primary atypical parkinsonisms - international parkinson and … · •parkinson’s disease (pd)...
TRANSCRIPT
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Primary Atypical ParkinsonismsPD plus syndromes
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• Parkinson’s disease (PD) is a chronic and progressive neurodegenerative disease.
• Initially thought to be rare in Africa but..
With kind permission JKA 2011
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Parkinson's disease
With kind permission MA 2010
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Basal ganglia
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What?
• Movement disorder(s)
• Ghana ? 12-15% of neurology clinic
• 3rd commonest cause of disability worldwide
• 1% over 60, 5% over 85 – now Ghana population ageing
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Case study
• 60 year old woman• Presented with poor balance and
falls• Also generalised ‘slowing down’
• O/E• MMSE – 20/30• Expressionless face, fixed stare• Impaired eye movements (up and
down gaze)• Dysarthria• Increased tone all 4 limbs and
neck/axial rigidity• Brisk refelxes
• What is the diagnosis?– Progressive supranuclear palsy
• What treatment would you recommend?– Levodopa, though response
usually minimal
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Progressive Supranuclear Palsy
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PSP
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The clinical definition of multiple system atrophy (MSA)
• is a progressive, idiopathic, degenerative process beginning in adulthood.
• Patients present with various degrees of parkinsonism, autonomic failure, cerebellardysfunction, and pyramidal signs that are poorly responsive to levodopa or dopamine agonists.
• Glial cytoplasmic inclusions (GCIs) and a neuronal multisystem degeneration are the pathologic hallmarks of this clinically variable disorder
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When is a Parkinsonian syndrome not idiopathic Parkinson's disease?
“Red flags”
• History of severe cerebral trauma, stroke, exposure to neurotoxins or anti-dopaminergic agents
• No rest tremor
• Symmetrical signs
• Early falls
• Associated ophthalmoplegia, pyramidal or cerebellar signs
• Associated autonomic dysfunction
• Rapid disease progression
• Poor response to levodopa
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“Parkinson’s plus” syndromes
1. Multiple system atrophy
• May look like PD (‘striatonigral degeneration type) –but little/no l-dopa response
• May be predominantly cerebellar syndrome (often with parkinsonism)
• May be predominantly autonomic failure (Shy-Drager type) • prominent urinary symptoms• postural hypotension
• Often more aggressive than IPD• Cognitive impairment rare• Minimal response to treatment
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MSA What sign?“Hot cross bun”
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PSP
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“Parkinson’s plus” syndromes
2. Progressive supranuclear palsy• Parkinsonian features but other key findings
– Early postural instability (often presents with falls)– Early cognitive impairment– Typical examination findings
• ‘staring eyes’• Trunkal rigidity• Impaired eye movements (esp downgaze)
• Poor prognosis and poor levodopa response
3. Cortico-basal degeneration• Parkinsonism, cognitive decline, apraxia
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CBD
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Corticobasal ganglionic degeneration(CBGD)
• is characterized by frontoparietal cortical atrophy in addition to degeneration within the extrapyramidal system.
• The disease tends to occur in those aged 60-80 years, with a mean age of onset of 63 years.
• CBGD is a rare syndrome No familial or environmental factors appear to influence CBGD.
• Progressive supranuclear palsy (PSP) and multiple system atrophy (MSA) may initially be confused with CBGD. However, the true diagnosis becomes clear as the apraxiaand dystonia develop.
• Again, the clinical and pathologic features of PSP and CBGD can overlap considerably.
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• Symptoms on long-term follow-up include focal or asymmetric rigidity, bradykinesia, postural and action tremor, and marked dystonia.
• These problems usually arise predominantly in one upper extremity.
• Limb apraxia may become a serious problem, with independent movements occasionally as severe as an alien limb.
• The incidence of limb apraxia is far higher in CBGD than in PSP, with a similar level of cognitive impairment.
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Diffuse Lewy Body Disease
• Diffuse Lewy body disease (DLBD) is a progressive neurodegenerative disorder characterized by the presence of parkinsonian symptoms and neuropsychiatric disturbances commonly accompanied by dementia.
• Progressive dementia is often the first and predominant symptom.
• Of note, longitudinal studies show that after a decade of motor symptoms, 78% of patients with Parkinson disease meet the criteria for dementia
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DLBD
• Approximately 20% of patients do not have any parkinsonian features.
• Neuropsychological deficits that have been described include aphasia, dyscalculia, and apraxia.
• A psychotic state develops in approximately 20% of patients.
• Depression, auditory and visual hallucinations, and paranoid ideation may occur.
• These patients are more likely to have cognitive adverse effects with levodopa therapy in early stages than patients with Parkinson disease.
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• Not uncommon
• Look out for red flags and non-response to Levodopa
• PD/PSP/CBD/MSA