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Neuro Flashcards

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0
Q

How can the lesions in MS be described?

A

Disseminated in space and time

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1
Q

What is Lhermitte’s sign and in which disease is it seen?

A

Seen in MS. It is when electric shock-like pain occurs in trunk and limbs when the neck is flexed

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2
Q

What is the mean age of onset of MS?

A

30

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3
Q

What kind of symptoms occur with MS?

A

Sensory- pins and needles, Lhermitte’s sign, trigeninal neuralgia, reduced vibration sense

Motor: myelitis, spastic weakness

Sexual/GU: erectile dysfunction, anorgasmia, urine retention

GI: constipation, swallowing disorder

Cerebellum: ataxia, intention tremor, monotonous speech

Eye: diplopia, hemianopia, INO, optic neuritis, pupil defects

Cognitive decline

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4
Q

What is the pathogenesis of MS?

A

Autoimmune disease attacking myelinated axons

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5
Q

What worsens MS symptoms?

A

Heat/exercise

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6
Q

What is the lifetime risk of MS in the UK?

A

1:1000

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7
Q

How is MS diagnosed?

A

Clinical diagnosis:
Past episodes e.g. Brief unexplained vision loss
Attacks lasting more than 1 hr with more than 30 days between attacks

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8
Q

What is seen in the CSF of MS patients

A

Oliclonal bands of IgG in >90% of patients

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9
Q

What can be used to identify a demyelinating event?

A

Evoked potentials

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10
Q

What are the differential diagnoses for MS?

A

Spinal cord neoplasms, subacute combined degeneration of the cord, transverse myelitis, progressive multifocal leukoencephalitis

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11
Q

What is the treatment for MS?

A 
Steroids (for acute attacks)
DMDs (to reduce number of relapses):
-interferon alpha and beta
-monoclonal antibodies (alemtuzumab and Natalizumab)
-azathioprine
-glatiramer acetate
Treatment of spasticity- baclofen, gabapentin
Treatment of tremor-Botox
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12
Q

What is the definition of a relapse?

A

Appearance of new symptoms or the return of old symptoms for a period of 24 hours or more in the absence of a change in core body temperature or infection

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13
Q

What are the 4 types of MS?

A

Relapsing-remitting
Secondary progressive
Primary progressive
Progressive-relapsing

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14
Q

What is CJD caused by

A

Prions- midfielder forms of normal proteins which can transform other proteins into prion proteins

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15
Q

What kind of changes do prion proteins cause in the brain?

A

Spongiform changes- cavities and tubulivesicular structures in the brain

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16
Q

What are the 2 types of CJD

A

Variant CJD- transmitted in BSE contaminated beef

Classic CJD- occurs spordically due to mutation

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17
Q

What is the mean age of onset for classic CJD and variant CJD?

A

Classic: 68
Variant: 28

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18
Q

What is the mean duration of illness for classic and variant CJD?

A

Classic: 4-5 months
Variant: 13-14 months

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19
Q

What are the symptoms of CJD?

A 
Progressive dementia
Focal neuro signs
Myoclonus
Depression
Eye signs
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20
Q

What is Guillain-Barré syndrome?

A

Acute inflammatory demyelinating neuropathy

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21
Q

What are the triggers for Guillain-Barré syndrome?

A

Campylobacter jejuni, CMV, mycoplasma, HZV, HIV, EBV, vaccinations

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22
Q

What is the treatment for Guillain-Barré?

A

Plasma exchange
IV immunoglobulin
Supportive therapy

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23
Q

What are the symptoms of Guillain-Barré?

A

Acute ascending paralysis with glove-and-stocking weakness
Progressive weakness of all 4 limbs (days- weeks)
Arreflexia
CN palsies
Paresthesiae
Autonomic dysfunction (sweating, ⬆️pulse)

