neurosciences Flashcards
define multiple sclerosis
autoimmune disease (T cell attack) causing demyelination of neurones
causes of multiple sclerosis
- genetic susceptibility + environmental trigger
- idiopathic
risk factors:
- genetics
- smokin
- EBV
- female
- vit D deficiency
- obesity
presenting symptoms of multiple sclerosis
LOVDIP
- optic neuritis MOST COMMON (unilateral loss of visual acuity, eye pain on movement, rapid central vision loss)
- sensory (pins and needles, burning, numbness)
- motor (stiffness, difficulty walking, spasms, heaviness)
- psychological (depression, amnesia, psychosis )
- autonomic (incontinence, urgency, impotence, hesitancy)
- sexual (erectile dysfunction)
- GI (swallowing difficulty)
- Cerebellum (scanning speech fails, intention tremor)
- Limb weakness, lethargy, Lhermitte’s sign (electroc shock when neck flexed)
- Optic neuritis
- Vertigo
- Diplopia
- Incontinence
- Parathesia (numbness/tingling)
examination findings of MS
- visual acuity test (loss of vision)
- loss of coloured vision
- visual field test (central scotoma - blind spot in normal vision if optic nerve affected)
- relative afferent pupillary defect
- Internuclear Ophthalmoplegia (lateral gaze causes failure of eye adduction)
- sensory (loss)
- motor (UMN lesion signs)
- cerebellum (dysdiadokinesia, ataxic wide gait, scanning speech, limb ataxia)
investigations for MS
- To diagnose Mcdonalds criteria
- no other alternative diagnosis
- dissemination in space lesions in different places
- dissemination in time - symptoms at different times
- Lumbar puncture
- Microscopy (exclude infection/inflammation)
- CSF electrophoresis (find mismatched oligoclonal bands)
- MRI brain, cervical/thoracic spines- hyperintense lesions perpendicular to corpus callosum. find brain lesions/ plaques
- Evoked potentials (visual, auditory somatosensory potentials - show reduced conduction velocity)
Management of MS
- MS relapses => high dose corticosteroids (oral methylprednisolone)
1. MS symptoms - visual problems- resolve on own
- fatigue- lifestyle changes +/- amantidine
- mobility - physiotherapy
- muscle spasms - baclofen/ physio
- emotional - antidepressants/ CBT
- sexual - viagra
- bladder- see urologist
- bowel - laxatives
- speech/language - therapist + changing diet
Define myasthenia gravis
autoimmune disease affecting the neuromuscular junction causing skeletal muscle weakness
causes of myasthenia gravis
- AUTOIMMUNE antibodies against nicotinic acetylchonine receptors => depleting post-synaptic receptors (most common type)
- paraneoplastic (SCLC)
Presenting symptoms of myasthenia gravis
muscle weakness - gets worse with more use
- ocular- diplopia, ptosis
- bulbar- facial weakness, hypernasal speech, difficulty smiling/chewing/swallowing
Define bulbar associated symptoms
- relating to medulla oblongata (affects bulbar muscles associated with cranial nerves with nuclei in medulla) CN X- XII
Examination signs of myasthenia gravis
- eye (ptosis, eye muscle paralysis/weakness = opthalmoplegia, check for ocular fatigue)
- put ice on eyes =>reduces ptosis
- reading aloud => dysarthria-slurred speech/ nasal speech
- Weakened power of limbs
What is the subtype of myasthenia gravis which has autoantibodies against pre-synaptic Ca2+ channel => less ACh release?
