Neurology Flashcards
Differentials for headache
- Tension headaches
- Migraines
- Cluster headaches
- Secondary headaches
- Sinusitis
- Giant cell arteritis
- Glaucoma
- Intracranial haemorrhage
- Subarachnoid haemorrhage
- Analgesic headache
- Hormonal headache
- Cervical spondylosis
- Trigeminal neuralgia
- Raised intracranial pressure (brain tumours)
- Meningitis
- Encephalitis
Red Flags for headaches
- Fever, photophobia or neck stiffness MENINGITIS OR ENCEPHALITIS
- New neurological symptoms HAEMORRHAGE, MALIGNANCY, STROKE
- Dizziness STROKE
- Visual disturbance TEMPORAL ARTERITIS OR GLAUCOMA
- Sudden onset occipital headache SUBARACHNOID HAEMORRHAGE
- Worse on coughing or straining RAISED INTRACRANIAL PRESSURE
- Postural, worse on standing, lying or bending over RAISED INTRACRANIAL PRESSURE
- Severe enough to wake from sleep
- Vomiting RAISED INTRACRANIAL PRESSURE OR CO POISONING
- History of trauma INTRACRANIAL HAEMORRAGE
- Pregnancy PRE-ECLAMPSIA
- Previous headache history + progression or change in character
Types of migraine
- Migraine without aura
- Migraine with aura
- Silent migraine (aura but no headache)
- Hemiplegic migraine (mimic stroke)
Triggers of migraine
- Chocolate
- Hangovers, dehydration
- Orgasms
- Cheese
- Oral contraceptives
- Lie-ins (abnormal sleep patterns)
- Alcohol
- Tumult = loud noise, strong smells, bright lights
- Exercise
- Stress
- Menstruation
- Trauma
Stages of migraine
- Premonitory/prodromal stage = 3 days before
- Aura = up to 60 mins
- Headache = 4-72 hours
- Resolution
- Post-dromal or recovery
Presentation of migraine
- Prodrome = yawning, cravings, mood/sleep changes
- Headache lasting 4-72 hours
o Moderate to severe intensity
o Pounding or throbbing in nature
o Usually unilateral but can be bilateral - Nausea and vomiting
- Photophobia/phonophobia (sound sensitive)
- Motion sensitivity
- Visual or other aura lasting 15-30 mins
o Sparks, lines, blurring in vision
o Loss of different visual fields
o Paraesthesiae spreading from fingers to face - Hemiplegic migraine
o Typical migraine symptoms
o Hemiplegia (unilateral weakness of limbs)
o Ataxia
o Speech = dysphasia or paraphasiae
o Changes in consciousness - Papilloedema
Management of migraine
- Dark quiet room and sleep
1. Paracetamol or NSAIDs + oral triptan
o S/E = Tingling, heat, tightness, heaviness, pressure
o CI = Hx of IHD or cerebrovascular disease - Antiemetics – if vomiting (metoclopramide)
Prevention of migraines
- Keep headache diary to identify triggers and avoid triggers
- Propranolol (not in asthma)
- Topiramate (not in pregnancy or women of child bearing age)
- Amitriptyline
- Botox
- 10 sessions of Acupuncture over 5-10 wks
- Supplementation with B2 (riboflavin)
- NSAIDS/triptans (if associated with menstruation)
Risk factors for tension headache
- Stress
- Sleep deprivation
- Bad posture
- Hunger (skipping meals)
- Eyestrain
- Anxiety/Depression
- Noise
- Alcohol
- Dehydration
Presentation of tension headache
- Can be episodic (<15 days/month) or chronic (>15 days/month for at least 3 months)
- Bilateral pressing/tight non-pulsatile headache
o Mild/moderate intensity
o Headaches can last from 30 mins to 7 days
o Comes on and resolves gradually - Scalp muscle tenderness
- Pressure behind eyes
- No visual changes
Management of tension headache
- Lifestyle
o Reassurance and lifestyle advice
o Stress relief and relaxation techniques
o Hot towels to local area - Medication
o Basic analgesia paracetamol, ibuprofen, aspirin
o TCA amitriptyline
Risk factors for cluster headache
Smoking
Triggers of cluster headache
- Alcohol
- Strong smells
- Exercise
Presentation of cluster headaches
- Episodic = clusters last 4-12 wks and followed by pain-free periods of months-yrs
- Chronic = attacks for more than 1 yr without remission
- Sudden excruciating stabbing unilateral pain around one eye, temple or forehead
o Rises in crescendo over mins and last 15 mins to 3 hrs
o Once or twice a day (usually same time)
o Often nocturnal/early morning = wakes patient from sleep - Vomiting
- Restlessness or agitation
- Red swollen, watering eye with lid swelling
- Facial flushing/sweating
- Rhinorrhoea (nasal discharge)
- Miosis (excessive pupil constriction)
- Ptosis (eyelid dropping)
Management of cluster headaches
- SC sumatriptan
- High flow O2 for 15 mins via non-breathable mask
Prophylaxis of cluster headaches
- 1st line = CCB (verapamil)
- Avoid alcohol
- Prednisolone (short course for 2-3 wks)
- Lithium
What is giant cell arteritis
Chronic vasculitis characterised by granulomatous inflammation in walls of medium and large arteries
Presentation of GCA
- New-rapid onset headache (temporal) <1m
- Temporal artery abnormality
