| Disease
| Pathogenesis
| Symptoms and Signs
| Investigations
| Treatment
|
| Left/Right Total Anterior Circulation Stroke -Large stroke with significant mortality and residual disability.
|
Large vessel atherosclerosis
Small vessel lipohyalinosis, Arteriosclerosis
Cardioembolic - AF, Mural thrombosis, atrial myxoma
Dissection - hypertension, trauma, Ehlers-danlos, Marfans
Vasculitis - Temporal arteritis, PAN
Venous thrombosis - hypercoag state, pregnancy, thrombophilia
| Bamford Classification 3 out of 3 of
1. Right/Left hemiparesis (right/left face/arm/leg) and/or Right/Left hemisensory loss
2. Right/Left homonymous hemianopia
3. Higher cortical dysfunction eg dysphasia, apraxia, neglect
Dominant side - Dysphasia, Alexia, Acalculia, R/L disorientation in Left MCA lesion
Non Dominant - dressing apraxia
|
Non contrast CT
MRI - suspect another diagnosis (eg tumour) or posterior fossa lesion suspected
FBC - polycythaemia
ESR and CRP - Vasculitis
U&E, LFT, Lipids, Glucose
ECG - AF, LVH, Recent MI
Transthoraic Echocardiogram - LVH, Poor LV function, LV aneurysm, R/L shunt "BUBBLE TEST"
Transoesophageal echo - ? PFO
Carotid dopplers if suitable for CEA
|
Consider thrombolysis
Swallowing assessment
TED stockings
Early mobilisation and stroke unit care
Aspirin and Dipyridamole
Consider Warfarin if after day 10 in infarct in AF
ACE Inhibitor + Thiazide if hypertensive after day 7
Look for underlying cause
Carotid endarterectomy (CEA) if corresponding Int carotid artery lesions
|
|
| Left/Right Partial Anterior Circulation Stroke - Similar to a TACS but less severe but with cortical involvement
|
Large vessel atherosclerosis
Small vessel lipohyalinosis, Arteriosclerosis
Cardioembolic - AF, Mural thrombosis, atrial myxoma
Dissection - hypertension, trauma, Ehlers-danlos, Marfans
Vasculitis - Temporal arteritis, PAN
Venous thrombosis - hypercoag state, pregnancy, thrombophilia
| 2 out of 3 of
1. Right/Left hemiparesis (right/left face/arm/leg) and/or Right/Left hemisensory loss
2. Right/Left homonymous hemianopia
3. Higher cortical dysfunction eg dysphasia, apraxia, neglect
Dominant side - Dysphasia, Alexia, Acalculia, R/L disorientation in Left MCA lesion
Non Dominant - dressing apraxia
|
As above
|
As above
| | |
| Lacunar strokes - Lacunar strokes are usually found deep below the cortical grey matter. Clinically differentiated from PACS by the absence of cortical features eg apraxia, dysphasia etc
| Small lacunes < 10-15 mm in size. Due to obstruction of small penetrating arteries - the lenticulostriate arteries which are branches of the middle cerebral artery and supply the corona radiata, basal ganglia, the internal capsule and thalamus and pons less than 10-15 mm in size
Hypertension - small vessels affected
In situ thrombosis
Cardioembolic
- Pure motor (damage to posterior limb of internal capsule)
- Pure sensory (thalamus)
- Sensorimotor (thalamus and posterior limb of int capsule)
- Ataxic (variable)
- Dysarthria and Clumsy hand (pons)
Non contrast CT but MRI preferred
FBC - polycythaemia
ESR and CRP - Vasculitis
U&E, LFT, Lipids, Glucose
ECG - AF, LVH, Recent MI
Transthoraic Echocardiogram - LVH, Poor LV function, LV aneurysm, R/L shunt "BUBBLE TEST"
Transoesophageal echo - ? Patent foramen ovale
|
As for Anterior circulation stroke except CEA
| | | |
| Posterior circulation stroke - more subtle in its milder forms. Overall reasonably good prognosis. Watch out for the rare but often quoted "top of the basilar" syndrome
|
Large vessel atherosclerosis
Small vessel lipohyalinosis, Atherosclerosis, Arteriosclerosis
Cardioembolic
Symptoms
Dizziness, Vertigo
Diplopia
Nausea, Vomiting
Nystagmus
Cerebellar signs
Altered consciousness
Horner's syndrome
Bulbar signs - impaired swallow
Chorea, hemiballismus
Non contrast CT but MRI preferred
FBC - polycythaemia
ESR and CRP - Vasculitis
U&E, LFT, Lipids, Glucose
ECG - AF, LVH, Recent MI
Transthoracic Echocardiogram - LVH, Poor LV function, LV aneurysm, R/L shunt "BUBBLE TEST"
Transoesophageal echo - ? Patent foramen ovale
|
As for Anterior circulation stroke except CEA
| | | |
| Multiple Sclerosis - Chronic demyelinating disease due to a possible autoimmune basis. Motor and sensory but no LMN lesions
|
Patients typically 20-40 years old
Commoner in northern latitudes
Affects cerebellum, brainstem and connections, periventricular white matter. Corpus callosum and spinal cord.
