19
Q
Subarachnoid haemorrhage
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx
- Presenting sx
- Investigation
A
- Arterial haemorhage into subarachnoid space
- 85% rupture of saccular aneurysm at base of brain (Berry), 10% perimesencephalic haemorrhage, 5% arteriovenous malformations, bleeding diathesis, vertebral artery dissection, RF: HTN, smoking, excess alcohol intake, saccular aneurysms associated with PCKD, marfan’s and ehlers-danlos syndrome
- 10/100,000; peak incidence = 40s
- Sudden onset worse headache ever, N/V, neck stiffness, photophobia, reduced level of consciousness
- Meningism = neck stiffness, kernig’s sign, pyrexia; GCS check for deterioration; sx of raised ICP - papilloedema, IV/III nerve palsies, HTN, bradycardia; focal neuro sx
- Bloods: FBC, U+Es, ESR/CRP, clotting; CT scan hyperdense areas in basal regions of skull due to blood; angiography for bleeding; LP = increased opening pressure, increased red cells, xanthochromia - straw coloured due to breakdown of RBC
20
Q
Subdural haemorrhage
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx
- Presenting sx
- Investigation
- Management
- Complications
- Prognosis
A
- Collection of blood that develops between the surface of the brain and the dura mater; acute <72hrs, subacute = 3-20d, chronic >3wks
- Trauma due to rapid acceleration and deceleration of the brain
- Acute - younger pts/associated with major trauma; more common than extradural haemorrhage; chronic - more common in the elderly
- Acute = hx of trauma with head injury, reduced conscious level; subacute = worsening headache 7-14d after injury and altered mental state; chronic = headache, confusion, cognitive impairment, psych syx, gait deterioration, focal weakness, seizures
- Acute = reduced GCS, ipsilateral fixed dilated pupil, pressure on brainstem = reduced consciousness and bradycardia; chronic = neuro exam may be normal; focal neural sx (3rd nerve palsy)
- CT head and MRI (higher sensitivity)
- x
- Acute = ALS protocol, cervical spine injury awareness, if ICP raised consider osmotic diuresis
- Conservative tx if small
- Surgical - prompt Burr hole/craniotomy
- Chronic - syx = Burr hole/craniotomy and drainage
- Children = percutaneous aspiration via open fontanelle
- Raised ICP, cerebral oedema, herniation, post-op = seizures, recurrence, intracerebral haemorrhage, brain abscess, meningitis, tension pneumocephalus
- Acute = underlying brain injury will affect function; chronic = better outcome than subdural haem, lower incidence of underlying brain injury
21
Q
Tension headache
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx andsx
- Investigation
- Management
- Complications
- Prognosis
A
- The most common type of headache which is coonsidered a normal, everyday headache -> divided into episodic (on <15d per month) or chronic (on >15d per month)
- Exact cause is unclear; well-known triggers = stress/anxiety, squinting, poor posture, fatigue, dehydration, missing meals, bright sunlight, noise; 1ry headaches
- Most common type of headache; more common in women and young adults; most people will experience a tension headache at some point in their lives
- Mild-moderate in severity; pressure/tightness around the head like band; pain tends to be bilat; often a relationship with the neck; can be disabling for few hours but no associated syx; gradual onset, variable duration, usually responsive to OTC medication; examination usually normal
- No investigations necessary
- Episodic: reassurance, address triggers, advice on avoinding meds that can cause medication-induced headaches; simple analgesia (ibuprofen, paracetamol, aspirin); Tricyclic antidepressants may be considered in frequently recurrent episodic tension headaches or chronic tension headaches
- No complications
- Good, not very severe/disabling, recurring
22
Q
Transient Ischaemic attack
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx
- Presenting sx
- Investigation
- Managment
- Complication
- Prognosis
A
