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.
Abstract:
We report on a case of a 79-year-old man with dropped head syndrome, where diagnostic tests, ruling out other differential diagnoses, confirmed the relatively rarely occurring condition known as isolated neck extensor myopathy. Biopsy of the neck extensor musculature was with classical pronounced fibrosis, without signs of myositis. The patient was treated with 3 cycles of corticosteroids with pronounced clinical improvement of his symptoms. This emphasizes the importance of considering isolated neck extensor myopathy as a differential diagnosis when encountered with a patient with dropped head syndrome and the importance of trying therapy with corticosteroids even when myositis is absent.
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