o Tenderness on palpation of temporal artery
o Thickening
o Nodularity
o Red overlying skin
o Pulsation (reduced or absent) - Visual disturbances
o Loss of vision
o Diplopia
o Changes to colour vision
o Fundoscopy pallor and oedema of optic disc, cotton wool patches, small haemorrhages in retina - Scalp tenderness (brushing hair)
- Scalp necrosis
- Intermittent jaw claudication
- Systemic features Low grade fever, Fatigue, Anorexia, Weight loss, Depression, Night sweats
- Features of PMR proximal muscle pain, morning stiffness, tenderness, aching
Investigations for GCA
- Raised inflammatory markers ESR >50
- Temporal artery biopsy skip lesions
- Creatine kinase and EMG normal
- Vision testing
o Anterior ischaemic optic neuropathy
o Temporary visual loss
o Diplopia
o Swollen pale disc and blurred margins
Management of GCA
- MEDICAL EMERGENCY
- Urgent high-dose Glucocorticoids = 40-60mg oral prednisolone per day for 1-2 yrs
o Given before temporal artery biopsy
o No visual loss prednisolone
o If visual loss give IV methylprednisolone
o Should be dramatic response - Urgent ophthalmology review = Visual damage often irreversible
- Bone protections with bisphosphonates
- Low dose aspirin
Complications of GCA
- Loss of vision
- Aortic aneurysm
- Aortic dissection
- Large artery stenosis
- Aortic regurgitation
- CVD = MI, HF, stroke, peripheral arterial disease
- Scalp necrosis, peripheral neuropathy, depression, confusion, encephalopathy, deafness
Management of analgesia overuse headache
- Withdrawal of analgesia
o Immediately for paracetamol, NSAIDs or triptans
o Gradually for opiates
Presentation of trigeminal neuralgia
- 90% unilateral, 10% bilateral
- Intense electricity-like shooting facial pain, comes on spontaneously
- Lasts between few secs to hrs
- Attacks worsen over time
Management of trigeminal neuralgia
- Carbamazepine
- Surgery to decompress or intentionally damage nerve
Causes of raised intracranial pressure
- Brain tumours
- Intracranial haemorrhage
- Idiopathic intracranial hypertension
- Abscesses or infection
Presentation of raised intracranial pressure
- Constant headache (often nocturnal)
o Worse on waking, coughing, straining or bending forward - Vomiting
- Altered mental state
- Visual field defects
- Seizures
- Unilateral ptosis
- 3rd and 6th nerve palsies
Fundoscopy findings in papilloedema
o Blurring of optic disc margin
o Elevated optic disc
o Loss of venous pulsation
o Engorged retinal veins
o Haemorrhages around optic disc
o Paton’s lines (creases in retina around optic disc)
Risk factors for idiopathic intracranial hypertension
- Obesity
- Pregnancy
- Drugs = COCP, steroids, tetracyclines (lymecycline), Vit A, lithium
Presentation of idiopathic intracranial hypertension
- Headache
- Blurred vision
- Papilloedema
- Enlarged blind spot
- 6th nerve palsy
Management of idiopathic intracranial hypertension
- Weight loss
- Diuretics = acetazolamide
- Topiramate
- Repeated LP
- Surgery
o Optic nerve sheath decompression and fenestration
o Lumboperitoneal or ventriculoperitoneal shunt
Types of brain tumour
- Meningiomas (benign)
- Gliomas Astrocytoma, Oligodendroglioma, Ependymoma
- Glioblastomas (highly malignant)
- Pituitary tumours
- Acoustic neuroma (schwann cells)
- Secondary metastases (lung, breast, renal cell carcinoma, melanoma)
Presentation of brain tumours
- Asymptomatic when small
- Features of ICP headache, vomiting, seizures, 3rd and 6th nerve palsies, visual field defects, papilloedema, altered mental state, unilateral ptosis
- Fatigue
- Hearing loss, tinnitus, balance problems (acoustic neuroma)
- Temporal lobe dysphasia, amnesia
- Frontal lobe hemiparesis, personality change, Broca’s dysphasia, lack of initiative, unable to plan tasks
- Parietal lobe hemisensory loss, reduction in 2-point discrimination, dysphasia, astereognosis (unable to recognise object from touch alone)
- Occipital lobe contralateral visual defects
- Cerebellum dysdiadochokinesis (impaired rapidly alternative movement), ataxia, slurred speech (dysarthria), hypotonia, intention tremor, nystagmus, gait abnormality (DASHING)
Management of brain tumours
- Palliative care
- Surgery = remove mass depending on grade
- Chemotherapy (6 weeks post-op)
- Radiotherapy
- Oral dexamethasone = steroid to improve brain performance
- Oral carbamazepine = anticonvulsant
- Pituitary tumours
o Trans-sphenoidal surgery
o Radiotherapy
o Bromocriptine (block prolactin-secreting tumours)
o Somatostatin analogues (block GH secreting tumours)
Causes of brain abscess
- Extension of sepsis from middle ear or sinuses
- Trauma or surgery to scalp
- Penetrating head injuries
- Embolic events from endocarditis
Presentation of brain abscess
- Raised ICP
- Headache = dull, persistent
