Case 7: Hypokinetic, metabolic neuropathy, MND Flashcards
1
Q
Hypokinetic movement disorder
A
Parkinsonism compromises the syndrome of slowness (bradykinesia) which involve rigidity and resting
2
Q
Parkinsonism disorders
A
- Parkinson’s: degenerative and slowly progressing, gets worse with time
- Multiple system atrophy
- Progressive supranuclear palsy:
- Dementia with Lewy bodies: memory problems, visual and sleeping problems early on. Get motor issues later
- Cortico basal syndrome: extremely rare. Can become mute or severe flexion of one arm
- Vascular: lots of small strokes in the basal ganglia. DAT scan will be normal. Can have frontal lobe signs as well
- Drug induced: antipsychotics (try to reduce medication or switch not always possible)
- Metabolic
3
Q
Atypical parkinsonism prognosis
A
- No disease-modifying therapies are available
- Levodopa tends to beineffective
- Life expectancy isshorterthan Parkinson’s Disease, most tend to be less than 10 years from diagnosis
- The mainstay is therefore supportive management
4
Q
Progressive supranuclear palsy
A
- Falls backwards early in condition.
- May have frontal lobe sign: Prominent cognitive issues (loss of executive function, disinhibition)
- Axial (body) rigidity
- Difficulty controlling impulses (can choke on food)
- Downward gaze palsy: Difficulty controlling eye movement (vertical- up and down), get double vision.
- Rapid, more aggressive progression. Poor response to L-dopa
5
Q
Multiple system atrophy
A
- Parkinsonism and autonomic issues (postural hypotension), cerebellar features.
- More severe condition, present with falls, lower life expectancy
- Earlysevere autonomicdysfunction (Postural hypotension may lead to faints), urinary incontinence, erectile dysfunction
- Anal Sphincter EMG is sometimes used to support diagnosis
- Camptocormia refers to abnormal flexion of the thoracolumbar spine on walking, have severe anterior flexion of neck (antecollis): may need wheelchair
- Dont have tremor, have REM sleep disorder
6
Q
MSA with predominant parkinsonism (MSA-P)
A
Following features may be prevalent:
- Babinski sign, hyperreflexia
- Stridor
- Rapidly progressive Parkinsonism
- Poor response to levodopa
- Postural instability and dysphagia within 3 years of motor onset, gait ataxia.
7
Q
MSA with predominant cerebellar signs (MSA-C)
A
- Babinski sign, hyperreflexia
- Stridor
- Parkinsonism with suggestive imaging (hypometabolism on FDG-PET in putamen)
8
Q
MSA investigations
A
- Bloods: FBC, U&E, CRP, LFT (infectious or metabolic abnormality)
- HIV, syphilis serology- infectious cause of cerebellar and cognitive symptoms
- Copper study- Wilsons
- MRI brain may show putaminal, pontine, and middle cerebellar peduncle (MCP) atrophy in MSA.
- Brain stem PET scan with [18F]fluorodeoxyglucose may show hypometabolism in MSA.
- Neuropsychiatric tests: exclude dementia
- Levodopa trial: wont improve in MSA
9
Q
Lewy body disease
A
- Dementiaprecedesthe Parkinsonian features
- Early impairement in attention and executive function
- Fluctuates in contrast to other types of dementia
- Visual hallucinations- often children/animals
- Could be considered as secondary parkinsonism rather than a Atypical Parkinsonism
- Management: Both acetylcholinesterase inhibitors (e.g. donepezil, rivastigmine) and memantine can be used as they are in Alzheimer’s. Avoid neuroepileptics
10
Q
Corticobasal degeneration
A
- Very rare
- Asymmetrical signs,one limb most affected
- Cortical sensory loss - ‘alien limb syndrome’, dyspraxia, shape recognition deficits
- Non-fluent aphasia
- Stimulus sensitive myoclonus
- Dystonia refers to co-contraction of antagonistic muscles that causes intense stiffness - can have many causes
Anti-emetic used in Parkinsonism syndromes: Domperidone
11
Q
Key features of different Parkinsonism syndrome
A
- Idiopathic Parkinson’s disease: classical features and demographic
- Familial Parkinsons disease: Early onset, strong family history
- Drug induced Parkinsonism: Dopamine antagonist drugs i.e. antipsychotics
- Vascular Parkinsonism: significant vascular disease
- Lewy body dementia: early dementia
- Progressive supranuclear palsy: Backwards falls, gaze palsy, axial rigidity
- Multiple systems atrophy: severe autonomic dysfunction
Treating Parkinson’s plus: MDT approach with Parkinson’s specialist nurse
12
Q
Drug induced Parkinsonism
A
- Antiemetics (Metoclopramide, prochloperazine)
- Typical Antipsychotics (e.g. Haloperidol)
- Atypicals (e.g. Aripiprazole)
- They are dopamine antagonists. Can also cause akathisia (the sensation of inner restlessness and inability to keep still).