Lambert-Eaton
symptoms:
- muscle weakness gets better after repeated use/ exercise
- gait difficulty before eye signs
- autonomic problems (constipation, dry eyes)
Investigations of myasthenia gravis
- Bloods
- autoantibodies -ACH receptor/MUSK antibody
- Creatine kinase-exclude myopathies
- Electromyography (measures muscle response/ electrical activity to nerve stimulation of muscle)
- CT- exclude thymoma
- CXR - lung malignancies
Management for myasthenia gravis
- avoid triggers (tiredness, stress, infection, medicines, surgery)
- Pyridostigmine (anti cholinesterase) - helps electrical signals travel between nerves and muscles v short acting + diarrhoea/bradycardia risk
- Steroids (prednisolone) or higher dose immunosuppressants
- Surgery - remove abnormally large thymus gland
Treatment for a myasthenia crisis (sudden severe breathing/ swallowing problems)
- oxygen
- ventilator
- intravenous immunoglobulin therapy - to temporarily improve muscle strength
- plasmapheresis -filters harmful antibodies from blood
Define Parkinson’s disease
degeneration of dopaminergic neurons between substantia nigra => striatum due to mitochondrial DNA dysfunction
*>80% neuron loss = symptomatic
Causes of Parkinson’s disease
- sporadic parkinson’s => unknown cause
- secondary causes
- neuroleptic therapy for schizophrenia
- repeated head trauma
- post-encephalitis
- Wilson’s
- HIV
- MPTP toxin from illegal drug contamination
- familial genes
Presenting symptoms of Parkinson’s (TRAP BS)
- INSIDIOUS ONSET
- Tremor
- Rigidity
- Akinesia (difficulty initiating movement)
- Postural instability (frequent falls)
- BRADYKINESIA (slow + STIFF movements)
- smaller handwriting (micrographia)
- mental slowness (bradyphenia)
Non-motor symptoms of Parkinson’s
- depression + dementia
- dribbling saliva
- frequency/ urgency
- reduced smell
- visual hallucinations
Examination signs of Parkinson’s
- pill rolling tremor (4-5Hz) asymmetrical
- lead pipe rigidity + increased tone
- cogwheel rigidity (tremor worse on distraction)
- gait - shuffling, stooped, less arm swing, freezing
- FACE: frontalis overactivation-furrowed brows, hypomimic face, soft monotonous voice, drooling, synkineses (involuntary movement one side)
Investigations for Parkinson’s
- symptoms improve after levodopa
Exclude other conditions
- bloods- Wilson’s
- CT/brain MRI- hydrocephalus (can cause gait decline)
- SPECT scan
Management of Parkinson’s
- L-DOPA (levodopa) + peripheral DOPA decarboxylase inhibitor
*efficacy reduces over time so need higher doses or OTHER DRUGS
- DA receptor agonists
- Anti-cholinergics for tremor
- COMT inhibitor
- MAO-B inhibitor
Mechanism + side effects of L-DOPA
- levodopa crosses blood brain barrier gets converted to dopamine by dopamine decarboxylase => dopamine => stimulates dopamine receptors.
- SIDE EFFECTS: dyskinesia (involuntary movements), dystonia (involuntary muscle tightness) psychosis, visual hallucinations*
define encephalitis
inflammation of brain parenchyma
causes of encephalitis
VIRAL (common)
- Herpes simplex virus
- VZV
- Mumps
- adenovirus
- Coxsackie
- RBV
- HIV
non-viral
- syphillis
- Staph. aureus
- autoimmune
- paraneoplastic
causes of enceophalitis in immunocompromised patients
- toxoplasmosis
- listeria
- CMV
presenting symptoms of encephalitis
- infectious (headache, fever, vomiting, rash, cold sores, conjunctivitis)
- neurological symptoms (dysphagia, hemiplegia), behavioral changes (confusion)
- seizure history
- CHECK TRAVEL Hx
Signs of encephalitis on examination
- reduce consciousness
- deteriorating GCS
- Seizures
- pyrexia
- raised ICP (cushing’s response =>hypertension/bradycardia/irreglar breathing
Investigations for encephalitis
Bloods
- FBC (high WCC= viral cause)
- U&E (encephalitis => SIADH)
- glucose
- viral serology
- ABG
- Toxoplasma IgM titre
- Malaria film
CT/MRI
- exclude lesion/ mass
- HSV => oedema in temporal lobe
Lumbar puncture
- high lymphocytes
- high monocytes
- high protein
- glucose (normal/low)
- viral PCR
Electroenceophalogram (assess seizures)
Management of encephalitis
- resuscitate - ITU
- IV antibiotics
- mechanical ventilation
- anticonvulsants/ antipyuretics/ anti-emetics
- analgesia
Differences between meningitis and encephalitis
- meningism symptoms: photophobia/ neck stiffness
- seizures + cranial nerve palsy rare in meningitis
- minimal altered mental state in meningitis
Define Wernicke’s encephalopathy
thiamine deficiency causing neurological symptoms due to biochemical lesions of the CNS (hypothalamus, brain stem, cerebellum)
