A 60-year old female presented with decreased movements for the last 2 years with rigidity and vertical large square wave jerks. The most likely diagnosis is?
Ans. d. Progressive supranuclear palsy (Ref: Harrison 19/e p2604, 2610, 18/e p3311)The most likely diagnosis in a 60-year old female presenting with decreased movements for the last 2 years with rigidity and vertical large square wave Jerks is progressive supranuclear palsy."Progressive supranuclear palsy should be considered whenever a middle aged or elderly person-person presents with history of repeated falls and has an extrapyramidal syndrome accompanied by nuchal dystonia and paralysis of voluntary down gaze."Parkinson's disease* A degenerative disorder caused by degeneration of substantia nigra (pars compacta), and idiopathic in etiology.* Clinical feature: Tremors, rigidity and bradykinesia.Lewy body dementia* Progressive cognitive decline and dementia are essential for diagnosis* Other features: fluctuating cognition, recurrent well formed detailed visual hallucinations, spontaneous features of ParkinsonismMultisystem atrophy* Characterized by:- Autonomic failure involving urinary incontinence or an orthostatic decrease of blood pressure within 3 min of standing by at least 30 mm Hg systolic or 15 mm Hg diastolic,- Poorly levodopa responsive parkinsonism- Cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia or cerebellar oculomotor dysfunction)Progressive supranuclear palsy* Marked impairment of voluntary downward gaze and horizontal gaze.Q* Extended rather than flexed dystonic posturingQ* Absence of tremorQ* Poor response to antiparkinsonian medicationQSupranuclear Gaze Palsy (Steel-Richardson syndrome)A degenerative disorder where in there occurs toss of neurons in midbrain, pons, basal ganglion and cerebellum."Progressive supranuclear palsy should he considered whenever a middle aged or elderly person-person presents with history of repeated falls and has an extrapyramidal syndrome accompanied by nuchal dystonia and paralysis of voluntary down gaze"Factors which distinguish this from Parkinson'sMarked impairment of voluntary downward gaze and horizontal gaze.QExtended rather than flexed dystonic posturingQAbsence of tremorQPoor response to antiparkinsonian medicationQ
Parkinson's disease
Lewy body dementia
Multisystem atrophy
Progressive supranuclear palsy
C.N.S.