Kennedy Disease

Multiple Choice Questions in Neurology:

True and False statements about Kennedy Syndrome:

  1. CAG repeat
  2. Gynecomastia
  3. Testicular atrophy
  4. Peripheral neuropathy
  5. Ocular muscle involvement

Kennedy's Disease

Inheritance

  • X-linked recessive disorder
  • Trinucleotide repeat: CAG gene

Clinical Features

  • Slowly progressive limb-girdle type muscle weakness
  • Slowly progressive bulbar dysfunction
  • Early tremor
  • Muscle cramps
  • Fasciculations
  • Marked abnormal sensory nerve conduction study
  • Degeneration of both motor and sensory neurons
  • Lower motor neurons signs
  • Elevated serum creatine kinase
  • Abnormal sex hormone levels
  • Gynecomastia
  • Testicular atrophy
  • Diabetes mellitus

Note: Extraocular muscles are spared.

Kennedy Syndrome

References:

  • Fischbeck, K. H. "Kennedy disease." Journal of inherited metabolic disease 20.2 (1997): 152-158.
  • Sperfeld, Anne D., et al. "X-linked bulbospinal neuronopathy: Kennedy disease." Archives of Neurology 59.12 (2002): 1921-1926.

[Answers are A. CAG repeat-True, B. Gynecomastia-True, C. Testicular atrophy-True, D. Peripheral neuropathy-True, E. Ocular muscle involvement-False]

Kindly let me know your answers and reviews:

Which of the following is/are true about Kennedy's disease?

  1. It is also known as Foster Kennedy syndrome
  2. X linked spinobulbar muscular atrophy
  3. Lower motor neuron disorder
  4. Azoospermia
  5. Female > Male

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