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24
What is the prognosis for Guillain-Barré?
85% make a full recovery | 10% unable to walk at 1 year
25
Differential diagnosis for Guillain-Barré?
Acute myelopathy Chronic inflammatory demyelinating polyneuropathy HIV peripheral neuropathy Spinal cord compression
26
Signs and symptoms of spinal cord compression
``` Leg weakness Sensory loss Spinal/root pain may precede this Urine/faecal incontinence LMN signs at level UMN signs below level (but tone and reflexes reduced in acute cord compression) ```
27
What are the main causes of spinal cord compression?
1. Vertebral body neoplasms 2. Disc and vertebral lesions (trauma, degenerative disease) 3. Inflammatory (epidural abscess, TB) 4. Spinal cord neoplasms 5. Rarities- Paget's disease, epidural haemorrhage , bone cysts
28
Differential diagnoses for cord compression
``` Transverse myelitis MS carcinomatous meningitis Cord vasculitis (syphillis) Trauma Guillain-Barré Dissecting aneurism ```
29
What is the most common cause of cauda equina syndrome?
Lumbar disc prolapse at L4/L5 and L5/S1
30
Signs and symptoms of cauda equina lesion
``` Bladder and bowel dysfunction Mixed UMN/LMN leg weakness- ASYMMETRICAL Saddle numbness Back pain Erectile dysfunction Arreflexia ```
31
What are the causes of foot drop?
``` Diabetes Common peroneal nerve palsy Stroke Prolapsed disc MS ```
32
What causes leg weakness with no sensory loss?
Motor neurone disease Polio Parasaggital meningioma
33
What causes chronic spastic paraparesis?
``` MS Cord tumour Motor neurone disease SCDC Syringomyelia ```
34
What causes chronic flaccid paraparesis?
Peripheral neuropathy | Myopathy
35
What causes absent knee jerks and extensor plantars?
Motor neurone disease/myeloradiculitis Friedrich's ataxia SCDC Tabes dorsalis
36
What are the differential diagnoses for blackouts?
1. Epilepsy 2. Postural hypotension 3. Syncope 4. Cardiac causes
37
What is syringomyelia?
Fluid-filled cavity in the spinal cord
38
What is syringobulbia?
Fluid-filled cavity in the brainstem
39
What is the most common cause of syringomyelia and syringobulbia?
Blockage of CSF flow from the 4th ventricle (due to Arnold-Chiari malformation) Other causes: SAH, post infection, masses, post trauma, post AVM rupture
40
What is an Arnold-Chiari malformation?
Congenital herniation of the cerebellar tonsils through the foramen magnum
41
What is the normal presentation of syringobulbia or syringomyelia?
Presentation in 3rd/4th decade of life with pain and sensory loss (pain+temperature only) in the upper limbs and upper trunk only Damage to pyramidal tracts, anterior horn cells and medulla cause wasting in the hands and claw hand Spastic paraplegia nystagmus Horner's syndrome Charcot's joints Syringobulbia: nystagmus, tongue atrophy, pharyngeal/palatal weakness, facial sensory loss
42
What is the treatment for syringomyelia and syringobulbia?
Decompression of the foramen magnum
43
What is Friedreich's ataxia?
Progressive degeneration of the cerebellum and spinocerebellar tracts --> cerebellar ataxia, dysarthria, nystagmus Tendon reflexes absent (peripheral n. damage) Loss of dorsal columns--> absent joint position and vibration sense Loss of corticospinal tract--> weakness and up going plantar Pes cavus Optic atrophy Death by middle age
44
What is motor neurone disease?
Destruction of UMNs and anterior horn cells in brain and spinal cord
45
What is the life expectancy for MND and what do patients die of?
3 years- die of resp failure due to bulbar palsy and pneumonia
46
Is MND more common in men or women? Is it familial?
Men Most cases sporadic (not familial) Familial cases can be due to mutations in SOD-1 (responsible for clearing up free radicals)
47
What are the 4 clinical patterns of MND?
1. Progressive muscular atrophy 2. Amyotrophic lateral sclerosis 3. Progressive bulbar and pseudobulbar palsy 4. Primary lateral sclerosis
48
What is progressive muscular atrophy?
LMN lesion of the cord | Hands and arms- weakness, wasting, fasciculation
49
What is amyotrophic lateral sclerosis?
Disease of lateral corticospinal tracts and anterior horn cells Progressive spastic tetra paresis/paraparesis LMN signs
50
What is progressive bulbar and pseudobulbar palsy?
