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![MSA
• Parkinsonism, cerebellar and pyramidal signs, and
autonomic dysfunction.
• Two clinical phenotypes are
– Parkinsonism (MSA parkinsonian type [MSA-P])
– Predominant cerebellar ataxia (MSAYcerebellar type [MSA-C])](https://image.slidesharecdn.com/parkinsonplussyndrome-191029155802/85/Parkinson-plus-syndrome-34-320.jpg)
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Parkinson plus syndrome
- 1. PARKINSON PLUS SYNDROME/ ATYPICAL PARKINSONISM SYNDROME Dr Ahmad Shahir Mawardi Neurologist, Hospital Kuala Lumpur 22nd October 2019
- 3. Atypical Parkinsonism • challengingto diagnose • underrecognized due to overlap with other parkinsonisms
- 7. Red Flags forDifferentiating Atypical Parkinsonism From PD Nikolaus R. McFarland, Diagnostic Approach to Atypical Parkinsonian Syndromes, Continuum, 2016;22(4):1117–1142
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- 11. PSP • 5% to6% atypical parkinsonism • Annual incidence : 5 per 100,000 in individuals between the ages of 50 and 99 years • average age of onset : 63 to 66 years • mean survival: 5 to 8 years from diagnosis • Hallmarks of the disease: – early postural instability, unexplained falls, vertical supranuclear palsy, and progressive dementia
- 12. PSP : Features •Gait in PSP : – stiff, broad based, with knees extended and arms abducted – as clumsy like a ‘‘drunken sailor’’ or ‘‘dancing bear,’’ – includes large lateral deviations and step asymmetry. – turning: tend to pivot • The cause of falls is multifactorial: – axial rigidity, bradykinesia, loss of postural reflexes, freezing, a visual- vestibular component, and decreased insight. – ‘‘Rocket sign’’- patients who have lost insight into their postural instability and ‘‘rocket’’ out of their chair without assistance, resulting in a high risk for falling. • Retropulsion and falling into their chair are also common.
- 13. PSP : Features •A key feature of PSP includes inability to perform volitional saccades and progressive supranuclear ophthalmoparesis. – limitation of upgaze (non-specific) – Limitation of downgaze (most sensitive) – Later --> upgaze and lateral gaze palsies. – Slowed saccades and reduced optokinetic nystagmus – square-wave jerks • Complete gaze palsy and involuntary ocular fixation --> ‘‘Mona Lisa’’ stare or stone face. • Ability to overcome gaze limitation with vestibular or cervical-ocular reflex maneuvers.
- 14. PSP : Features •Blurred vision : decreased blink and tear production, drying of the cornea, and ocular irritation. • Diplopia: convergence insufficiency • photosensitivity, decreased eye blink, blepharospasm, and eye- opening apraxia, leading to eyebrow furrowing (procerus contraction), • vertical wrinkling of the forehead, referred to as procerus sign.
- 15. PSP : Features •characteristic facial appearance from the rigid bradykinesia and dystonia in facial musculature. • Bulbar features : progressive dysarthria that is often spastic or hypernasal, hypokinetic, and monotonous. • Speech: slow, stuttering, echolalia, and occasional involuntary vocalizations, apraxia of phonation The hypertonic facial muscles produce facial folds and a worried, astonished expression.
- 16. PSP : Features •Progressive dysphagia --> aspiration, pneumonia, and early death. • mood disturbance : apathy, depression, disinhibition, dysphoria, anxiety, and irritability, emotional lability • Cognitive decline and dementia
- 17. PSP : Diagnosis •progressive disorder with onset after the age of 40 with postural instability, significant falls,and supranuclear gaze palsy.
- 18. PSP : Pathology •The hallmark is abnormal deposition of tau and associated pathology • There is associated gliosis and degeneration that is marked by midbrain atrophy, loss of pigmented cells in the substantia nigra, and atrophy of the subthalamic nucleus, superior cerebellar and middle cerebellar peduncles, dentate nucleus, and frontal cortex.