- Fever
- Focal neurology = e.g. oculomotor nerve palsy
- Nausea
- Papilloedema
- Seizures
Management of brain abscess
- NEUROLOGICAL EMERGENCY
- Surgery Craniotomy and abscess cavity debrided
- IV Abx IV 3rd generation cephalosporin and metronidazole
- ICP management Dexamethasone
Risk factors for subarachnoid haemorrhage
- Berry aneurysms
o Hypertension
o Connective tissue disorders = Marfan or Ehlers-Danlos syndrome
o Autosomal dominant polycystic kidney disease
o Coarctation of aorta - Smoking
- Excessive alcohol consumption
- Cocaine use
- Sickle cell anaemia
- Neurofibromatosis
Presentation of SAH
- Thunderclap headache during strenuous activity
o Severe occipital pain peaking within 1-5 mins - Meningism Neck stiffness, Photophobia
- Nausea and vomiting
- Speech changes, weakness, seizures
- Depressed level of consciousness, coma
- Kernig’s sign = unable to extend patients leg at knee when thigh is flexed
- Brudzinski’s sign = when patients neck is flexed by doctor, patient will flex hips and knees
- Papilloedema
- Vision loss or diplopia (double vision)
- Fixed dilated pupils = signs of CN3 palsy caused by pressure on CN3 seen in posterior communicating artery aneurysm
- Marked increase in BP
Investigations for SAH
- Non-contrast CT head = ‘star-shaped lesion’
- If CT head within 6 hrs of Sx onset and normal consider alternative diagnosis
- If CT head done >6hrs after Sx onset and normal do a LP
- Lumbar puncture at >12 hrs
o Xanthochromia (yellow CSF caused by bilirubin)
o Red cell count raised
o Normal or raised opening pressure - Angiography = locate source of bleeding
- ECG ST elevation
Management of SAH
- Refer to neurosurgery
o Endovascular coiling or clipping = treat aneurysms - Nimodipine (CCB) = prevent vasospasm
- Intubation and ventilation
- IV fluids and maintain BP
- Lumbar puncture or insertion of shunt = hydrocephalus
- Antiepileptic medications
Complications of SAH
- Very high mortality and morbidity
- Rebleeding
- Cerebral ischaemia
- Hydrocephalus
- Hyponatraemia due to SIADH
- Vasospasm
- Seizures
Risk factors for SDH
- Elderly
- Traumatic head injury
- Cerebral atrophy/increasing age
- Alcoholism
- Dementia
- Anticoagulation
- Physical abuse of infant
Cause of SDH
Rupture of bridging veins in outermost meningeal layer
Presentation of SDH
- Raised ICP symptoms
- Headache
- Sleepiness
- Vomiting and nausea
- Personality change
- Unsteadiness
- Seizures occasionally
- Raised BP
- Hemiparesis or sensory loss
- Stupor, coma
Investigations for SDH
- Non-contrast CT head <48 hrs
o Crescent shape on 1 hemisphere and not limited by cranial sutures
o Dark/hypodense = chronic
o Bright hyperdense = acute
Management of SDH
- ABCDE then refer to neurosurgeons
- Burr hole craniotomy and clot evacuation
- IV mannitol to reduce ICP
- Phenytoin (if seizures)
- Address cause of trauma = Fall due to cataract, arrhythmia, abuse etc
Complications of SDH
Tectorial herniation and coning
Cause of extradural haemorrhage
Traumatic head injury leading to laceration of middle meningeal artery
Presentation of EDH
- Brief post-traumatic loss of consciousness or initial drowsiness
- Severe headache
- Nausea and vomiting
- Confusion
- Seizures
- Rapid rise in ICP
- Hemiparesis (weakness of half side of body) with brisk reflexes
- Ipsilateral pupil dilates
- Breathing becomes deep and irregular = brainstem compression
- Decreased GCS
- Coning = Brain herniates through foramen magnum
- Bradycardia and raised BP
Investigations of EDH
- CT head = biconvex shape and limited by cranial sutures
- Skull XR = fracture lines crossing course of middle meningeal artery
Management of EDH
- ABCDE
- Refer to neurosurgery
o Burr hole craniotomy
o clot evacuation +/- ligation of bleeding vessel - IV mannitol for increased ICP
- Maintain intubation and ventilation
Causes of cerebellar disorder
- Stroke or TIA
- Hydrocephalus
- Posterior fossa tumours or abscess
- Thiamine deficiency
- Vitamin E deficiency
- Gluten sensitivity
- Meningo-encephalitis or intracranial abscess
- Acute or chronic viral infections (HIV)
- Parasitic infections
- Creutzfeldt-Jakob disease
- Alcohol
- Carbon monoxide poisoning
- Drugs = barbiturates, phenytoin, piperazine, antineoplastic drugs
- Drug overdose
- Trauma
- Multiple sclerosis
- Lung, gynae, breast cancer or Hodgkin’s lymphoma
- Friedreich’s ataxia
- Cerebral oedema of chronic hypoxia
- Inherited metabolic disorders
- Cerebral palsy
What is cerebral palsy
Group of permanent movement and posture disorders that limit activity due to non-progressive lesion of motor pathways in developing brain
Prenatal factors for cerebral palsy
o Multiple gestation
o Chorioamnionitis
o Maternal respiratory tract or genito-urinary infection treated in hospital