Destruction of UMN (pseudobulbar palsy) and LMN (bulbar palsy) in cranial nerves Dysarthria+dysphagia Wasting and fasciculation of tongue
51
What is primary lateral sclerosis?
Affects corticospinal tract in arms, legs and face | Progressive tetra paresis
52
What symptom is characteristic of MND?
Fasciculation | Weakness in arms and legs
53
What's the differential diagnosis for MND?
Cervical spine lesion
54
What is the management of MND?
Riluzole- Na channel blocker that inhibits glutamate release (slows disease progression) Ventilator support PEG feeding
55
What is spinal muscular atrophy?
Group of rare disorders Anterior horn cells destroyed Symmetrical wasting and weakness of limbs
56
What percentage of over-65s and over-80s are affected by dementia?
10% of over-65s | 20% of over-80s
57
What are the most common dementias?
Alzheimer's (65% of all dementias) Fronto-temporal dementia Vascular dementia Dementia with Lewy bodies
58
What is the disease progression and symptoms of Alzheimer's?
``` Progresses over years Short term memory loss Disintegration of personality and intellect Difficulty with language Apraxia Agnosia Visuospatial skills ```
59
What is the pathophysiology of Alzheimer's?
Neurofibrillary tangles | Amyloid plaques
60
Which investigations are done in dementia?
``` MMSE FBC LFTs TFTs Measure B12 And folate Brain CT ```
61
Management of Alzheimer's?
AChE inhibitors- rivastigmine, donepezil, galantamine
62
What is the prognosis for Alzheimer's?
Average survival= 8-10 years
63
What is the progression of vascular dementia?
Stepwise deterioration | Hx of TIAs or strokes
64
What is the disease course of dementia with Lewy bodies?
Fluctuating cognition with profound variation in attention and alertness Memory loss may not occur in early stages Impaired attention and visuospatial ability Visual hallucinations Parkinsonism Depression and sleep disorders
65
What are the general causes of mononeuropathy?
Compression Entrapment Direct damage
66
What is carpal tunnel syndrome?
Entrapment neuropathy | Median nerve is compressed as it passes through carpal tunnel
67
What's the cause of carpal tunnel?
Mostly idiopathic | Associated with: hypothyroidism, diabetes, pregnancy, obesity, RA, acromegaly
68
What are the features of carpal tunnel?
Pain and parenthesiae in the hand Worse at night Hanging the hand out of the bed and shaking it out can relieve pain Tinnel's sign: tapping on carpal tunnel is painful Weakness+wasting of the bar muscles Sensory loss- palm and palmar aspects of radial 3.5 fingers
69
Treatment for carpal tunnel?
Nocturnal splints Local steroid injections Surgical decompression
70
What is mononeuritis multiplex
Multiple mononeuropathy
71
What are the causes of mononeuritis multiplex?
``` Indicates systemic disease: Diabetes Leprosy Vasculitis Sarcoidosis Anyloidosis Malignancy Neurofibromatosis HIV Guillain-Barré ```
72
What are the differential diagnoses for Parkinson's disease?
Drug-induced Parkinsonism (prochlorperazine) Parkinson-plus syndromes (progressive supra nuclear palsy and multiple system atrophy) Dementia with Lewy bodies Post-encephalitic Parkinson's Normal pressure hydrocephalus Vascular Parkinson's
73
What kind of symptoms may occur in a poly neuropathy?
Sensory- paraesthesiae, numbness 'glove and stocking' distribution, pain in extremities, unsteadiness Motor- weakness Autonomic- postural hypotension, urinary retention, erectile dysfunction, diarrhoea, ⬇️sweating, cardiac arrhythmias, impaired pupillary responses
74
Name some causes of poly neuropathy
``` Guillain-Barré Drugs e.g. Isoniazid, phenytoin Diabetes Thyroid disease Amyloidosis Vitamin deficiency : B1, B12, B6 Alcohol, lead, arsenic Chronic inflamm. demyelinating polyn. ```
75
Which investigations should be done in a patient with polyneuropathy?
``` FBC ESR Serum B12 BM U&E LFTs ```
76
What is Miller Fisher syndrome
Variant of Guillain-Barré which affects ten cranial nerves to the eye Characterised by ophthalmoplegia and ataxia
77
Which test is done to confirm diagnosis of Guillain- Barré?
Nerve conduction study
78
What is the consequence of vitamin B1 (thiamin) deficiency?
Wernicke-Korsakoff syndrome Polyneuropathy Cardiac failure
79
What causes thiamin deficiency?
Alcholism, beriberi, starvation
80
What is the consequence of vitamin B6 (pyridoxine) deficiency?
