A case of isolated neck extensor myopathy responding favorably to immunotherapy

Håvard Larsen1, Pauline W Bogaard, Lorenz Oppel

  • 1Departments of *Neurology; and †Pathology, Aalborg University Hospital, Aalborg, Denmark.

Insights

Isolated neck extensor myopathy caused dropped head syndrome in an elderly man. Corticosteroid therapy improved symptoms, even without signs of myositis, highlighting its diagnostic and therapeutic importance.

Area of Science:

  • Neurology
  • Pathology

Background:

  • Dropped head syndrome presents a diagnostic challenge, often necessitating the exclusion of various neurological and muscular disorders.
  • Isolated neck extensor myopathy is a rare condition that can manifest as dropped head syndrome.

Observation:

  • A 79-year-old man presented with dropped head syndrome.
  • Diagnostic workup excluded other potential causes, confirming isolated neck extensor myopathy.
  • Neck extensor muscle biopsy revealed significant fibrosis but no evidence of myositis.

Findings:

  • The patient received three cycles of corticosteroid treatment.
  • A pronounced clinical improvement in dropped head syndrome symptoms was observed following corticosteroid therapy.

Implications:

  • This case underscores the importance of considering isolated neck extensor myopathy in the differential diagnosis of dropped head syndrome.
  • Corticosteroid therapy may be beneficial for dropped head syndrome attributed to isolated neck extensor myopathy, even in the absence of myositis.

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