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Medicine

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Neurology

Question
13 out of 18
 

A 76 year old male comes with a history of frequent falls and difficulty in looking downwards and laterally. The diagnosis is: (AIIMS May 2010)



A Alzheimer's disease

B Supranuclear palsy

C Amyotropic lateral sclerosis

D Oculomotor nerve palsy

Ans. B

Supranuclear palsy

C. Progressive Supranuclear Palsy – (AIPG 2006) ( Ref. Hari-18th ed., pg- 3310)

1). Also known as Steel- Richardson –Syndrome.

2). Degenerative disorder characterised by neuronal loss, gliosis and neurofibrillary tangles in part of CNS which are involved with maintenance of posture & equilibrium namely in mid brain, pons, basal ganglia and dentate nuclei of cerebellum.

3). Age b/w 45 – 75 years. Males > females

4). Vertical supranuclear gaze paresis, with difficulty in down gaze.

5). Failure of voluntary saccadic gaze in a vertical plane.

6). Later smooth pursuit movements are also affected.

7). Axial dystonia in extension esp of neck Frequent falls.

8). Limb rigidity and brady kinesia

9). Tremors unusual.

10). Pseudobulbar palsy – Facial weakness, dysarthria, dysphagia and exaggerated jaw Jerk and gag reflexes.

11). Pseudobulbar effect – Exaggerated and inappropriate emotional responses.

12). DTR – Brisk. Plantar ↑↑.

13). Cerebellar signs positive.

14). Intellectual functions are impaired.

Extra Edge: The clinical features are characteristic

"Progressive supranuclear palsy should be considered whenever a middle aged or elderly 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's

1). Marked impairment of voluntary downward gaze and horizontal gaze

2). Extended rather than flexed dystonic posturing

3. Absence of tremor

4). Poor response to antiparkinsonian medication

Neurology Flashcard List

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