Peripheral neuropathies – Paediatrics MCQ

All of the following are example of peripheral neuropathies except?
A. Abetalipoproteinemia
B. Charcot-Marie-Tooth disease
C. Werdnig Hoffman disease
D. Dejerine Sottas disease

Correct answer : C. Werdnig Hoffman disease

Abetalipoproteinemia duodenal mucosal biopsyDuodenal biopsy in a case of abetalipoproteinemia. . H&E stain. Shows characteristic clear enterocytes due lipid accumulation.

  • Abetalipoproteinemia is a progressive ataxic neuropathy with retinitis pigmentosa and steatorrhoea.
  • Charcot-Marie-Tooth disease is a hereditary motor and sensory neuropathy. It is characterised by weakness of extremities and hammer toe.
  • Dejerine Sottas disease is also a hereditary motor and sensory neuropathy. It is associated with distal weakness and ataxia.
  • Werdnig Hoffman disease is spinal muscular atrophy type 1. It presents within first 6 months of life. It presents with generalised symmetrical weakness, more in proximal muscle groups with hypotonia.

Ref: Ghai Essential Pediatrics, 7th edition, pages 574-577

Image credits: User:Nephron via http://commons.wikimedia.org/wiki/File:Abetalipoproteinemia_-_high_mag.jpg

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