Neurology 2 Flashcards
Shingles aetiology
VZV reactivation
Immunosuppression
- Elderly
- HIV
- Steroids
- Chemotherapy
Shingles presentation
Pre-eruptive
- Itching and burning
- Paraesthesia
- B-symptoms
Painful rash - Dermatomal!
- Clusters of small vesicles
Herpes zoster ophthalmicus - Ophthalmic branch of trigeminal
- Visual loss
- Needs urgent referral
- Hutchinson sign - Rash on nose
Shingles management
Acyclovir - Within 72 hours
Pain relief
- Paracetamol
- Ibuprofen
- Gabapentin
- Pregabalin
- Amitriptyline
Housekeeping
- Contagious - Chickenpox
- Vaccine offered 70-79
Bell’s palsy aetiology
Facial nerve paralysis - Acute, unilateral
Ipsilateral LMNL
Infection - EBV / HSV SOL - Parotid tumour GBS Forceps delivery Increased risk in pregnancy and DM
VZV - Ramsay Hunt syndrome
Bell’s palsy presentation
CN7 palsy
- Speech disturbance
- Eating disturbance
- Dry eyes
- Taste - Loss of anterior 2/3 tongue
- Post-auricular pain/numbness
- Hearing - Hyperacusis
Bell’s palsy investigations and management
Serology - VZV
CT/MRI
Management - Prednisolone + protect the eye
- Consider antivirals
GBS pathophysiology
Immune-mediated
Demyelination of PNS
Triggered by infection
Campylobacter jejuni
GBS presentation
AAAAA - Symmetrical
Ascending weakness - Proximal muscles Absent reflexes Autonomic dysfunction - Urinary retention - Tachycardia - Arrhythmias Abnormal eyes - Diplopia Ataxia
Paraesthesia
Respiratory depression
GBS investigations
Nerve conduction studies
Anti-ganglioside antibodies
Spirometry
LP - Protein ^
ECG - Arrhythmias
GBS management and complications
IV IG
ECG - Monitor arrhythmias
VTE prophylaxis
Severe - Plasmapheresis
Complications
- Respiratory depression
- Death
MS aetiology
+ Twins?
Cell-mediated AI disorder
Demyelination in CNS
3x more common in women
Aged 20-40
Monozygotic twin with MS = 30% risk
Dizygotic twin with MS = 2% risk
MS types
Relapsing-remitting - Most common
- Acute attacks followed by periods of remission
- 65% develop secondary progressive after 15 years
Secondary progressive
- Relapsing-remitting patients who have deteriorated
- Signs/symptoms present between relapses
- Gait and bladder disorders
Primary progressive - 10%
- Progressive deterioration from onset
- More common in elderly
MS presentation
Visual
- Optic atrophy
- Uhthoff’s phenomenon - “Worse after a bath”
Internuclear ophthalmoplegia
Sensory
- Numbness / paraesthesia
- Trigeminal neuralgia
- Lhermitte’s - Limb paraesthesia with neck flexion
Motor - Spastic weakness - Most common in legs
Cerebellar - Ataxia - Usually in acute relapse
Urinary incontinence
Sexual dysfunction
Intellectual deterioration
MS investigations
MRI - 2 lesions disseminated in time and space
LP - Oligoclonal bands
Anti-MOG antibodies
MS management
Acute - Methylprednisolone - 5 days
Chronic
- Beta-interferon
- Glatiramer acetate
- Natalizumab
- Fingolimod
Symptom control
- Muscle relaxant - Baclofen
- Tremor - BB
MG aetiology
AI disorder
Antibodies to acetylcholine receptors
More common in women
Exacerbations caused by…
- Drugs - BB, opioids, gent, Li
- Pregnancy
- Infection
- Change in environment
MG presentation
Weakness and fatigability!
Eyes
- Diplopia
- Ptosis
- Peek sign - Gentle sustained lid closure, then separate
Bulbar - D
- Dysphagia
- Dysphasia
- Difficulty chewing
Proximal weakness
Normal reflexes
“Can’t walk upstairs”
“Difficulty watching TV - Eyes get tired”
Ask them to count to 50 - Voice trails off!