- 19. PSP : Diagnostics •Clinical diagnosis • MRI brain : atrophy of the midbrain and superior cerebellar peduncles --> hummingbird signs
- 20. PSP : TherapeuticStrategies • symptomatic • multidisciplinary team approach including physical and occupational therapy, speech pathology, neuropsychology, psychiatry, social work, and palliative care. • Physical therapy is critical for gait and postural instability, fall prevention and to develop an exercise program to maintain mobility. • In later stages --> a feeding tube • Parkinsonism, a levodopa trial up to 1200 mg/d • Amantadine has been reported to have benefit for gait and dysphagia
- 21. PSP : TherapeuticStrategies • Coenzyme Q10 : slight improvement in cognitive function. • Painful dystonic posturing of the neck and limbs, blepharospasm, and eye opening apraxia : botulinum toxin. • Cognitive decline and dementia : cholinesterase inhibitors e.g rivastigmine • Mood disturbance including depression, anxiety, and irritability: antidepressants. • pseudobulbar : dextromethorphan-quinidine
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- 25. CBD • Now referredto as CBS • The classic presentation : asymmetric rigidity, dystonia, and ideomotor apraxia • may overlap with FTD, primary progressive aphasia, Alzheimer disease, posterior cortical atrophy, and PSP.
- 26. CBD • The meanonset : sixth decade • Mean survival :7 years from diagnosis.(Poor prognosis) • Typical features : – marked asymmetry, focal rigidity, alien limb phenomenon sensory loss, language deficits, frontal/cortical dementia, oculomotor dysfunction (gaze palsy, impaired convergence), bulbar impairment, postural instability, gait difficulty, hyperreflexia, and extensor plantar response. • Poor levodopa response
- 27. CBD • Ideomotor apraxia –inability to perform a skilled motor task despite having intact language, motor, and sensory function. – e.g inability to imitate gestures or mimic a certain task (eg, use a screwdriver or cut with a pair of scissors). • Alien limb phenomenon – abnormal grasping, posturing, or spontaneous levitation of an arm or leg, but can also include pursuit or avoidance of a tactile stimulus in the opposite or contralateral limb. • Dementia : late feature • Neuropsychiatric :fronto-striatalparietal predominance with deficits in attention, concentration, verbal fluency, language, praxis, and executive and visuospatial function. • Cortical findings such as aphasia, limb apraxia, and graphesthesia depend on the hemisphere predominantly affected.
- 28. CBS: phenotypes proposed newdiagnostic criteria for four CBD phenotypes
- 29. CBD: Pathology • CBDis characterized by symmetrical cerebral atrophy, which is typically present despite the asymmetric clinical presentation. • Pathologic diagnosis is characterized by widespread but topographic deposition of 4R-predominant, hyperphosphorylated tau in neurons and glia, astrocytic plaques, and corticobasal inclusions.
- 30. CBD: Diagnostics • Neuroimaging: Asymmetric frontoparietal atrophy • (FDG-PET) similarly can sometimes reveal asymmetric cortical metabolism.
- 31. CBD : TherapeuticStrategies • mainly supportive. • rehabilitative services and multidisciplinary approaches • Regular exercise • levodopa dose trial up to 800 mg/d to 1200 mg/d • myoclonus : Clonazepam or levetiracetam • rigidity and dystonic contractures : muscle relaxants and botulinum toxin can
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- 34. MSA • Parkinsonism, cerebellarand pyramidal signs, and autonomic dysfunction. • Two clinical phenotypes are – Parkinsonism (MSA parkinsonian type [MSA-P]) – Predominant cerebellar ataxia (MSAYcerebellar type [MSA-C])
- 35. MSA • Median age: 58 years • Mean survival : 6 -9 years • Disease progression is faster than in PD
- 36. *MSA-P > MSA-C(Western hemisphere)
- 38. MSA • MSA-P isoften poorly responsive to dopaminergic therapy. – limited by orthostatic symptoms – Cpx : levodopa-induced dyskinesia , early orofacial dystonia is a red flag for MSA-P.