o Thyroid disease, iodine deficiency, TORCH
o Maternal thrombotic disorders (factor V Leiden mutations)
o Teratogen exposure (warfarin)
o Fetal genetic and metabolic disorders
o Fetal brain malformations
o Placental abruption
Perinatal factors for cerebral palsy
o Preterm birth
o Low birth weight
o Respiratory distress
o Birth asphyxia
o Intraventricular haemorrhage
o Hyperbilirubinaemia
o Neonatal sepsis
o Neonatal encephalopathy
Postnatal factors for cerebral palsy
o Head injuries (<3y)
o Meningitis
o Intraventricular haemorrhage
o Hypoglycaemia
o Hydrocephalus
Presentation of cerebral palsy
- Low APGAR score at birth
- Posturing
o Abnormal limb/trunk posture
o Persistent toe-walking - Oropharyngeal problems
o Tongue thrusts, grimacing, swallowing difficulties
o Feeding difficulties - Strabismus
- Tone
o Abnormal tone early infancy (increased or decreased)
o Asymmetric hand function before 12m
o Abnormal gait
o Difficulty in changing nappies (stiff limbs)
o Delayed motor milestones not sitting by 8m, not walking by 18m
o Late head control, rolling and crawling - Evolutional response
o Persistent primitive reflexes (palmar grasp, rooting)
o Failure to develop equilibrium and protective responses - Reflexes
o Deep tendon reflexes increased
o Plantar reflexes up going - Diarrhoea
- Learning difficulties
- Epilepsy
- Squints
- Hearing impairment
Management of cerebral palsy
- MDT coordinated care
- Minimise complications and comorbidities
- Treat spasticity
o Oral diazepam
o Oral and intrathecal Baclofen
o Botox
o Anticonvulsants - Analgesia
- Surgical treatment
o Repair of scoliosis, tendon lengthening, osteotomy
o Selective dorsal rhizotomy
Complications and comorbidities of cerebral palsy
- Feeding difficulties
- Sialorrhoea/drooling
- Aspiration, recurrent chest infection
- Vomiting, regurgitation, GORD
- Osteopenia, osteoporosis
- Constipation
- Incontinence
- Visual impairment
- Hearing impairment
- Epilepsy
- Learning disability
- Communication difficulties
- Behavioural difficulties
- Mental/psychological health problems, neurodevelopmental disorders
- Pain
- Sleep disturbance
- Impaired social interaction and participation
- Reduced quality of life
- Reduced life expectancy
Causes of seizures
- Abnormal blood vessels in brain
- Alzheimer’s/dementia
- Use of drugs = cocaine
- Alcohol withdrawal
- Head injury years before onset
- Cerebrovascular disease = cerebral infraction or haemorrhage
- CNS infection = meningitis or encephalitis
- Stroke/brain tumour
Generalised tonic clonic seizures
o Loss of consciousness
o Muscle tensing and muscle jerking
o Tongue biting, incontinence, groaning, irregular breathing
o Prolonged post-ictal = confused, drowsy, irritable, depressed
Management of generalised tonic clonic seizures
o (male) = sodium valproate
o (female child-bearing age) = lamotrigine or carbamazepine
Focal seizures
o Affect hearing, speech, memory and emotions
o Hallucinations
o Memory flashbacks/ Déjà vu
o Automatisms lip smacking, grabbing, plucking
Management of focal seizures
o carbamazepine or lamotrigine
o sodium valproate or levetiracetam
Absence seizures
o Children (most stop as they get older)
o Blank, stares into space then abruptly returns to normal
o Unaware of surroundings and won’t respond
o Lasts 10-20s
Management of absence seizures
o ethosuximide
o sodium valproate
Atonic seizures
o Brief lapses in muscle tone
o Last under 3 mins
o Begin in childhood
o Lennox-Gastaut syndrome
Management of atonic seizures
o sodium valproate
o lamotrigine
Myoclonic seizures
o Sudden brief muscle contractions (sudden ‘jump’)
o Remains awake
o Juvenile myoclonic epilepsy
Management of myoclonic seizures
o sodium valproate
o lamotrigine, levetiracetam, topiramate
Infantile spasms/West syndrome
o Starts around 6m
o Clusters of full body spasms
o Poor prognosis
Management of infantile spasms
1st line = prednisolone or vigabatrin
Other features of epileptic seizures
- Jacksonian march = seizure marches up/down motor homunculus starting in face or thumb
- Post-ictal Todd’s palsy = paralysis of limbs involved in seizure for several hours
- Frontal lobe head/leg movements, posturing, post-ictal weakness, Jacksonian march
- Parietal lobe paraesthesia
- Occipital lobe floaters/flashes
Advice for epileptic patients
o Inform DVLA = cannot drive until seizure free for 6m post seizure or 1 year with established epilepsy
o Avoid swimming/bathing alone, dangerous sports
o Breastfeeding considered safe with most AED
o Lamotrigine most suitable if wanting to get pregnant
Differences between epilepsy and syncope
Tongue biting
Head turning
Muscle pain
Loss of consciousness
Cyanosis
Post-ictal symptoms