Signs and symptoms disseminated in time and anatomical location
McDonald criteria may be used
Affects saltatory conduction
Clinical patterns of disability and time
Primary progressive
Secondary progressive
Relapsing remitting 70%
Fulminant
Optic neuritis - altered monocular vision/blindness, eye pain, red inflamed disc which later becomes pale
Cord lesion with spastic paraparesis
Worsened with heat - Uthoff's phenomenom
Trigeminal neuralgia (always consider MS esp in younger patient)
Fatigue can be debilitating - look for other causes anaemia, thyroid
Transverse myelitis - leg weakness, sensory level, bowel and bladder disturbance
Cerebellar - ataxia, nystagmus, past pointing
Brainstem - Diplopia, swallowing difficulties, Internuclear ophthalmoplegia
Regional sensory loss - tingling paraestheisa
Lhermittes sign - neck flexion gives electric shock pain
MRI with gadolinium of brain and cord - shows plaques and demyelination. Periventricular, corpus callosum, brainstem, spinal cord.
Visually evoked responses delayed
Lumbar puncture - oligoclonal bands (non specific and less required test)
|
Young and female do statistically better
Acute episodes - IV Methylprednisolone shorten flare ups but no benefit long term
Relapsing remitting disease - Beta interferons and Glatiramer acetate. Strict criteria for usage.
Manage spasticity, fatigue, bladder, bowels
Multidisciplinary approach
| | | |
| Guillain Barre syndrome - Typically an Acute inflammatory demyelinating polyneuropathy (AIDP) in 90% presenting with an acute weakness onset over days with areflexia sometimes following a recent infective illness
|
Pathogenesis seems to be an immune response to a foreign substance eg CMV, Campylobacter which excites an immune response against shared epitopes on myelinated cells.
Various antibodies found - anti GD1a (Acute axonal degeneration), GQ1b (Miller-Fisher variant)
Inflammatory cells have easier access to nerve roots so proximal weakness and back pain can mark onset
Some Back pain - involvement of nerve roots
Paraesthesia in lower limbs
Ascending polyneuritis but often proximal
Difficulty walking and getting out of a chair
Motor weakness and Areflexia
Decreased sensation distally
Autonomic neuropathy
Reduced FVC and respiratory failure
Miller Fisher variant - ataxia, ophthalmoplegia, areflexia and anti-GQ1 antibody
Lumbar puncture - raised protein but cells < 50
Abnormal nerve conduction studies
|
IVIG
Plasmapheresis
Steroids - have no value and not used
Intensive care if FVC < 1.5 L
ECG and Pulse BP - autonomic irregularities
Physiotherapy to reduce wasting, contractures and encourage positioning to await recovery
LMWH - prevent DVT/PE
| | | |
| Chronic Inflammatory Demyelinating polyneuropathy
| Similar to GBS
| Same as GBS but a slower onset over weeks/months
| Same as GBS
|
IVIG
Plasmapheresis
Steroids
|
| Epilepsy - A seizure is an abnormal paroxysmal discharge of cerebral neurones resulting in a clinical event and Epilepsy is the tendency to have seizures. Increased mortality associated.