- Rapidly developing focal disturbace of brain function of presumed vascular origin that resolves completely within 24h
- Usually embolic (carotid atherosclerosis) but may be thrombotic; emboli can also arise from the heart: AF, mitral valve disease, atrial myxoma; RF = HTN, smoking, DM, hert disease (valvular, ischaemic, atrial fibrillation), peripheral arterial disease, polycythaemia rubra vera, COCP, hyperlipidaemia, alcohol, clotting disorders
- More common with increasing age, more common men, 15% of stroke pts would have experienced a previous TIA
- Any pt presenting with acute neurological syx that resolve completely within 24h should be given 300mg aspirin immediately and assessed urgently within 24h; hx: TIAs usually last 10-15 min, clinical features depend on part of brain affected:
- Carotid territory = unilat, most often affect motor area: weakness an arm, leg or one side of the face, dysarthria, broca’s dysphasia, amaurosis fugax (painless fleeting loss of vision caused by retinal ischaemia)
- Vertebrobasilar territory = homonymous hemianopia (if opthamic cortex is involved); may be bilateral visual impairment; may be hemiparesis, hemisensory syx, diplopia, vertigo, vomiting, dysarthria, dysphagia or ataxia; ask about weakness, facial drooping, gait disturbance, confusion, memory loss, dysarthria or abnormal behaviour, check for simultaneous cardiac syx
- Neurological examination may be normal because the TIA may have resolved by the time you do it; check pulse irregular rhythm, auscultate the carotids to check for bruits
- 1ry care investigations: urinalysis, FBc, U+Es, lipids, LFTs, TSH, ECG (may show AF/previous MI); 2ry care: unenhanced CT may show haemorrhage; investigate for source of emboli: ECG (24hr tape or cardiac monitoring may be considered if paroxysmal AF is suspected), doppler US of carotid and vertebral arteries
- Acute neuro syx that resolve completely within 24hrs should be given 300 mg aspirin immediately and assessed urgently within 24hrs;
- pts confirmed TIA: clopidogrel - 300mg loading dose and 75mg thereafter, high-intensity statin therapy (atorvastatin 20-80mg);
- 2ry prevention: anti-platelets, anti-HTN, lipid-modifying tx, management of AF
- Assessment of future stroke risk in TIA pts: ABCD2 score
- Recurrence and stroke
- Very high risk of stroke in the first month after the TIA and up to 1yr afterwards
23
Q
Trigeminal neuralgia
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx and sx
- Investigation
A
- Neuralgia involving one or more of the branches of the trigeminal nerves, often causing severe pain
- thought to be due to compression of the trigeminal nerve by a loop of artery or vein; 5-10% cases are thought to be due to tumours, MS or skull base abnormalities
- Peak incidence: 50-60yrs, prevalence increases with age, more common in females, some familial component
- Sudden unilat, brief, stabbing pain in the distribution or one or more branches of the trigeminal nerve; recurrent; pain lasts from a few seconds to a couple of mins; periods of remission can vary; some experience preceding syx; pain is described as being shock-like; triggers: vibration, skin contact, brushing teeth, oral intake, exposure to wind
- Dx is clinical; doubt over underlying cause then specialist may request MRI brain
24
Q
Wernicke’s encephalopathy
- Definition
- Aetiology/RF
- Epidemiology
- Presenting Syx
- Presenting sx
- Investigation
A
- The presence of neurological syx caused by biochemical lesions of the CNS following exhausting of vit B (particularly thiamine) reserves
- Main cause: Chronic alcohol consumption resulting in thiamine deficiency by causing: inadequate nutritional thiamine intake, decreased thiamine absorption, imapired thiamine utilisation by cells;
- other conditions causing thiamine deficiency: chronic subdural haematoma, AIDS, hyperemesis gravidarum, thyrotoxicosis;
- thiamine deficiency causes abnormal cell function in cerebral cortex, hypothalamus and cerebellum