Sensory neuropathy
81
What can cause vitamin B6 deficiency?
Isoniazid therapy
82
What is the consequence of vitamin B12 deficiency?
Subacute combined degeneration of the cord | Distal sensory loss, absent ankle jerks, exaggerated knee jerks, up going plantars
83
Give an example of a hereditary sensorimotor neuropathy and explain the symptoms
Charcot-Marie-Tooth (common peroneal nerve palsy) Progressive distal limb wasting and weakness Loss of sensation and reflexes in legs
84
What is the difference in clinical | Presentation between a neuropathy and a muscle disease?
Reflexes and sensation normal in muscle disease but not neuropathy
85
What are polymyosotis and dermatomyositis?
Acquired muscle diseases Polymyositis- inflammation and necrosis of skeletal muscle fibres Dermatomyositis- when skin is involved
86
Symptoms of Polymyositis and dermatomyositis
Symmetrical progressive muscle weakness and wasting of proximal muscles of shoulder and pelvic girdle Difficulty rising from a chair and raising hands above head Dysphagia, dysphonia Dermatomyositis- purple discolouration of eyelids, erythema over knuckles, arthralgia, dysphagia
87
How is myosotis treated?
Prednisolone
88
What condition is associated with myasthenia gravis in 70% of patients under 40?
Thymic hyperplasia
89
What is the clinical presentation of myasthenia gravis?
Weakness and fatiguability of ocular, bulbar and proximal limb muscles (weakness improves after rest) Ptosis Diplopia Difficulty in talking, chewing, swallowing, breathing Ask pt to sit with arms outstretched and look for slow downward drift
90
Which antibodies are specific for myasthenia gravis?
Anti-AChR autoantibodies
91
Which investigations are done in suspected myasthenia gravis?
Nerve stimulation tests- decrease in evoked potential | CT/MRI of mediastinum to look for thymoma
92
What is the differential diagnosis for myasthenia gravis
``` Thyroid ophthalmopathy Myotonic dystrophy Brainstem cranial nerve lesions Botulism Lambert-Eaton myasthenic myopathic syndrome ```
93
Treatment for myasthenia gravis
Anticholinesterases Azathioprine Plasmapherisis and IV Ig (for myasthenic crisis) Thymectomy (for thymoma)
94
What is myasthenic crisis?
When respiratory and bulbar muscles become weak
95
What is myotonia? What are examples of some myotonic conditions?
Delayed muscle relaxation after contraction (e.g. Difficulty releasing grasp after shaking hands) Examples: dystrophia myotonica, myotonia congenita
96
How does Duchenne muscular dystrophy present and what is the disease course?
Presents with weak proximal leg muscles- clumsy walking and difficulty standing Progresses to other muscle groups- resp failure Severe disability and death in late teens
97
What are symptoms of dystrophia myotonica?
Progressive distal muscle weakness with myotonia, ptosis, facial muscle weakness and wasting
98
What are the symptoms of myotonia congenita?
Mild isolated myotonia accentuates by rest and cold
99
What are the causes of low CSF opening pressure
CSF leak | Recent lumbar puncture
100
Which invesitgations are done in syringomyelia?
MRI
101
What are the causes of high CSF opening pressure?
Meningitis Tumour Intracranial haemorrhage Benign intracranial hypertension
102
What does cloudy/purulent CSF | signify?
Meningitis
103
What does bloody CSF signify?
``` Traumatic tap (hit a blood vessel during lumbar puncture) Subarachnoid haemorrhage ```
104
What are the causes of raised CSF protein?
``` Meningitis Brain abscess Infra cerebral haemorrhage Neoplastic disease Guillain-Barré MS ```
105
CSF glucose should be .....% that of the level in the blood
40-60%
106
What are the signs of type 1 neurofibromatosis?
Cafe ah lair spots Freckling in skin-folds Nodular neurofibromas Lisch nodules
107
Which dural venous sinus is the most commonly thrombosed?
``` Sagittal sinus (47% of cases) Transverse sinus (35% of cases) ```
108
What are the signs of sagittal sinus thrombosis?
Headache, vomiting, seizures, decreased vision, papilloedema
109
What are the signs of transverse sinus thrombosis?
Hedache +/- mastoid pain, focal CNS signs, seizures, papilloedema
110
What are the symptoms of cortical vein thrombosis?