Causes of wenicke’s encephalopathy
- chronic alcohol consumption
- malnutrition (low B1)
- eating disorders
- prolonged vomiting (in chemo)
- GI malignancy
- thyrotoxicosis
- subdural haematoma
- AIDS
Presenting symptoms of wernicke’s enceophalopathy
- confusion
- vision changes - diplopia, eye movement abnormalities, ptosis
- loss of muscle coordination- unsteady gait
- amnesia + inability to form new memories
- hallucinations
Examination findings of wernicke’s encephalopathy
- confusion
- opthalmoplegia (nystagmus, lateral rectus palsies)
- ataxia- wide abnormal gait
- reduced reflexes
- low temp, rapid pulse
Investigations for Wernicke’s encephalopathy
Bloods:
- FBC (high MCV sign of alcoholics, WCC- infection => confusion)
- finger prick glucose => confusion
- U&Es (metabolic imbalances => confusion)
- serum thiamine
- LFTs
- ABG - hypercapnia/hypoxia => confusion
Rarer:
- CT/MRI of brain
Management of wernicke’s encephalopathy
High suspicion => resus with fluids, thiamine + magnesium + multivitamins
Low suspicion => thiamine + magnesium + folic acid
Long term: Continue with dietary thiamine
Define motor neuron disease
- build up of ubiquitinated protein which cause progressive motor neuron degeneration => death to lateral corticospinal tract
- also known as amyotrophic lateral sclerosis/ Lou gehrig’s
*associated with frontotemporal dementia
Subtypes of motor neuron disease
- ALS (amytropic lateral sclerosis/ Lou-gehrigs)- LMN/UMN lesion signs
- progressive muscle atrophy variant - LMN lesion signs
- progressive Bulbar Palsy variant (affects CN IX-XI)=> dysarthria, dysphagia, wasted fasiculated tongue, brisk jaw jerk reflex
- Primary lateral sclerosis- UMN lesion signs
Presenting symptoms of motor neuron disease
- muscle weakness
- swallowing difficulty
- behavioural changes (disinhibition)
- speech problems (slurred, reduced volume)
examination findings of motor neuron disease
UMN lesion signs
- Babinki’s (toes curling up)
- hypertonia
- hyperreflexia
- spastic weakness
LMN lesion signs
- fasiculations
- muscle wasting
- hypotonia
- hyporeflexia
- flaccid weakness
*NO SENSORY LOSS
Investigations for motor neuron disease
Bloods
- FBC
- raised ESR
- raised CK
- anti-GMI ganglioside antibodies
Other
- EMG - electrical activity produced by muscles
- Nerve conduction study- usually normal
- CT/MRI- spinal chord compression/ brainstem lesions
- Lumbar puncture - exclude infection/ meningitis
- spirometry - assess resp muscle depression
Management of motor neuron disease
Riluzole (blocks glutamate release as XS injures nerve cells) + monitor FBC/LFTs (hepatotoxic drug)
Treat other symptoms:
- muscle weakness- physiotherapy
- resp depression- non-invasive positive pressure ventilation
- depression- anti-depressants
- dysphagia - diet change / percutaneous endoscopic gastrostomy
Define Bell’s palsy
gradual unilateral peripheral facial nerve palsy (weakness, paralysis) affecting all facial zones equally
Causes of bell’s palsy
UNKNOWN
swelling/inflammation of facial nerve on one side due to viral infection
- HSV-1
- herpes zoster virus
- Epstein-barr
- coxsacchie virus (foot-mouth disease)
- adenovirus
- flu
- rubella
Presenting symptoms of bell’s palsy
- rapid onset of unilateral mild facial weakness to total paralysis
- facial droop + can’t make facial expressions (no smile)
- dry eye- due to loss of blink function
- pain behind jaw + ear on affected side
- change in hearing sensitivity on one side
- drooling
- loss of taste
- headaches
Differentials for bell’s palsy
bilateral weakness => lyme disease, Guillan-Barre, sarcoidosis, leukaemia
chronic recurrence =>
fever, rashes => lyme disease, autoimmune
Benign facial nerve schwannoma
trauma to temporal bone
Investigations for bell’s palsy
mainly CLINICAL don’t need investigation/tests
- Bloods (WCC - exclude infection)
- evoked electromyography + needle electromyography (no motor unit potentials)
- serum borrelia burgdorferi (check for Lyme-disease parasite)
Management for bell’s palsy
- reassure patient
- eye-protection (glasses/ artificial tears to stop development of keratopathy)
- corticosteroids (prednisilone) if more than >72hrs => reduce inflammation
- soft foods if difficulty chewing
For severe:
- anti-virals (valociclovir/ aciclovir)
- surgical decompression
- refer to secondary care (>3 weeks symptoms, atypical findings, changes in vision)
What is meningitis?
inflammation of meninges (pia/arachnoid mater) usually due to infection.