| Pathogenesis
Idiopathic
Brain injury - surgery, trauma, stroke, encephalitis, tumour, hypoxia
Congenital
Drug induced
Eclampsia
Simple (Consciousness unaffected) Complex (consciousness affected)
Partial seizures start locally and suggest a structural cause. Generalised - both hemispheres involved from onset
Can start focal and go on to be generalised
Triggered by tiredness, menstruation, drugs, alcohol
Investigations
MRI is the imaging modality of choice now particularly where there are focal or partial symptoms
EEG
| Treatment of status
ABCs - High flow Oxygen and manage airway
IV or IV lorazepam / IV phenytoin / Get ITU help early
Ask why ? infection ? intracranial event ? CT
Treatment of Epilepsy
| | | |
| Dystrophica Myotonica - autosomal dominant disorder with facial, cardiac and myotonic features x
|
Seen in Male and female diagnosed age 15-40
CTG trinucleotide repeats and anticipation.
Frontal balding
Ptosis and cataracts
Smooth expressionless forehead
Wasted facial/Sternomastoid/Shoulder and Quads
Myotonia - difficulty relaxing following contraction
Male and female infertility
Learning disability
Heartblock
ECG - heart block
Genetic analysis
| Treatment
Genetic advice
Phenytoin may help myotonia
| | | |
| Motor Neurone Disease/Amyotrophic lateral sclerosis(US term) - a severe and progressive degenerative neurological disease with mixed upper and motor neurone weakness - wasting and hyperreflexia. No sensory involvment. Exclude other treatable causes
|
Age > 60 years but also seen in younger
Familial cases (10% of cases) have impaired superoxide dismutase (SOD1) activity suggesting a possible oxidative damage hypothesis.
End result is degeneration of corticospinal and corticobulbar neurones and anterior horn spinal nerves too.
Wasting + hyperreflexia + no sensory, spared eyes, sphincters intact
Clinical forms
Amyotrophic lateral sclerosis - LMN + UMN
Progressive bulbar palsy - (includes pseudobulbar palsy)
Progressive muscular atrophy - LMN predominant
Lateral sclerosis - UMN only
Weakness respiratory muscle
Absent sensory symptoms and signs and involvement of extraocular muscles rare
Investigations very much to exclude other diagnoses.
MRI of head and neck
EMG fasiculations and fibrillation potentials
Mild Raised CK with cramps and spasticity
|
Holistic Support and General nursing care
Skin care and hydration
Riluzole may have some value - trials suggest adds 2 months has been approved by NICE
PEG feeding in some cases
Mean Prognosis is 2 years survival after diagnosis
Terminal care and palliation
| | | |
| Myasthenia Gravis - autoimmune disease resulting in fatiguable weakness due to damage to postsynaptic Ach receptor
| Antibodies produced to the Nicotinic Acetyl choline receptor block neuromuscular transmission. Leads to weakness of certain muscle groups. Immune mechanism. Commoner in women.
|
A weakness that fluctuates and is fatiguable
Worse later in day and after exercise
Motor weakness
Diplopia and Ptosis
Myasthenic weak smile "snarl"
Dysphagia
Respiratory weakness
Unable to raise chin
|
EMG - electrodecremental response with repeated stimulation
Anti Acetylcholine receptor antibodies
Tensilon test IV edrophonium improves symptoms and signs in 3-5 mins
CT chest to look for thymoma
Raise Ach using Acetyl cholinesterase blockers eg edrophonium
Long term Pyridostigmine 30-60 mg qds
Corticosteroids, Azathioprine, Mycophenolate, Cyclosporin A
Thymectomy in under 60s
| | |
| Peripheral neuropathy
| Pathogenesis
| Symptoms
| Signs
| Treatment
|
| Brain tumours - can be primary brain tumours arising from glial cells/neurones or other tissues. Also can be secondary from other sites. Neuroimaging and possible biopsy indicated.