MG investigations
Electromyography - Decreased evoked potentials
Anti-MuSK antibodies
Anti-AchR antibodies
CT - Thymoma
Spirometry
MG management and complications
Pyridostigmine
Prednisolone
Azathioprine
Complications - Respiratory
Lambert-Eaton syndrome - Associated with SCLC
- Repeated muscle contraction
- Increased strength
Myasthenic crisis
Known MG
Increasing generalised or bulbar weakness
Respiratory depression
Management
- Intubation / ventilation
- Plasma exchange - 2-3 sessions
- IVIG - 4-5 days
- Prednisolone
- Eculizumab
MND aetiology
Apoptosis of motor neurons - SOD1 mutation
ALS - Most common - Mixed
- UMN + LMN
- Loss of neurons in motor cortex
Progressive muscular atrophy - LMN - Distal to proximal
Primary lateral sclerosis - UMN
Progressive bulbar palsy
- Facial muscles, tongue, muscles of chewing/swallowing
- Loss of function of brainstem motor nuclei
MND presentation
UMN + LMN
UMN
- Upgoing plantars
- Increased reflexes
- Clonus
- Spasticity
LMN
- Fasciculations
- Decreased reflexes
- Wasting
- Atrophy
Asymmetrical + Purely motor = ?MND
MND presentation
Bulbar + Limb
Bulbar - D
- Dysphagia
- Dysphasia
- Dysarthria
- Drooling
- Emotionally labile
Limb
- Foot drop
- Asymmetrical weakness
- Wasting - Thenar + Ant. tibialis
Asymmetrical + Purely motor = ?MND
MND complications
Speech difficulties
Aspiratory pneumonia
Respiratory failure
UTI
Constipation
Immobility complications - Skin ulcers
MND investigations
Clinical diagnosis
El Escorial diagnostic criteria
EMG
- Decreased action potential
- Increased amplitude
MND management and prognosis
Symptom control
Spasticity - Baclofen
Feeding - NG/PEG
NIV - BiPAP
Respiratory failure - Opioids
MDT
- PT
- OT
- SALT
Riluloze prolongs life by 3 months
Prognosis - 2-4 years
- 50% die within 3 years
- Respiratory failure
Meningitis aetiology
Neonates - GBS / E.Coli
Children / adults - NM / Strep P
Viral
- HSV
- EBV
- Mumps
- Adenovirus
Meningitis presentation
- Neck stiffness
- Fever
- Headache
Photophobia
Non-blanching purpuric rash
Kernig’s sign
Brudzinski’s sign
Late presentation…
- Seizures
- FND
- Coma
Meningitis investigations
Sepsis 6
LP - CI if ICP ^ Blood cultures PCR virus FBC U&E CRP/ESR Glucose ABG
Meningitis CSF findings
Bacterial
- Cloudy
- PMNs ^
- Protein ^^^
- Glucose - LOW
Viral
- Clear
- Lymphocytes
- Protein ^
- Glucose - Normal / Low
TB
- Cloudy / Fibrin web
- Monocytes
- Protein ^^^
- Glucose - Very LOW
Meningitis management
Community - IM BENPEN
IV Cef
Listeria - Amox
Household contact - Rifampicin
Paediatrics < 3 months - IV Amox + Cef > 3 months - IV Cef - Fluids - Cerebral monitoring
Notify PHE!
Meningitis complications
Sensorineural deafness
Abscess
Septicaemia
Encephalitis aetiology
Inflammation of cerebral cortex
HSV Enterovirus HIV Mumps Measles - Spontaneous sclerosing panencephalitis Lyme disease TB
Encephalitis presentation
- Personality changes
- Fever
- Headache
FND
ICP ^
Seizures
Coma
FND + Personality changes = Encephalitis (vs Meningitis)
Encephalitis investigations and management
LP - Viral picture
- Clear
- Lymphocytes
- Protein ^
- Glucose - Normal or LOW
Bloods - Culture, PCR, clotting, ESR
EEG
MRI - Temporal lobe involvement?
Management - IV acyclovir
Horner’s syndrome aetiology
STC!
Central - S
- Stroke
- MS
Preganglionic - T
- Tumour
- Thyroidectomy
- Trauma
Post-ganglionic - C
- Cluster headache
- Cavernous sinus thrombosis
- Carotid dissection
Horner’s syndrome presentation
Ptosis
Miosis - Small pupil
Anhidrosis - Unilateral loss of sweating
Enophthalmus - Sunken eye
Horner’s syndrome investigations and management
Apraclonidine - Affected eye does not dilate
CT - Suspected tumour
CXR - Pancoast tumour
Management - Treat cause
Anterior cord syndrome aetiology
Ischaemia of the anterior spinal artery
- Branch of vertebral artery
- Comes from aorta
Most commonly due to aortic compromise
- Dissection
- Aneurysm
- Vasculitis
Anterior cord syndrome presentation / investigations / management
Paralysis
Loss of pain and temperature sensation
Fine touch preserved - Dorsal column intact
Areflexia
Autonomic failure
- Urinary/bowel
- Sexual dysfunction
Investigations - CT angiography
Management - Treat cause
Cerebellar disorders aetiology
Vitamin D
Vascular - Stroke Infection - Meningitis, encephalitis, VZV, mumps Trauma AI Metabolic - B12, thiamine, hypothyroid, hypoPTH Idiopathic Neoplasm Degnerative Drugs - Li, phenytoin, isoniazid, Met.