=> causes cerebral oedema which increases intracranial pressure => herniation/ reduces cerbral perfusion
Types of meningitis
- bacterial meningitis
- meningicoccal disease (specifically nisseiria meningitis bacteria)
- meningococcal septicemia
causes of meningitis (bacterial/ viral/ fungal)
bacterial:
- neonates (E.coli, listeria monocytognes, group B streptococci),
- childen/ adult (nisseiria meningitis, streptococcus pneumoniae, haemophilius influenza)
- elderly (streptococcus pneumonia, listeria monocytognes)
viral: enterovirus, Herpes Simplex Virus, mumps, Varicella roster virus, HIV
fungal: cryptococcus (common in HIV patients)
risk factors for meningitis
- >5 years or >60
- immunocompromised (HIV)
- immunosupressed
- sinuitis
- basal skull fratures
- splenectomy
- alcoholoism
- sickle cell
presenting symptoms of meningitis
non-specific signs:
- sudden fever
- sudden headache
- leg pain
- cold hands/feet
- abnormal skin
later:
- MENINGISM (stiff neck, photophobia)
- vomiting
- reduced consiousness
- altered mental state
- petechial - NON-BLANCHING RASH
specific:
- bacterial meningitis - photophobia, Kernig’s sign (flex knee to right angle and striaghten leg=> flex meninges => PAIN), Brudinski’s sign
- meningococcal disease - non-blanching rash
- septiciemia - mottled skin, cold hands/feet, hypotension, shock, reduced capillary refill
examination findings for meningitis
- photophobia
- neck stiffness
- kernigs sign (knee can’t extend when hip is flexed) -bacterial meningitis
- brudzinski’s sign (flexion of hip when knee is flexed)- bacterial meningitis
- infection signs (hypotension/ pyrexia/ tachycardia/ altered mental state/ non-blanching rash)
what symptoms do babies/children present with for meningitis
- commonly non-specific symptoms of infection (fever/ vomiting/ upper respiratory tract)
- high pitched crying
- hypotension
- irritability
- poor feeding
- seizures (sign of bacterial meningitis)
management for meningitis
- Immediate:
- if in GP / paramedic setting => IM/IV benzylpenicillin ASAP (UNLESS PENICILLIN ALLERGY) *infant =300mg, child=600mg adult =1,200mg
- non-blanching rash/sepsis => antibiotics first then transfer
- TRANSFER TO SECONDARY CARE (999/112)
IV fluids + ABs (cefotaxime). if >55- add ampicillin for listeria
- if no raised ICP => do Lumbar puncture
- IV dexamethosone (if lumbar puncture shows bacterial meningitis)
- Continue monitoring in ITU + adjust ABs for organism( cefotaxime)
investigations for meningitis in hospital setting
- Bloods:
- FBC (if low WCC = immunocompromised NEED HELP)
- LFT
- U&E
- coagulation screen (PT)- can develop clotting disorders (DIC)
- glucose
- blood PCR (EDTA sample - check for N. meningitidis)
- imaging - CT to exclude mass lesion + raised intracranial pressure
- Lumbar puncture - check CSF for MC&S (microscopy, culture& sensitivity), gram stain, glucose, protein, virology, lactate
When can a lumbar puncture be done before CT for suspected meningitis?
- if GCS 15 + no focal symptoms + no symptoms of raised intracranial pressure
lumbar puncture results for
- bacterial meningitis
- viral meningitis
- TB meningitis
- Bacterial=> cloudy/turbid CSF, high neutrophils, v. high protein, low glucose, high pressure
- Viral=> clear CSF, high lymphocytes, high protein, normal glucose
- TB/ fungal => fibrinous CSF, high lymphocytes, high protein, low gluocse
prognosis for meningitis
- bacterial meningitis + septicemia => 10-40% mortality
- viral meningitis => self-limiting
define stroke
rapidly developing focal disturbance of vascular origin lasting >24hrs
*sudden brain cell death due to artery rupture or blockage
- ischaemic
- haemorrgaic -increased blood displaces brain tissue => oedema/swelling/irritation of brain
types + causes of stroke
- ischaemic - vessel stenosis/occlusion
- thrombus (dehydration, thrombophillia)- lacunar infarcts, middle cerebral infarcts
- embolism (from carotid artery dissection/ AF)
- hypoptension
- vasculitis
- cocaine (arterial spasm)
- haemorraigic (20%)- vessel rupture => subarachnoid haemorrhage
- hypertension
- microaneurysm in brain vessels rupture (Berry, atriovenous malformations)
- haemorragic necrosis (tumour/infection)
- venous outflow obstruction (central venous thrombosis- oedema=> bleed in brain)
- trauma
- altered homeostasis (on anticoags - worsen ruptured vessels)
risk factors for stroke
- age >55
- FHx of stroke
- hypertension
- smoking
- diabetes
- previous TIA/ stroke
- Atrial fibrillation
- Carotid artery stenosis
presenting symptoms of stroke (Hx)
- SUDDEN ONSET
- unilateral weakness (facial/arm)
- dysphasia (difficulty speaking) , dysarthia (slurred speech)
- ataxia/ impaired coordination (more in problems with posterior circulation)
- visual distubrance -loss of sight in one eye (carotid artery stenosis)
- impaired consciousness + not confused
- sensory loss
- headache (differentials migraines/subarachnoid haemorrhage)
PMHx - AF/MI/ valvular problems/ carotid artery stenosis/ head/neck trauma