- 39. MSA • Common featuresMSA-P&C: sleep disturbance, autonomic failure, and respiratory dysfunction, which can precede motor signs by several months to years. • These red flags may help to distinguish MSA from PD – Orthostatic hypotension is a frequent – Urogenital dysfunction tends to occur early in MSA compared to PD – Pisa syndrome (lateral bending of the trunk); camptocormia (abnormal forward flexion of the trunk) – Respiratory insufficiency. • Dementia (14% -16% )
- 40. MSA: Pathology • Oligodendroglialcytoplasmic inclusions and multisystem neurodegeneration, including putamen, substantia nigra, pons, inferior olivary nucleus, cerebellum, and intermediolateral cell column of the thoracic and sacral spinal cord. • The degree of involvement determines the predominant presentation or subtype of MSA.
- 41. MSA: Diagnostics • Diagnosisis based on clinical criteria MRI brain • "Hot cross bun sign" (Pontine atrophy and gliosis ) • bilateral T2 hypointensity in the posterolateral putamen, representing iron deposition, and slit hyperintensity in the lateral margin of the putamen.
- 42. MSA: Diagnostics • PETscans : decreased striatal and frontal metabolism. • DAT (125I-ioflupane) SPECT : asymmetric reduced striatal binding.46 • Autonomic testing : – tilt-table testing – 24-hour ambulatory blood pressure and heart rate monitoring – baroreceptor sensitivity (ie, the baroreflex mechanism or ability to regulate blood pressure by controlling heart rate, contractility, and peripheral resistance) for orthostatic hypotension. – Sweat testing, gastric emptying study (for gastroparesis), and urodyamics for urinary dysfunction. • Polysomnogram: sleep apnea, periodic limb movements, and rapid eye movement (REM) sleep behavior disorder.
- 43. MSA: Therapeutic Strategies •supportive therapy • involve allied health care and rehabilitation • Dopaminergic therapy : 30% - 60% showed initially respond • DA: no data • DBS: poor response
- 44. MSA: Therapeutic Strategies •Autonomic symptoms (orthostatic hypotension) • Conservative – oral hydration, increased salt intake, and compression stockings or abdominal binder • Pharmacologic therapy – fludrocortisone, desmopressin, Midodrine, droxidopa, Pyridostigmine • For neurogenic bladder, antispasmodics or botulinum toxin injections, Alpha-blockers for BPH, intermittent self- catheterization or placement of a suprapubic catheter.
- 45. 4. Dementia LewyBody (DLB) • early-onset,rapidly progressive dementia The clinical criteria for DLB in addition to early dementia: (1) parkinsonism that is coincident with or follows dementia onset; (2) fluctuating cognition, awareness, or alertness; and (3) recurrent visual hallucinations. • Additional features include – gait instability, falls, syncope or transient loss of consciousness, delusions/paranoia, depression, REM sleep behavior disorder, and neuroleptic sensitivity.
- 46. DLB : Pathology •Diffuse Lewy bodies are the hallmark in DLB as well as in PD dementia • Incidental Lewy bodies may indicate preclinical disease. • Spreading from brainstem to neocortex
- 47. DLB : Diagnostics •primarily clinical. Imaging studies • MRI brain : diffuse cerebral atrophy with relative preservation of the occipital and mesial temporal lobes • SPECT : occipital hypoperfusion • FDG-PET :cingulate island sign, or preservation of FDG- PET metabolism in the posterior cingulate relative to the cuneus and precuneus
- 48. DLB : Treatment •symptomatic • involves coordination of caregivers and allied health personnel. Sx Tx Parkinsonisms Levodopa (may exacerbate psychosis & hallucinations). DA: not used MAOIs, amantadine, & anticholinergics: best avoided (potential to worsen cognition and psychosis) hallucinations and psychosis quetiapine, clozapine, pimavanserin (phase 3 trials) Cognitive dysfunction cholinesterase inhibitors e.g Rivastigmine, donepezil. Memantin (mild benefit) comorbid depression, apathy, and anxiety
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