Side effects of sodium valproate
Teratogenic (contraception advice and avoided in women/girls unless alternative)
Liver damage and hepatitis
Hair loss
Tremor
Increased appetite and weight gain
Ataxia
Thrombocytopenia
Side effects of carbamazepine
Agranulocytosis
Aplastic anaemia
Induces P450 system so many drug interactions (e.g. Warfarin)
Dizziness and ataxia
Drowsiness
Inappropriate ADH secretion
Side effects of phenytoin
Folate and vitamin D deficiency
Megaloblastic anaemia
Osteomalacia
Peripheral neuropathy
Lymphadenopathy
Side effects of lamotrigine
Stevens-Johnson syndrome or DRESS syndrome (life threatening skin rashes)
Leukopenia
Presentation of non-epileptic attack disorders
- Seizures lasting over 2 mins
- Gradual onset
- Fluctuating course
- Violent thrashing movements
- Side-to-side head movement
- Asynchronous movements
- Eyes closed
- Recall for period of unresponsiveness
- Pelvic thrusting
- Family member with epilepsy
- History of mental health conditions and/or trauma
- Crying after seizure
- Doesn’t occur when alone
Causes of facial nerve palsy
UMN lesions
* Unilateral = cerebrovascular accidents, tumours
* Bilateral = pseudobulbar palsies, MND
LMN lesions
* Bell’s palsy
* Ramsay-Hunt syndrome
* Infection = otitis media, HIV, lyme disease
* Systemic = DM, sarcoidosis, leukaemia, MS, GBS
* Tumours = acoustic neuroma, parotid tumours, cholesteatomas
* Trauma = surgery, base of skull fractures
Difference between UMN and LMN facial nerve palsy
UMN lesion = forehead will be spared
LMN lesion = forehead will not be spared
MAangement of bell’s palsy
Majority recover over several weeks (up to 12m)
Within 72 hrs of symptoms Prednisolone
* 50mg for 10 days
* 60mg for 5 days followed by 5 day reducing regime of 10mg a day
Eye care
* Lubricating eye drops
* Tape eye closed at bed time
* Ophthalmology review
If no improvement after 3 wks refer urgently to ENT
Refer to plastic surgery if long-standing weakness for surgical decompression
Presentation of horner’s syndrome
Partial ptosis = upper eyelid drooping
Miosis = pupillary constriction
Hemifacial anhidrosis = absence of sweating
Facial flushing
Orbital pain/headache
Presentation of Parkinson’s disease
- Gradual onset of symptoms
- Resting Tremor
o Often asymmetrical
o Usually most obvious in hands (pill rolling tremor)
o Improved by voluntary movements and made worse by anxiety/distracted
o Using other hand exaggerates tremor
o Issue with repetitive hand movements with worsening in rhythm the longer attempted
o Not present in sleep - Rigidity = extrapyramidal lesion
o Cogwheel rigidity
o Increased tone in limbs and trunk
o Spasticity where resistance falls away as movement continues
o Resistance to passive movement of joint (tension give way in small increments)
o Can cause pain and problems with turning in bed - Bradykinesia
o Slow to initiate movement and slow, low-amplitude excursions in repetitive actions
o Reduced blink rate
o Monotonous hypophonic speech (indistinct and flat)
o Difficulty with fine movements
o Micrographia = smaller writing
o Expressionless face (facial masking) - Gait
o Reduced asymmetrical arm swing
o Stooped posture
o Shuffling gait (small steps, dragging foot)
o Difficulty initiating movement (standing to walking) and turning
o Poor balance and postural instability
o Forward tilt - Other
o Depression
o Sleep disturbance and insomnia
o Loss of sense of smell
o Cognitive impairment and memory problems
o Drooling of saliva and swallowing difficulty = late feature
Management of Parkinson’s disease
- Physiotherapy and Physical activity
- Oral levodopa along with peripheral decarboxylase inhibitor (Co-careldopa/ co-beneldopa)
o Delay use with other drugs first
o Becomes less effective over time - Dopamine agonists = Oral ropinirole/ oral pergolide
o Avoid bromocriptine/cabergoline - Monoamine oxidase B inhibitors = oral selegiline or oral rasagiline
- Catechol-O-methyl transferase inhibitors = Oral entacapone/ tolcapone
- SSRIs for depression = Oral citalopram
- Anti-psychotics = Oral quetiapine
- Deep brain stimulation = Help those who are partly-dopamine responsive
- Surgical ablation of overactive basal ganglia circuits
Differences between Parkinson’s and benign essential tremor
Parkinson’s
Asymmetrical
4-6Hz
Worse at rest
Improves with intentional movement
Other parkinson’s features
No change with alcohol
BET
Symmetrical
5-8Hz
Improves at rest
Worse with intentional movement
No other Parkinson’s features
Improves with alcohol
Side effects of levodopa
Dyskinesias (abnormal movements associated with excessive motor activity)
e.g. dystonia = excessive muscle contraction leade to abnormal postures/ exaggerated movement
e.g. chorea = abnormal involuntary movements
