Term
| DEscribe the pathogenesis of MND. |
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Definition
Degeneration of:
- UMN's at anterior horn of SC
- motor nuclei in motor cortex |
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Term
| Describe the progression and outlook of MND |
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Definition
Rapid, relentless progression
Death in 5 yrs
Increased in bulbar palsy as Inc aspiration pneumonia |
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Term
| Describe the epidemiology of MND |
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Definition
icreasing incidence with age
peak onset 60-70yrs
Males > Females |
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Term
| What are the three patterns of MND? |
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Definition
1. Amyotrophic Lateral Sclerosis
2. Progressive muscular atrophy
3. Progressive bulbar/pseudobulbar palsy |
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Term
| Describe the features of Amyotrophic Lateral Sclerosis MND. |
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Definition
- lateral corticospinal tract disease
- mix of UMN and LMN signs
-Bulbar features |
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Term
| Describe the features of progressive muscular atrophy MND |
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Definition
| - LMN signs: wasting / fasiculation / weakness |
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Term
| Describe the features of progressive bulbar/psudobulbar palsy MND. |
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Definition
| dysarthria / dysphagia and tongue fasiculation |
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Term
| Describe the features of progressive bulbar/psudobulbar palsy MND. |
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Definition
| dysarthria / dysphagia and tongue fasiculation |
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Term
| What is the "treatment" for MND? |
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Definition
SUPPORTIVE
-physio and walking aids
- speech and language therapy
-liquidised food/PEG tube (Later)
DRUGS
-Riluzole = glutamate antagonist
-IGF-1 on trial
slow progression by couple of months |
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Term
| What is mysthenia gravis? |
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Definition
| Autoimmune condition characterised by weakness and fatigueability of proximal limbs / occular muscles / bulbar muscles |
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Term
| Describe the pathogenesis of myasthenia |
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Definition
| - auto antibodies to post-synaptic Ach receptor = receptor loss |
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Term
| what are the clinical features of myasthenia? |
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Definition
Ftigability on repetetive tasks
- diplopia
- ptosis
- downward drift of outstreched arms |
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Term
| How would you investigat myasthenia? |
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Definition
1. Check acetycholine receptor antibodies
2. EMG/nerve stimulation
3. Endrophonium test (=anticholinesterase) should see improvement of weakness |
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Term
| How do you treat myasthenia gravis? |
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Definition
1. anticholinesterase: neostigimine
2. immunosuppressives |
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Term
| What the aetiological factors of MS? |
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Definition
1. Genetics: HLA A3
2. Defect in cytokine response: increaseIgG seen in CSF
3. Diet high in animal fat?
4. Increased risk if before puberty in high risk area
5. Viral infection: CSF features of measles rubella varicella infection during teens |
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Term
| Describe the pathology of MS. |
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Definition
1. Plaques of demyelination in optic nerve, SC, periventricular white matter and corticospinal and dorsal columns
Monocyte infiltration: NO release = damage |
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Term
| Is functional recovery possible in MS? If so - why? |
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Definition
| Yes: no remylination but increased Na+ channels = increased conduction |
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Term
| What occular features are seen in MS? |
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Definition
1. blurring/dec vision/central scotoma
2. Optic neuritis: sudden bluring/loss of vision
3.RAPD
4.INO
5. Utoff's phenomenon |
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Term
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Definition
Inflammation of optic nerve.