Symptoms: Thunderclap headache, seizures. Signs: encephalopathy, slowly evolving focal deficits (paresis, speech disorders, decreased cognition, decreased vision) Usually occurs with sinus thrombosis Galen thrombosis=rare cause of CVT associated with vascular malformation
111
What are the differential diagnosis for intracranial venous thrombosis?
SAH, meningitis, encephalitis, intracranial abscess, arterial infarction
112
Which investigations are done in intracranial venous thrombosis?
CT/MRI venography, LP
113
What is the treatment for intracranial venous thrombosis?
Heparin | Streptokinase
114
What are some causes of intracranial venous thrombosis?
Pregnancy, OCP, head injury, dehydration, intracranial malignancy, extracranial malignancy (hypercoagulation), recent LP, hyperthyroidism, meningitis/TB, androgens...
115
When should you suspect an extradural haemorrhage?
If, after head injury, conscious level falls after a lucid interval
116
What is the most common cause of extradural haemorrhage?
Fracture of temporal/parietal bone causing laceration of middle meningeal artery (can also be caused by tearing of dural venous sinuses)
117
What are the differential diagnoses for extradural haemorrhage?
Epilepsy, Carotid dissection, carbon monoxide poisoning
118
What are the signs and symptoms of an extradural haemorrhage?
Raised ICP, decreased GCS, headache, vomiting, confusion, seizures, ipsilateral pupil dilatation, bilateral limb weakness, deep and irregular breathing (brainstem compression)
119
What is pseudobulbar palsy caused by?
Damage to the corticobulbar tracts. | MND, MS, stroke, brainstem disorders, neurosyphilis, head injury.
120
What are the signs and symptoms of pseudobulbar palsy?
Donald Duck speech, paralysed tongue, exaggerated jaw jerk, dysphonic, dysphagic, facial muscles paralysed
121
What happens to speech in myasthenia gravis?
Soft, slurred, monotonous speech which gets worse the longer the patient talks.
122
When should subdural haematoma be suspected?
Fluctuating conscious level, insidious physical/intellectual slowing, sleepiness, headache, personality change, unsteadiness Risk factors: falls (alcoholics, epileptics), anticoagulation
123
What are the causes of subdural haematoma?
Bleeding from bridging veins between cortex and venous sinuses Note: does not have to be trauma! Intracranial HYPOtension, dural metastases
124
What is a cause of the following presentation: - bilateral facial palsy - uveitis - parotitis (swollen parotid glands) - fever
Heerfordt's syndrome (a presentation of sarcoidosis) Resolves spontaneously in a few weeks
125
Can myasthenia gravis and Lyme disease cause bilateral facial palsies?
Yep
126
What is Ramsay-Hunt syndrome?
Facial palsy associated with HZV - LMN facial palsy (forehead sparing) - Pain around ear - Vesicles in and around external auditory meatus
127
Give 3 examples of mainly motor polyneuropathies
Guillain-Barre syndrome, Lead poisoning, Charcot-Marie-Tooth syndrome
128
Give 3 examples of mainly sensory polyneuropathies
Diabetes mellitus, Renal failure, leprosy
129
What are some causes of autonomic neuropathy?
Sjogren's syndrome, HIV, SLE, leprosy, Guillain-Barre, paraneoplastic syndrome, diabetes
130
What are the signs of parasympathetic neuropathy?
erectile dysfunction, nocturnal diarrhoea, urine retention, Holmes-Adie pupil
131
What are the signs of sympathetic neuropathy?
Ejaculatory failure, postural hypotension, decreased sweating, Horner's syndrome
132
What is cervical spondylosis?
Chronic cervical disc degeneration with herniation of disc material, calcification and osteophytic outgrowths
133
What are the signs and symptoms of cervical spondylosis
``` Can cause cord compression --> progressive spastic quadraparesis with sensory loss below neck Painful neck movement Neck stiffness Crepitus (cracking) on moving neck Stabbing/dull arm and wrist pain Signs of cord compression: -Spastic leg weakness (often one leg is worse) -weakness and clumsiness in hands -heavy legs -numb hands -foot drop/poor walking -incontinence ```
134
What are the signs and symptoms of radiculopathy?
Pain (electrical sensations) in arms or fingers at level of compression Dull reflexes Sensory disturbance (numbness, tingling, reduced pain and temp sensation) LMN effects in muscles innervated by affected root UMN affected below level
135