e.g. athetosis = involuntary twisting or writhing movements (fingers, hands, feet)
Reduced efficacy over time even if dose increased
On-dyskinesias = hyperkinetic, choreiform movement whenever drugs work
Off-dyskinesias = fixed, painful dystonic posturing
Freezing = unpredictable loss of mobility
Causes of essential tremor
- Physiological
- Post-traumatic
- Medication
- MS
- Parkinsonism
- Metabolic derangement
- Wilson’s disease
- Cerebellar disease
- Basal ganglia lesions
- Dystonias
- Vitamin deficiency (B1)
Management of essential tremor
- May not require treatment (mild)
- Intermittent treatment (mild-moderate)
- Treatment options
o Propranolol
o Primidone
o Others = topiramate, atenolol, alprazolam, clonazepam, gabapentin - Head tremor = botox
- Deep brain stimulation
- Thalamotomy
- High-intensity focused US with MRI guidance
Presentation of Huntington’s disease
- Psychiatric problems (begins first)
o Cognitive, psychiatric, mood problems
o Behavioural change = Aggression, addictive behaviour, apathy and self-neglect
o Depression/anxiety - Chorea
o Relentlessly progressive, jerky, explosive, rigidity involuntary movements = ceases when sleeping
o May begin as general restlessness, unintentionally initiated movements and lack of coordination - Speech difficulties (dysarthria)
- Swallowing difficulties (dysphagia)
- Seizures
- Eye movement disorders
- Dementia = Impaired cognitive abilities and memory
Management of Huntington’s disease
- Counselling to patient and family = Genetic counselling to any children of patients
- SALT
- Advanced directives and End of life care planning
- Symptomatic management
o Antipsychotics (olanzapine)
o Benzodiazepines (diazepam)
o Sulpiride = depresses nerve function
o Tetrabenazine = dopamine depleting agent
o Antidepressants (SSRIs) = seroxate
o Antipsychotic mediation (Neuroleptics) = haloperidol
o Treat aggression = Risperidone
Presentation of normal pressure hydrocephalus
- Gait abnormality (WOBBLY)
- Urinary incontinence (WET)
- Cognitive impairment (WACKY)
o Memory loss
o Inattention
o Inertia
o Bradyphrenia (slowness of thought) - Dizziness
- Spasticity
- Sudden ‘freezing’
- Pyramidal tract signs
- Brisk reflexes
Management of normal pressure hydrocephalus
- CSF shunt (ventriculoperitoneal)
- Carbonic anhydrase inhibitors (acetazolamide)
- Repeated LP
Complications of CSF shunt
- Shunt occlusion
- Catheter breakage
- CSF hypotensive headaches
- Cerebral infarct
- Haemorrhage
- Infection
- Seizures
Causes of stroke
- Ischaemia
o Thrombus formation or embolus
o Atherosclerosis
o Large artery stenosis
o Shock
o Vasculitis
o Hypoperfusion = sudden drop in BP
o Hyperviscosity = polycythaemia and sickle cell - Haemorrhage
o Trauma
o Aneurysm rupture
o Anticoagulation
o Thrombolysis
o Carotid artery dissection
o Subarachnoid haemorrhage
Classifications of stroke
- Oxford stroke (Bamford) classification
- Total anterior circulation infarcts = middle and anterior cerebral arteries
- Partial anterior circulation infarcts = smaller arteries of anterior circulation
- Lacunar infarcts = arteries around internal capsule, thalamus and basal ganglia
Presentaiton of anterior cerebral artery stroke
o Contralateral hemiparesis
o Sensory disturbances
o Lower extremity > upper
o Gait apraxia = loss of normal function of lower limbs
o Truncal ataxia = can’t sit/stand unsupported and tend to fall backwards
o Incontinence
o Akinetic mutism = decrease in spontaneous speech, stuporous state
Presentation of middle cerebral artery stroke
o Contralateral arm and leg weakness
o Sensory loss
o Contralateral homonymous hemianopia
o Aphasia = inability to understand speech
o Dysphasia = deficiency in speech generation
o Facial droop
Presentation of posterior cerebral artery stroke
o Contralateral homonymous hemianopia with macular sparing
o Cortical blindness
o Visual agnosia = can see but not interpret visual information
o Prosopagnosia = cannot see faces
o Colour naming and discriminate problems
o Unilateral headache
Weber’s syndrome
o Ipsilateral CNIII palsy
o Contralateral weakness of upper and lower extremity
Posterior inferior cerebellar artery stroke
o Ipsilateral facial pain and temperature loss
o Contralateral limb/torso pain and temperature loss
o Ataxia
o Nystagmus
Anterior inferior cerebellar artery stroke
Lateral pontine syndrome
Ipsilateral facial paralysis and deafness
Retinal artery stroke
Amaurosis fumax
Basilar artery stroke
‘locked in’ syndrome
Lacunar stroke
o Unilateral weakness of face and arm, arm and leg or all 3
o Pure sensory loss
o Ataxic hemiparesis = cerebellar and motor symptoms
Emergency diagnosis of stroke
- ROSIER tool
- Stroke likely if score above 0
Investigations for stroke