- Treatment is to remove offending drugs
13
Q
Subacute combined degeneration of the spinal cord (Vitamin B12 and E deficiency)
A
- Tracts affected: lateral corticospinal tracts, dorsal column, spinocerebellar tracts
- Bilateral spastic paresis
- Bilateral loss of proprioception and vibration sensation
- Bilateral limb ataxia
14
Q
GBS (guillain Barre)
A
- Begins in lower extremities and extends bilaterally: weakness, ataxia and paraesthesia. Progresses to paralysis
- Preceded by mild respiratory or intestinal infection
- Can progress to have issues with respiration, talking, swallowing, bowel and bladder function
- Acute symmetrical diffuse sensorimotor polyneuropathy
- Treat: IV immunoglobulins and occasionally plasma exchange
15
Q
Breath count
A
- Used to test for respiratory failure in neurological issues: approximates FVC
- Take the biggest possible breath inwards and count as loud as they can on the out breath
- If <15 significantly impaired respiratory function. Each count is 100ml so 15 is 1.5L
16
Q
Chronic neuropathy
A
- Develop over months/years
- Usually axonal damage: have sensory, motor and autonomic changes
- First lost: ankle dorsiflexion, painful (excrutiating burning) or painless sensory neuropathy
- Causes: diabetes, alcohol excess, B12 deficiency, HIV, Chemotherapy, Amiodarone, Autoimmune (Lupus, sarcoidosis)
17
Q
Charcot marie tooth: chronic neuropathy
A
- A genetic sensorimotor neuropathy, often begins in childhood
- Demyelinating condition: affects extremities first
- Causes issues with proprioception, muscle power, Sensory and motor disturbances with muscle wasting in the lower legs, feet, forearms and hands. Foot drop and hammer toe (toe gets hooked under dorsum of foot).
- Genetics: chromosome 17, the PMP22 gene, autosomal dominant or de novo
- Treatment: physiotherapy and orthotics to support musculature
18
Q
Investigations for metabolic neuropathy
A
- Bloods: FBC, HBA1C, U&E, CRP/ESR, B12, TSH, HIV/hepatitis, serum electrophoresis
- MRI brain and spine: to look for central causes
- Nerve conduction studies (NCS): tells us nerve strength (amplitude) or delays (slowed conduction or conduction block). Shows if its an axonal or demyelination problem
- Electromyography (EMG): motor unit activation once the impulse reaches the muscle
19
Q
Diabetic neuropathy and bloods
A
Bloods for diabetic neuropathy: FBC, U&E, TFT, HBA1c, B12/folate
Diabetic neuropathy: Chronic diffuse sensory neuropathy. Causes damage to peripheral nerves due to accumulation of advanced glycation end products, oxidative stress and inflammatory pathways
20
Q
Progression of diabetic neuropathy
A
- Reduced sensation, pain and temperature discrimination to your feet bilaterally in a glove and stocking distribution
- Neuropathic pain
- Eventually proprioception, vibration and motor nerves may be affected. Then early signs of motor involvement with loss of ankle dorsiflexion, reduced ankle reflexes and slowed high stepping gait