Papillitis: at nerve head
Retrobulbar neuritis: further back
Seen in MS/herpes zoster / vascultis / chloramphenicol/SLE |
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Term
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Definition
= Relative afferent pupilliary defect
Failure of swinging light test: eyes dilate when move to affected side. |
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Term
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Definition
| Worsening of optic neuritis from hot weather / fever / exercise |
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Term
| What brainstem signs are seen in MS? |
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Definition
Diplopia: II/III/IV/VI
Charcot's triad: nystagmus / intention tremor / scanning speech
Facial weakness
Ataxia
Dysarthria/Dysphagia |
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Term
| What spinal cord signs are seen in MS? |
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Definition
1. difficulty walking
2. Incontinence
3. Spastic paralysis
4. sensory disturance in both dorsal and spinothalamic tracts
5. Lhermitte's sign |
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Term
| What is Lhermitte's Sign? |
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Definition
Flex neck = elctrical shock sensation down spine
Indicates disease of dorsal column
Seen in MS and others such as cervical spondylosis/disc herniation/radiation myelopathy |
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Term
| How do you treat acute relapses of MS? |
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Definition
High dose corticosteroids - 3 days
Methylprednisolone |
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Term
| How do you prevent relapses in MS? |
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Definition
B-interferon and azathioprine = immunosupressives
Glatiramer Acetate: Myelin basic protein |
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Term
| What other treatment is required in MS? |
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Definition
Treat symptoms:
Fatigue: amantadine
Spasticity: Baclofen
Int tremor: Anti-Ach
Incontinence: Anti Ach/catheterise
Pain: simple analgesics NSAIDs
Physio/OT/home help |
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Term
| Describe the pathology of Parkinsons disease. |
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Definition
Idiopathic accelerated degeneration of dopamine recoptors in basal ganglia. (degeneratiojn of pars compacta and substantia nigra) loss of dopamine = depigmentation
Lewy bodies (eosinophilic) seen in basal ganglia |
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Term
| Why do you get a tremor in Parkinsons disease? |
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Definition
| Dopamine normally unhibits Acetylcholine. When less dopamine = more acetylcholine = tremor |
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Term
| In what situations might u get parkinsonism? |
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Definition
Head injury - boxing
MPTP - heroin
Pesticide exposure
Neuroleptic medication
lacunar infarct of basal ganglia |
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Term
| What are the clinical features of parknisons disease? |
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Definition
Tremor (resting- pill-rolling)
Rigidity (lead pipe and cog-wheel)
Bradykinesia (diff initiating movement)
Postural instability (simian stoop + festinant gait. Narrow base so falls common)
Mask like facies = facial imobility
Micrographia
Stare: decreased spontaneous blinking
monotone speech / slurring
dysphagia: drippling
constipation and urinary problems
increased sweating/greasy face
muscle pain
dementia depression |
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Term
| WHat is the difference between Parkinsons Disease and Parkinsonism? |
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Definition
PD: idiopathic neurodegenerative disorder that is responsive to L-dopa
Parkinsonism: secondary to another cause not responsive to L dopa |
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Term
| What are the SENSORY Symptoms of parkinsons disease? |
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Definition
- anosomia: no smell
- ageusia: no taste
- Pain: partic shoulder /back
- Paraesthesia |
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Term
| Where is dopamine produced and what inhibits it? |
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Definition
Produced in substantia nigra and adrenal medulla
Broken down by COMT and MAO (enzymes) |
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Term
| Describe the 3 dopaminergic pathways in the brain |
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Definition
1. Tuberofundibular System = DA released into hypophyseal vessels - inhibits prolactin
2. Nigrostriatal pathway = DA from SN to striatum. Controls movement. Direct pathway = promotes movement. Indirect pathway = inhibits movement
3. Mesocortical / Mesolimbic system: from mid brain to frontal cortex and limbic system. For memory, personality, attention. |
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Term
| What do akinetic-rigid syndromes cause? Give examples? |
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Definition
Causes parkinsonism.
Parkinson's disease
Lewy Body Dementia
Drugs: neuroleptics/haloperidol/metoclopromide
Multisystem Atrophy
Progresive Supranuclear Palsy (steele-richardson-olszewski syndrome)
Toxins: MPTP/manganese/mercury
Wilson's disease
CJD |
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Term
| In what condition is L-dopa contraindicated? |
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Definition
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Term
| What condition are COMT inhibitors contraindicated in? |
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Definition
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Term
| What 2 main trypes of drugs are used in parkinsons disease? |
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Definition
1. Drugs that increase dopamine
2. Anticholinergics |
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Term
| What does the Fasiculus Gracilis carry? What tract is it part of? |
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Definition
| Fibrs from the dorsal column - that synapse in medulla and decussate to form medial lemiscus |
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Term
| What is the pyramidal tract otherwise known as? |
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Definition
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Term
| What tract decussates in the medullary pyramids? |
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Definition
| Lateral corticospinal (pyramidal) tract |
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Term
| What tract carries neurones that respond to pain and temperature and deep touch? |
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Definition
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