|
Primary brain: Glial cells - Gliomas : Milder Astrocytomas to aggressive Glioblastma mulitforme, Meningiomas
Secondary: Breast, Lung, Colorectal, Thyroid, Testicular, Renal cell and malignant melanoma
HIV : non-Hodgkin's lymphomas of B-cell type
Symptoms due to local effects and general rise in ICP
Childhood tumours are mainly posterior fossa which present with hydrocephalus
Headache - worse in morning and on stopping/straining
Changed personality, Seizures
Stroke like episodes when tumour bleeds, Coma
Craniopharyngioma in the suprasellar region, causing pituitary dysfunction and bitemporal hemianopia
CT with contrast /MRI with gadolinium
Look for primary
|
Discuss with Neurosurgeons whether to biopsy lesion
Start anticonvulsants with one seizure
Dexamethasone to reduce inflammation and oedema
Chemotherapy/radiotherapy or surgery of use in some
| | | |
| Colloid cyst in the third ventricle - sudden death in children and young adults
| Causing intermittent hydrocephalus
|
Causing intermittent hydrocephalus
Sudden death
| Signs - headache, raised ICP and coma, Sudden death
|
Surgical treatment
| |
| Pituitary lesions -Hormonal active benign tumours producing localised pressure effects and secondary hormonal effects
| Vertical height
> 10 mm = macroadenoma
= 10 mm = mesoadenoma
< 10 mm = microadenoma
Check visual fields. Ask about
Morning Headache and signs of hypopituitarism, Decreased sex drive, impotence, amenorrhoea
Prolactin - reduces testosterone and causes mild gynaecomastia and in some cases galactorrhoea
Non functioning - headache and visual effects
Growth hormone - causes acromegaly
Others
| Signs
| Treatment
| | |
| Syringomyelia/bulbia
| Pathogenesis
| Symptoms
| Signs
| Treatment
|
| Charcot Marie Tooth
| Pathogenesis
| Symptoms
| Signs
| Treatment
|
| Polymyositis/Dermatomyositis
| Pathogenesis
| Symptoms
| Signs
| Treatment
|
| Subarachnoid Haemorrhage - blood in the subarachnoid space due most commonly to a leaking berry aneurysm. Aim is to treat aneurysm where possibly before major vascular brain injury
|
Berry Aneurysm due to weakness at points between arteries in circle of willis
Arteriovenous malformations
Tumours, dissections rare causes
Idiopathic
Association between Berry aneurysm and Polycystic kidneys
Thunderclap headache
Neck stiffness
Coma
Coma + recovery
Neck stiffness
Seizure
Painful IIIrd nerve palsy (Posterior communicating aneurysm)
None
|
Initial ABCs + Hydration + Nimodipine
Non contrast CT shows bleed in 95%
CT Negative need LP at 6-12 hrs for Blood and Xanthochromia
CT angiography to identify aneurysm 1)Anterior communicating 2)Middle cerebral 3) Posterior communicating 4)Basilar
Berry aneurysms - clip via neurosurgeons or coil by neuroradiologists
| | | |
| Migraine - recurrent familiar headaches with systemic symptoms and usual precipitants such as stress, sunshine, menstruation, foods, exercise, irregular meals
| Spreading waves of cortical depression. Exact underlying cause unclear. Possible Calcium channels in some familial variants
Onset often in childhood
Patient has typical well known attacks
Classical migraine - preheadache aura
Common migraine - headache + nausea and no aura, GI disturbance
aura may be visual or weakness or some other transient neurological manifestation - can mimic a TIA
Photophobia - prefers peace and quiet in dark room
Sleep if possible helps
A clinical diagnosis
CT may be indicated if other causes need excluded
Consider CT in new onset older patient
Acute attack
High dose Aspirin, Ibuprofen
Sumatriptans
Prevention
Beta Blockers eg Propranolol
Low dose Amitriptyline
Sodium Valproate
Pizotifen
| | | |
| Idiopathic intracranial hypertension / Benign Intracranial hypertension - Headache and potential loss of sight with papilloedema in young females but first exclude venous thrombosis
|
Unknown cause but affects young females.
Pregnancy and OCP, thrombophilia, retinoids, Vitmain A, tetracycline
Headache classical for increased ICP - mimics a brain tumour
Nausea
Blurred vision and blind spot enlarged
Possible VIth nerve lesion
CT normal
LP - high opening pressure after CT/MR
Exclude venous thrombosis with MRI/MRV
|
Weight loss
Treat underlying cause
V/P shunting
Monitor for visual loss
| | | |
| Cauda equina lesion
| Pathogenesis
| Symptoms& Signs
| Investigations
| Treatment
|
| Cervical cord lesion
| Pathogenesis
| Symptoms& Signs
| Investigations
| Treatment
|
| Essential tremor- rather benign in most but can be disabling and socially embarrassing. Main focus is not to be misdiagnosed as Parkinson's disease
| Familial - autosomal dominant
|
Tremor in anxious situations
Classically helped by alcohol
Head nodding - titubation
Voice tremor
| None
|
Reassurance that its not PD
Try Beta Blockers or primidone
| |
| Idiopathic Parkinson's disease - degenerative disease of the substantia nigra resulting in impaired function of the normal neural extrapyramidal motor circuits producing a classical clinical picture
|
80% Degeneration of the substantia nigra
loss of dopamine releasing neurones.
