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Related Experiment Videos

[Dropped head plus syndrome; a case report].

Kotaro Morino1, Yasusi Kita, Yukihiro Yoneda

  • 1Neurology Service, Hyogo Brain and Heart Center at Himeji.

Rinsho Shinkeigaku = Clinical Neurology
|January 20, 2004
PubMed
Summary

A patient with dropped head plus syndrome, a rare myopathy, experienced significant neck extensor weakness. This condition, characterized by progressive limb weakness, showed remarkable improvement with azathioprine treatment.

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Area of Science:

  • Neurology
  • Myology
  • Clinical Medicine

Background:

  • Dropped head plus syndrome is a non-progressive myopathy presenting with severe neck extensor weakness.
  • This case highlights a rare presentation of myopathy with distinct neurological and pathological features.

Observation:

  • A 74-year-old man presented with progressive neck extensor weakness, fatigue, and later limb weakness.
  • Neurological examination and blood tests were initially normal, but needle EMG revealed myopathic changes.
  • Muscle biopsy showed nonspecific myopathic changes and rimmed vacuoles.

Findings:

  • The patient's symptoms significantly improved with azathioprine, indicating its therapeutic potential.
  • Corticosteroids were ineffective, further supporting the diagnosis and treatment response.

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  • This case represents the first reported instance of dropped head plus syndrome in Japan.

    • Implications:

    • Azathioprine demonstrates efficacy in managing dropped head plus syndrome.
    • Early diagnosis and targeted treatment are crucial for improving patient outcomes.
    • This case expands the understanding of dropped head plus syndrome's clinical spectrum and geographical distribution.