Where is pain referred to in: a) Trapped nerve at C3-C4 b) Trapped nerve at C5 c) Trapped nerve at C6 d) Trapped nerve at C7 e) Trapped nerve at C8
a) Neck and trapezius pain b) Shoulder pain and pain in arm to below the elbow c) Pain worse on wrist flexion d) Pain down arm into 3rd finger e) Pain down arm into 4th and 5th fingers
136
What are the motor + sensory defects associated with: a) C5 root involvment b) C6 root involvement c) C7 root involvement d) C8 root involvement
a) Weak deltoid+supraspinatus, reduced supinator jerk, numb elbow b) Weak biceps+brachioradialis, reduced biceps jerk, numb thumb and index finger c) Weak triceps+finger extension, reduced triceps jerks, numb middle finger d) Weak finger flexors+small hand muscles, numb 4th and 5th fingers
137
What are differential diagnoses for cord compression?
MS, nerve root neurofibroma, SCDC, bone/cord tumour, intramedullary spinal sarcoidosis
138
What are the management options for cervical spondylosis?
Neck collar Surgical root decompression (laminectomy or laminoplasty) Transforaminal steroid injection
139
What is the difference between laminectomy and laminoplasty?
``` Laminectomy= portion of bone removed Laminoplasty= screws and plates ```
140
What are the features of myopathies/muscular dystrophies
Gradual onset of symmetrical proximal weakness (also distal in myotonic dystrophy) Specific muscle groups affected Tendon reflexes preserved No paraesthesiae/bladder problems
141
What are the symptoms and signs of fascioscapulohumeral dystrophy?
``` Weakness of face ('ironed out' expression) Cannot puff out cheeks Cannot raise arms Winging of scapula Foot drop Scoliosis Horizontal clavicals Anterior axillary folds ```
142
What are the signs and symptoms of Becker's muscular dystrophy?
Similar to Duchenne's, but later presentation
143
What is the inheritance pattern of: a) Duchenne's b) Becker's c) fascioscapulohumeral
a) X-linked recessive b) X-linked recessive c) autosomal dominant
144
What do myotonic disorders cause? What is the most common one?
``` Tonic muscle spasm (myotonia) Most common= dystrophia myotonica (autosomal dominant chloride channelopathy) Signs+symptoms: -hand/foot drop -weak sternocleidomastoids -myotonia -facial weakness -cataracts -male frontal baldness -diabetes -testis/ovary atrophy -cardiomyopathy ```
145
Give examples of acqured myopathies (late onset)
Part of systemic disease: | Cushing's, hyperthyroid, malignancy, hypocalcaemia, hypercalcaemia
146
Give an example of an inflammatory myopathy
Inclusion body myositis: - weakness in quads, finger flexors and pharyngeal muscle - intranuclear inclusions seen in histology
147
What are the complications of type 1 neurofibromatosis? How common are they?
Occur in 30% of cases Mild learning disability Local effects of neurofibromas (nerve root compression, GI bleeds and obstruction, bone lesions, ⬆️BP, sarcoidosis) Malignancy (in 5% of pts)- optic glioma, sarcomatous change in neurofibroma
148
What is the management of type 1 neurofibromatosis
Yearly management of BP | Can excise neurofibromas
149
What is the prevalence of type 1 and type 2 neurofibromatosis? How are they inherited?
Type 1: 1 in 2500 Type 2: 1 in 35000 Both autosomal dominant
150
What are the characteristic signs of type 2 neurofibromatosis?
Bilateral vestibular Schwannoma (acoustic neuroma) Schwannonatosis (multiple tender Schwannomas without the bilateral vestibular Schwannomas) Juvenile posterior subcapsular lenticular opacity (type of cataract)
152
What are the differential diagnosis for NF1?
McCune-Albright syndrome Multiple lentigenes Urticaria pignentosa
153
What is the differential diagnosis for NF2?
NF1 | Schwannomatosis
154
What neurological problems can HIV cause?
``` Meningitis (fungal/TB) Toxoplasmosis Dementia Encephalomyelitis Cord problems Mononeuritis multiplex Guillain-Barré syndrome ```
155
What is HTLV-1 myelopathy/tropical spastic paraplegia?
Slowly progressing spastic paraplegia Paraesthesiae Sensory loss Disorders of micturation
156
What is the treatment of Parkinson's disease?
L-dopa (dopamine precursor which can cross the BBB) Entacapone (dopamine decarboxylase inhibitor- reduced SEs by stopping peripheral conversion of L-dopa) Selegiline (MAO-B inhibitors- increases effect of L-dopa) Bromocriptine, ropinirole cabergoline (dopamine agonists) Benzatropine (muscarinic antagonists- reduce tremor and drooling)