- Alcohol related brain damage accounts for 10-24% of all dementia; prevalence rates higher in areas of socioeconomic deprivation; higher prevalence in 50-60yr olds
- Vision changes: diplopia, eye movement abnormalities, ptosis; loss of muscle coordination, unsteady gait; loss of memory; inability to form new memories; hallucinations
- Triad: confusion, ophthalmoplegia and ataxia; pt usually mentally alert with vocab, comprehension, motor skills, social habits and naming ability maintained; some show sx of polyneuropathy; may be hyporeflexic; abnormal gait and coordination; eye abnormalities on movement: nystagmus, bilat lat rectus palsy, conjugate gaze palsy; low temp; rapid pulse; some cachectic
- Korsakoff’s psychosis is after further deterioration with amnesia and confabulation
- Dx mainly based on hx and exam; possible: FBC (high MCV common in alcoholics), U+Es (exclude met imbalance as confusion), LFTs, glucose, ABG (hypercapnia and hypoxia can confuse), serum thiamine; CT head useful
25
Q
Definition
Bells Palsy
A
Idiopathic LMN facial nerve palsy
26
Q
S+S
Bell’s Palsy
A
- Prodrome of pre-auricular pain;
- followed by unilateral facial weakness and droop,
- max severity = 1-2d; 50% experience facial, neck or ear pain or numbness;
- hyperacuisis due to stapedius paralysis;
- loss of taste;
- tearing or drying of exposed eye
- LMN weakness of facial muscles, affecting ipsilateral muscles of facial expression, doesn’t spare the muscles of the upper part of the face;
- Bell’s phenomenon = eyeball rolls up but eye remains open when trying to close their eyes;
- sensation is normal despite reporting numbness;
- examine ears to check for causes of facial nerve palsy
27
Q
Management
Bell’s palsy
A
- Protection of cornea with protective glasses/patches or artificial tears;
- high dose corticosteroids useful within 72hrs (only if ramsey-hunt syndrome is excluded;
- surgery - lateral tarsorrhaphy
28
Q
Definition
Cluster headache
A
Characterised by recurrent, severe headaches on one side of the head, typically the eye, recurring over a period of several weeks
29
Q
S+S
Cluster headache
A
- 2 types =
- Episodic (periods lasting 7d-1yr, separated by pain free periods lasting a month or longer, clusters last between 2-3wks),
- chronic (occurring for 1yr without emission/short lived remissions of less than 1month, arising de novo or from episodic ones)
- Pattern of occurrence =
- bouts lasting 6-12wks;
- once every 1/2yrs, at the same time every year;
- typically at night 1-2hrs after falling asleep
- Nature of syx =
- Pain comes on rapidly over ~10min;
- pain is intense, sharp and penetrating, centered around the eye/temple/forehead and unilateral, lasting about 45-90min;
- pain occurs 1x/2x daily;
- autonomic features = ipsilateral lacrimation, rhinorrhoea, nasal congestion, eye lid swelling, facial swelling, flushing, conjunctival injection, partial Horner’s;
- pts will pace around
- Triggers = alcohol, exercise and solvents, sleep disruption
30
Q
Definition
Dementia
A
- Chronic and progressive deterioration of cognitive function due to organic brain disease; irreversible and consciousness isn’t impaired
- Different types:
- Alzheimer’s 50% = degen of cerebral cortex, with cortical atrophy and reduction in ACh production
- Vacular dementia 25% = brain damage due to several incidents of Cerebrovascular disease (strokes/TIAs);
- Lewy body dementias 15% = deposition of abnormal proteins within the brain stem and neocortex
- Frontotemporal dementia = specific degen of frontal and temporal lobes
31
Q
S+S
Dementia
A
- Alzheimer’s - insidious onset
- Vascular - stepwise decline
- Lewy Body - fluctuating levels of consciousness, hallucinations, falls and parkinsonian syx
- Frontotemporal - behavioural changes and intellectual changes
- All forms of dementia are associated with progressive loss of memory and cognitive function
32
Q
Definition
Encephalitis
A
- Inflammation of the brain parenchyma