- Urgent non-contrast CT head/ diffusion weighted MRI before treatment = exclude haemorrhage
- Pulse, BP, ECG = look for AF
- Blood glucose = exclude hypoglycaemia
- Specialist imaging
o Establish vascular territory affected
o Diffusion-weighted MRI (GS)
o Non-contrast CT alternative
o Carotid USS (carotid stenosis)
o Endarterectomy (remove plaques or carotid stenting)
Management of ischaemic stroke
o ABCDE = Hydration and oxygen if needed
o Aspirin 300mg (after CT) and continue for 2 weeks
o Thrombolysis with alteplase (within 4.5 hrs after excluding intracranial haemorrhage)
o Thrombectomy if occlusion is confirmed on imaging depending on location and time (not after 24 hrs)
o Clopidogrel 24 hrs after thrombolysis
o Monitoring for post thrombolysis complications (repeat CT)
o Stroke rehab
Management of haemorrhagic stroke
o Frequent GCS monitoring
o Antiplatelets contraindicated
o Any anticoagulants should be reversed (Warfarin beriplex and vit K)
o Control hypertension
o Manual decompression of raised ICP
o Diuretic = Mannitol
SEcondary prevention of stroke
- Lifelong platelet treatment = aspirin and dipyridamole/clopidogrel daily
o Give PPI cover for aspirin (omeprazole) - Treat modifiable risk factors
o Cholesterol treatment = simvastatin (not started immediately)
o AF treatment = warfarin/ new oral anticoagulants (apixaban)
o Blood pressure treatment = ACE-I (ramipril)
Management of TIA
- Immediate 300mg aspirin unless
o Patient has bleeding disorder or taking anticoagulant
o Already taking low dose aspirin
o Aspirin contraindicated - Urgent review by specialist within 24 hrs
- Secondary prevention
o Clopidogrel
o Aspirin + dipyridamole
o Atorvastatin - Cannot drive for minimum 4 week
What is myasthenia gravis
Autoimmune disease against nicotinic acetylcholine receptors in neuromuscular junction
Exacerbating factors of myasthenia gravis
- Penicillamine
- Quindine, procainamide
- Beta-blockers
- Lithium
- Phenytoin
- Abx = gentamicin, macrolides, quinolones, tetracyclines
- Pregnancy
- Hypokalaemia
- Infection
- Change of climate
- Emotion
- Exercise
Associations of myasthenia gravis
- Thymomas
- Autoimmune disorders: pernicious anaemia, autoimmune thyroid disorders, rheumatoid, SLE
- Thymic hyperplasia
Presentation of myasthenia gravis
- Muscle weakness and fatigability
o Worse with muscle use, at end of day, in pregnancy, hypokalaemia, infection, emotion, drugs (opiates, BB, gentamicin)
o Improves with rest, in morning
o Affect proximal muscles and small muscles of head and neck - Extraocular muscle weakness Prolonged upward gazing exacerbate diplopia
- Eyelid weakness (repeated blinking) causing ptosis
- Weakness in facial movements
- Difficult with swallowing
- Fatigue in jaw when chewing
- Slurred speech
- Repeated abduction of one arm 20 times
Investigations for myasthenia gravis
- Autoantibodies: Acetylcholine receptor antibodies (85%), Muscle-specific kinase antibodies
- Single fibres electromyography
- CT/MRI of thymus gland
- Edrophonium/tensilon test = IV edrophonium chloride temporarily relieves weakness
- Nerve conduction study
- Ptosis improves by >2mm after ice application to shut lid for >2mins
- Test FVC
- Check for thymectomy scar
Management of myasthenia gravis
- Reversible acetylcholinesterase inhibitors Pyridostigmine 1st line
- Immunosuppression (prednisolone or azathioprine)
- Monoclonal antibodies (rituximab)
- Bone protection, PPI
- Thymectomy
Side effects of acetylcholinesterase inhibitors
increased salivation, lacrimation, sweats, vomiting, miosis, diarrhoea
Complications of myasthenic gravis
- Myasthenic crisis = weakness of respiratory muscles
o Acute worsening of symptoms
o Triggered by another illness (respiratory tract infection)
o Can lead to respiratory failure
o Plasma exchange and IV immunoglobulin
o Non-invasive Ventilation with BIPAP or full intubation and ventilation
Presentation of cauda equina
- Low back pain
- Bilateral sciatica
- Reduced sensation/ pins and needles in perinanal area
- Decreased anal tone
- Urinary/bowel dysfunction
- Erectile dysfunction
- Variable leg weakness is flaccid and areflexic (LMN)
- Saddle anaesthesia
Presentation of motor neurone disease
- Gait Stumbling, spastic, tripping over
- Cramps
- Wrist and foot drop
- Insidious, progressive weakness of muscles throughout body affecting limbs, trunk, face and speech
- Increased fatigue when exercising
- Clumsiness, dropping things
- Slurred speech
- Dysarthria, dysphagia, nasal regurgitation of fluids and choking
- Jaw jerk is normal/absent
- Speech is quiet, hoarse or nasal
- Weak grip and shoulder abduction
LMN signs
o Muscle wasting
o Reduced tone
o Reduced reflexes
o Split hand sign = Thumb side of hand seems adrift due to excessive wasting around it