157
How does LEMS (Lambert-Eathon myasthenic syndrome) differ from myasthenia gravis?
MG- normal/brisk reflexes | LEMS- hyporeflexia
158
What are the symptoms of temporal lobe epilepsy?
Automatisms Abdominal rising sensation/pain beforehand Deja vu Dysphasia Impaired awareness and little memory of the event Hallucinations
159
What are the symptoms of frontal lobe epilepsy?
``` Motor features e.g. posturing Jacksonian march Behavioural disturbances Dysphasia Todd's palsy post-seizure ```
160
What are the symptoms of parietal lobe epilepsy?
``` Sensory disturbances Motor symptoms (if seizure spreads to pre-central gyrus) ```
161
What kind of epilepsy do childhood febrile convulsions predispose to?
Temporal lobe epilepsy
162
What is a common cause of temporal lobe epilepsy?
Hippocampal sclerosis
163
What are the symptoms of an anterior cerebral artery infarction?
Weak/numb contralateral arm and leg No facial involvement Bilateral infarction--> akinetic mutism
164
What are the symptoms of a middle cerebral artery infarction?
Contralateral hemiparesis Contralateral hemisensory loss Dysphasia (dominant hemisphere lesions) Apraxia (non-dominatnt hemisphere lesions)
165
What are the symptoms of a posterior cerebral artery infarction
Contralateral homonymous hemianopia
166
What are the symptoms of lacunar infarcts?
Pure motor/pure sensory symptoms Ataxic hemiparesis Dysarthria Clumsy hand
167
What are the symptoms of infarction of the vertebrobasilar circulation?
``` hemianopia cortical blindness diplopia vertigo nystagmus ataxia dysarthria dysphasia hemiplegia unilateral/bilateral sensory symptoms ```
168
What is the clinical difference between conus medullaris/cauda equina lesion and lesions higher up in the cord?
Conus medullaris/cauda equina- weakness is flaccid and arreflexic Lesions higher up in spinal cord: weakness is spastic and hyperreflexic
169
What is the most common cause of weak legs and cord compression?
Secondary malignancy (breast, lung, prostate, thyroid, kidney) in the spine
170
What are the symptoms of conus medullaris lesions?
``` Mixed UMN/LMN leg weakness Early urinary retention Constipation Back pain Sacral sensory disturbance Erectile dysfunction ```
171
What are the causes of a resting tremor? How is a resting tremor abolished?
Parkinsonism | Abolished by movement
172
What are the causes of postural tremor? When is it present?
Benign essential tremor (improves with alcohol) Thyroxicosis Anxiety Beta-agonists Tremor is present on maintained posture and may persist on movement
173
What is re-emergent tremor?
Postural tremor developing after a delay of 10 seconds (e.g. in Parkinsons) DBS may help
174
What are the causes of chorea?
Huntington's disease | Sydenham's chorea (occurs after rheumatic fever. Spontaneous remission within weeks)
175
What are the causes of hemiballismus?
Vascular lesion of subthalamic nucleus (hemiballismus occurs contralateral to lesion)
176
What is athetosis and what are the causes?
Athetosis consists of slow, confluent, purposeless movements Most common cause= cerebral palsy
177
Tics occur in which syndrome?
Tourette's syndrome
178
What are the causes of myoclonus?
``` CJD Metabolic problems (metabolic flap- liver/kidney failure, thalamic stroke (unilateral flap), hyponatraemia, hypercapnia) ```
179
What are the treatments for benign essential myoclonus?
valproate, clonazepam, piracetam
180
What is the cause of tardive dyskinesia
Chronic extposure to dopamine agonists (antipsychotics, antiemetics)
181
What is dystonia?
Prolonged muscle contractions causing abnormal posture or repetitive movements
182
What is idiopathic generalized dystonia and how is it treated?
``` Childhood onset Starts in one leg and spreads along that side of the body Progresses over 5-10 years Autosomal dominant Treatment: Trihexyphenidyl, DBS Exclude Wilson's disease ```
183
What is the treatment for focal dystonias (e.g. writer's cramp, blepharospasm)
Botox
184
What are the causes of acute dystonia? What are the signs and symptoms?
Caused by drugs: - antipsychotics - antiemetics (cyclizine) Signs and symptoms: - torticollis (head pulled back) - oculogyric/oromandibular spasm (eyes drawn up and mouth movements