- Commonly due to viral infection = HSV, VZV, mumps, adenovirus, coxsackie, EBV, HIV, japanese encephalitis;
- non-viral (rare) = syphillis, staph aureus;
- immunocompromised pts = CMV, toxoplasmosis, listeria;
- autoimmune/paraneoplastic associated with certain AB
33
Q
S+S
Encephalitis
A
- Usually self-limiting and mild, subacute onset, headache,
- fever, vomiting, neck stiffness, photophobia,
- behavioural changes, drowsiness, confusion,
- hx of seizures, focal neuro syx (dysphagia/hemiplegia), detailed travel hx
- Reduce consciousness, deteriorating GCS,
- seizures, pyrexia,
- sx of meningism = neck stiffness, photophobia, Kernig’s test +ve;
- sx of raised ICP = Cushing’s response (HTN, bradycardia, irreg breathing), focal neural sx, mmse may reveal cognitive/psych disturbance
34
Q
Investigations
Encephalitis
A
- Bloods -> FBC, U+Es, glucose, viral serology, ABG;
- MRI/CT -> exclude mass lesion, HSV causes oedema of temporal lobe on MRI;
- Lumbar puncture -> high lymphocytes/monocytes/protein, glucose normal, viral PCR;
- EEG -> epileptiform activity;
- brain biopsy rarely needed
35
Q
Definition
Epilepsy
A
- Tendency to recurrent unprovoked seizures;
- >2 seizures for epilepsy to dx;
- seizures = paroxysmal synch cortical electrical discharges,
- types of seizures =
- focal = localised to specific cortical regions, divided into complex (consciousness affected) and simple (consciousness NOT affected)
- Generalised = affecting whole of the brain, consciousness; types inc. tonic-clonic, absence, myoclonic, atonic, tonic
- 1ry epilepsy syndromes;
- 2ry seizures = tumour, infection (meningitis), inflammation (vasculitis), toxic/metabolic (Na imbalance), drugs (alcohol withdrawal), vascular (haemorrhage), congenital abnormalities, neurodegen diseases, malignant HTN/eclampsia, trauma;
- look like seizures = syncope, migraine, non-epileptiform seizure disorder;
- pathophysiology of seizures = imbalance in inhibitory/excitatory currents/NT in brain, precipitants are anything that promotes excitation of the cerebral cortex
36
Q
S+S
Epilepsy
A
History of seizures
- Rapidity of onset,
- duration of episode,
- consciousness changed?,
- tongue biting/incontinence,
- rhythmic synch limb jerking,
- post ictal abnormalities (confusion/exhaustion), drug hx
- Focal seizure = frontal lobe focal motor seizure (motor convulsions, Jacksonian march, Todd’s paralysis),
- temporal lobe seizures (aura, hallucinations),
- frontal lobe complex partial seizure (loss of consciousness, involuntary actions/disinhibition, rapid recovery);
- Generalised =
- Tonic-clonic (vague syx before attack, tonic (generalised muscle spasms) then clonic phase (repetitive synch jerks), faecal/urinary incontinence, tongue biting, post-ictal - impaired consciousness, lethargy, confusion, headache, back pain, stiffness),
- absence (childhood onset, loss of consciousness but maintains posture, pts stops talking and stares into space for a few seconds, no post-ictal phase),
- non-convulsive status epilepticus (acute confusional state, often fluctuating, difficult to distinguish from dementia)
37
Q
Management
Epilepsy
A
- Status epilepticus tx = initiated early, ABC approach, glucose check, IV lorazepam or IV/PR diazepam (repeat again after 10 min if seizure doesn’t terminate) - IV phenytoin considered if seizures recurr after next dose, consider also GA, or treat the cause, check plasma levels of anticonvulsants;
- Newly dx epilepsy tx = Start anti-convulsants after >2 unprovoked seizures,
- focal = lamotrigine/carbamazepine,
- generalised = Na valproate, only ONE drug (others inc. phenytoin, levetiracetam, clobazam, topiramate, gabapentin, vigabatrin);
- Pt education = avoid triggers, use seizure diaries, women of child-bearing age (!), drug interactions
38
Q
Investigations
Epilepsy
A
- Bloods -> FBC, U+Es, LFT, glucose, Ca, Mg, ABG, toxicology screen, prolactin;
- EEG -> helps to confirm dx, helps classify epilepsy;
- CT/MRI -> structural, space occupying or vascular lesions;
- other due to 2ry causes