Cerebellar disorders presentation
DANISHH - P
Dysdiadochokinesis Ataxia - Cerebellar vermis Nystagmus Intention tremor Slurred speech Heel-shin test Hypotonia
Pendular reflexes
Peripheral neuropathy aetiology
Motor
- Infection
- GBS
Sensory
- DM
- B12 deficiency
- Uraemia
Alcohol
SLE
Thyroid disease
Chemo/radio
Peripheral neuropathy investigations
Bloods
- FBC
- U&E
- LFT
- Toxicology
- Cultures - Infection?
- Anti-Ro / Anti-La - SLE?
Nerve conduction studies - GBS?
Neurofibromatosis
Type 1 - Ch17 - AD
- SKIN LESIONS
- CAFE AU LAIT SPOTS
- Axillary freckles
- Optic lesions
- Scoliosis
- Phaemochromocytoma
Type 2 - Ch20 - AD
- TUMOURS
- B/L ACOUSTIC NEUROMAS
- Ependymomas
- Schwannomas
- Meningiomas
Brain tumours aetiology
Metastatic - Most common - Non-surgical
- Lung - Most common
- Breast
- Bowel
- Skin - Melanoma
- Kidney
Glioblastoma multiforme
Most common primary tumour
Imaging
- Solid tumour
- Central necrosis
- Rim enhances contrast
- BBB disruption - Vasogenic oedema
Histology - Pleomorphic with necrotic areas
Management
- Surgical excision
- Chemo/Radio
- Dex - Reduce oedema
Prognosis - Poor - 1 year
Meningioma
Second most common primary tumour
Benign extrinsic
Arise from dura mater
Cause symptoms by compression rather than invasion
Typical locations
- Falx cerebri
- Superior sagittal sinus
- Convexity
- Skull base
Histology
- Spindle cells in concentric whorls
- Calcified psammoma bodies
Investigations - CT/MRI
Management - Surgery ± Chemo/Radio
Vestibular schwannoma
Benign
Arises from CN8 - Vestibulocochlear
Associated with NF2
Clinical features
- Hearing loss
- Facial nerve palsy (compression)
- Tinnitus
Histology
- Antoni A or B patterns
- Verocay bodies - Acellular areas surrounded by nuclear palisades
Management - Surgery ± Chemo/Radio
Pliocytic astrocytoma
Most common primary brain tumour in children
Histology - Rosenthal fibres
Corkscrew eosinophilic bundle
Pituitary adenoma
Benign tumour of pituitary gland
Secretory or non-secretory
Microadenomas < 1cm
Macroadenomas > 1cm
Clinical features
- ACTH - Cushing’s
- GH - Acromegaly
- Bitemporal hemianopia
Investigations
- CT/MRI
- Pituitary blood profile
Management - Hormonal / surgical
Brain abscess aetiology
VITAMIN DC
V - CHD - R-L shunting Infection - OM, dental, meningitis, endocarditis, mastoiditis, HIV, IVDU Trauma/surgery AI - DM, chronic granulomatous disease M Iatrogenic - Haemodialysis N
D
C - Prematurity
Brain abscess clinical features
Depends on site
Headache - Dull and persistent Meningism CN palsy - 3 or 6 Kernig / Brudzinski +ve Fever
Neonates - Increasing head circumference / bulging fontanelle
Brain abscess investigations
Sepsis 6
FBC - WCC ^
ESR / CRP ^
Blood cultures
Leucocytosis!
CT / MRI - Halo around lesion
Neonates - USS head
Brain abscess management
Surgery - Craniotomy + Debridement
IV abx - Cef + Met
ICP management - Dex
Myopathies aetiology
Inflammatory - Polymyositis
Inherited
- Duchenne’s / Becker’s
- Myotonic dystrophy
Endocrine
- Cushing’s
- Thyrotoxicosis
Alcohol
Myopathies clinical features
Symmetrical weakness - Proximal to distal
Difficulty standing from sitting
Difficulty getting out of bath
Normal sensation
Normal reflexes
No fasciculations
Duchenne’s aetiology
Progressive generalised muscle disease
X-linked
Becker muscular dystrophy is a milder form!
Duchenne’s clinical features
Lower limbs - Flexion > Extension
Delayed motor milestones - Ambulation > 18 months
Gower’s sign - Use of arms to stand from squatting
Contractures
Decreased tone/reflexes
Toe walking
Normal sensation
Urinary and bowel incontinence
Intellectual impairment
Associated with dilated cardiomyopathy
Duchenne’s investigations
Serum CK - 50-100x normal level
Genetic testing - Xp21 mutation
Duchenne’s management and prognosis
Prednisolone
PT
Surgery for contractures
LVEF < 40-50% - Cardioprotective drugs
- Carvedilol
- Perindopril
- Lisinopril
- LVAD
Prognosis - 40 year survival - 10-40%
- Progressive respiratory weakness - Treated with NIV