185
What is the treatment for acute dystonic reactions?
anti-cholinergics
186
What is the mortality rate after the first stroke? | What is the incidence of stroke?
12% | 1/1000 per year incidence
187
What are the 4 main causes of stroke?
Small vessel occlusion or thrombosis in situ Cardiac emboli Atherothromboembolism (e.g. from carotids) CNS bleeds (SAH, carotid artery dissection)
188
Which genetic diseases predispose to stroke?
CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leucocencephalopathy) Antiphospholipid syndrome (predisposes to clots)
189
What is a watershed stroke?
A stroke that occurs in the zone between 2 cerebrovascular territories
190
What are the modifiable risk factors for stroke?
Hypertension, smoking, diabetes, heart disease (valvular, ischaemic, AF), peripheral vascular disease, past TIA, carotid bruit, OCP, increased lipids, alcohol, increased clotting, syphilis
191
What are the signs of a haemorrhagic stroke?
Meningism, coma within hours, severe headache, decreased GCS
192
What is the plan of action in the first hour after a stroke?
1. Pulse, BP, ECG (do they have AF?, Cannot thrombolyse if the blood pressure is too high). Stop HRT if patient is on it. 2. Blood glucose- aim for 4-11 mmol/L 3. Urgent CT/MRI: Diffusion weighted MRI shows acute infarct. CT shows haemorrhage 4. Thrombolysis (if symptoms begain under 4.5 hours ago) 5. Nil by mouth- hydrate by IV 6. Antiplatelet agents if haemorrhagic stroke is excluded (aspirin)
193
What are the contraindications for thrombolysis?
``` Major infarct or haemorrhage on CT Mild/non-disabling deficit Recent birth, surgery, trauma or artery/vein puncture Past CNS bleed AVM/aneurysm Severe liver disease, varices or portal hypertension Seizures Anticoagulants or INR above 1.7 Platelets below 100 BP> 220/130 ```
194
What is the differential diagnosis for stroke?
``` Head injury Transient global amnesia Epilepsy (Todd's paresis) Space-occupying lesion Migraine (N.B. migraines can cause stroke) Demyelination Hypo/hyperglycaemia Infections Electrolyte disturbance Hypertensive encephalopathy Wernicke's encephalopathy Posterior reversible encephalopathy syndrome (PRES) Cerebral reversible vasoconstrictive syndrome (CRVS) ```
195
Which drug is used for thrombolysis?
Alteplase (tissue plasminogen activator)
196
How are ADC and DWI images used to identify stroke?
ADC- dark= new stroke light= old stroke DWI- dark=old stroke light=new stroke If bright on both ADC and DWI--> think demyelination or tumour
197
What is malignant MCA syndrome?
MCA occlusion Massive oedema Midline shift
198
What are the primary preventative measures for stroke (i.e. preventing a stroke in the first place)?
Control risk factors Folate supplements Lifelong anticoagulation for AF, prosthetic valves, rheumatic heart valves
199
What are the secondary preventative measures for stroke (i.e. preventing further strokes)
Control risk factors Antiplatelet agents- clopidogrel Anticoagulation after stroke from AF- warfarin
200
Which tests and investigations should be done after a stoke?
Tests for HTN- retinopathy, nephrpathy, enlarged heart on CXR ECG- to look for AF, valvular lesions after endocarditis etc. Carotid doppler- to look for carotid artery stenosis Hypo/hyperglycaemia Hyperlipidaemia ESR- to look for vasculitis Clotting screen- to look for thrombophilia Hyperviscosity- polycythaemia, SCD Thrombocytopenia Genetic tests- CADASIL
201
AF increases chance of stroke by how much?
4.5% per year
202
What is used for prevention of stroke in patients with AF?
Warfarin or dabigatran
203
What are the cardiac causes of stroke?
Non-valvular atrial fibrillation External cardioversion Prosthetic valves Acute MI (emboli arise in 10% of patients in the next 6-12 months) Paradoxical systemic emboli Cardiac surgery Valve vegetations from infective endocarditis
204
what is the death rate in stroke?
20-25%
205
What is lateral medullary syndrome?
``` Most common brainstem vascular syndrome Caused by occlusion of posterior inferior cerebellar a. Sudden vomiting and vertigo Ipsilateral: Horner's syndrome Facial numbness Cerebellar signs Palatal paralysis Diminished gag reflex ```

NOP (3 decks)

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