o Flaccid, fasciculating tongue
UMN signs
o Increased tone or spasticity
o Brisk reflexes
o Upgoing plantar responses
Management of MND
- Riluzole = slow progression of disease and extend survival
- Oral propantheline/amitriptyline = prevents drooling
- Treat dysphagia = blend food, NG tube, percutaneous catheter gastrostomy
- Oral baclofen = spasms
- Analgesia
- Non-invasive ventilation if respiratory failure
- Advanced directives and End of life care planning
Presentation of multiple sclerosis
- Symptoms pattern
o Usually progress over more than 24 hrs
o At first symptoms last days to wks then improve
o Uhthoff’s phenomena = Symptoms may worsen with heat/exercise
o Improve during pregnancy and postpartum - Charcot’s neurological triad Dysarthria, Nystagmus, Intention tremor
- Sensory features
o Dysaesthesia
o pins and needles
o decreased vibration sense
o trigeminal neuralgia
o Lhermitte’s sign = sudden sensation like electric shock travels down spine into limbs worse when bending head to chest - Motor Spastic weakness and myelitis
- Neuro Cognitive problems, Depression
- Autonomic urinary urgency, frequency, incontinence, erectile dysfunction
- Cerebellar loss of proprioception, positional vertigo, ataxia
- Dysphagia
- Constipation
- Nausea and vomiting
- Unilateral optic neuritis
- Eye movement abnormalities = Diplopia, hemianopia, pupil defects
- 6th cranial nerve palsy = internuclear ophthalmoplegia and conjugate lateral gaze disorder
- Horner’s syndrome
- Bell’s palsy
- Limb paralysis
Investigations of multiple sclerosis
- Diagnosis requires 2+ attacks affecting different parts of CNS (separated by time and space)
- MRI brain and spinal cord with contrast
o periventricular lesions
o white matter abnormalities
o multiple scattered plaques
o High signal T2 lesions
o Dawson fingers = hyperintense lesions perpendicular to corpus callosum - Lumbar puncture oligoclonal IgG bands which are not in serum (not specific)
- Increased intrathecal synthesis of IgG
Management of multiple sclerosis
- Acute relapse
1. Oral methylprednisolone for 5 days
2. IV methylprednisolone 3-5 days when failed or severe - Remission = dimethyl fumarate
- Treatment with DMARDs and biological therapy
- Symptomatic Tx
o Exercise
o Amitriptyline/gabapentin = neuropathic pain
o Antidepressants (SSRIs) = depression
o Tolterodine or catheterisation = urge incontinence
o Baclofen, physio = spasticity
o Reduce stress
o Vitamin D supplements
o Botulinium toxin infections = tremor
o Amantadine, CBT, exercise = fatigue
Causes of peripheral neuropathy
- Predominantly motor loss
o Guillain-Barre syndrome
o Porphyria
o Lead poisoning
o Hereditary sensorimotor neuropathies (Charot-Marie- Tooth)
o Chronic inflammatory demyelinating polyneuropathy
o Diphtheria - Predominately sensory loss
o Diabetes
o Uraemia
o Leprosy
o Vit B12 deficiency
o Amyloidosis
o Alcoholic neuropathy = Secondary to both direct toxic effects and reduced absorption of B vits - Sensory Sx before Motor
o Vitamin B12 deficiency
o Subacute combined degeneration of spinal cord
o Dorsal column usually affected first prior to distal paraesthesia
Presentation of Guillain-Barre syndrome
- History of respiratory or GI infections 1-3 wks prior to onset
- Back and limb pain
o Symptoms typically start at feet and progress upward
o Symptoms peak 2-4 wks
o Recovery period last months to yrs - Symmetrical ascending muscle weakness
o Starts 1-3 wks post infection
o Advance quickly, affecting all limbs at once
o Can lead to paralysis
o Proximal muscles more affected = trunk, respiratory and cranial nerves (CN7) - Reduced/absent reflexes
- Peripheral loss of sensation or neuropathic pain
- May progress to cranial nerves and cause facial nerve weakness
o Diplopia
o Bilateral facial nerve palsy
o Oropharyngeal weakness
o Papilloedema - Autonomic features Sweating, raised pulse, BP changes, arrhythmias, urinary retention, diarrhoea
Investigations for GBS
- Clinical diagnosis using Brighton criteria
- Nerve conduction studies (reduced signal through nerves)
o Decreased motor nerve conduction velocity
o Prolonged distal motor latency
o Increased F wave latency - LP (L4) CSF has raised protein but normal WCC and glucose
- Spirometry = Monitor FVC if respiratory involvement
Management of GBS
- Admit to hospital
- IV immunoglobulin for 5 days
o Contraindicated in patients with IgA deficiency - Plasma exchange (alternative to IV Ig)
Presentation of common peroneal nerve lesion
- Foot drop
- Weakness of foot dorsiflexion
- Weakness of foot eversion
- Weakness of extensor hallucis longus
- Sensory loss of dorsum of foot and lower lateral part of leg
- Wasting of anterior tibial and peroneal muscles