Eosinophilic intracellular Lewy bodies
Often asymmetrical pathological signs and clinical signs
Asymmetry makes IPD more likely
TRAP : Tremor, Rigidity (lead pipe or cog wheel), Akinesia, Postural instability
Freezing
Mask face, constipation, siallorhoea
Hypophonia, Micrographia
Dementia, Positive Glabellar tap (rarely done)
Depression - common and should be treated
Test functionality - doing up buttons, assessing gait
Diagnosis is mainly clinical
CT may be done to exclude other causes eg stroke, tumours
|
MAOB inhibitor eg selegiline or rasagiline
Levodopa + peripheral decarboxylase inhibitor
Dopamine agonists
Anticholinergics
Levodopa + COMT inhibitors
Treat depression if present
Surgery - deep brain stimulation
| | | |
| Parkinsonism - a syndrome characterised by Tremor, Rigidty and Akinesia
|
Idiopathic PD
Vascular damage
Drug induced MPTP, Neuroleptics, Dopamine blockers, Reserpine
Carbon monoxide poisoning, Manganese
Wilson's disease (copper overload)
Repeated trauma - boxers
Post encephalitis lethargica
May have additional features suggesting another cause
Postural instability, eye signs -suggests PSP
Symmetry, failure of response to L-Dopa - not typical for IPD
Early falls, Autonomic,Cerebellar signs - consider Multiple system atrophy
Diagnosis is mainly clinical
CT may be done to exclude other causes eg stroke, tumours
Copper studies in young. Drug history.
|
Levodopa + peripheral decarboxylase inhibitor
Stop offending drug
Treat for cerebrovascular disease
| | | |
| Huntington disease - inherited movement disorder and early onset dementia. Pathology shows mainly neuronal loss in caudate and putamen
|
Autosomal dominant
Gene for Huntingtin on Chromosome 4p.
CAG Trinucleotide repeats.
Demonstrates Anticipation :- The more repeats the earlier the symptoms.
Choreiform movements, Tics
Parkinsonism
Progressive dementia
Diagnosis is mainly clinical
CT may be done and shows atrophy of caudate
|
Sulpiride or Tetrabenazine may reduce chorea
| | | |
| IIIrd nerve lesion - nucleus is in the midbrain. Carries fibres to both pupil and ocular muscles and levator palpebrae superioris
| Can be medical or surgical.
Surgical - Posterior communicating Berry aneurysm presses on nerve or space occupying lesion
Medical - most commonly diabetes or small vessel vasculitis with ischaemia of nerve but peripheral lying fibres to constrictor pupillae spared
Complete ptosis
Eye points down and out
Dilated pupil (pupil normal in diabetes and medical causes)
Investigations
CT Angiography or MRA if Berry aneurysm suspected
Diabetes - Glucose, HbA1C
CRP/ESR for vasculitis
Treatment
Surgical causes may require referral
| IVth nerve lesion - nucleus is in midbrain. Supplies superior oblique
| Can be medical or surgical ( space occupying lesion)
|
Diplopia maximal on reading or descending stairs
CT or MR exclude space occupying lesion/other pathology
Exclude Diabetes - Glucose, HbA1C
CRP/ESR for vasculitis
|
Ophthalmology referral
| | |
| VIth nerve lesion - nucleus is in pons. long course. Supplies lateral rectus
|
Can be medical or surgical.
False localising sign in raised ICP
Failure to abduct
Diplopia maximal on looking to side
Investigations
CT / MR exclude space occupying lesion/other pathology
Diabetes - Glucose, HbA1C
CRP/ESR for vasculitis
| Treatment
Surgical causes may require referral
| | | | | | | |