Eight cases of sudden-onset dropped head syndrome: patient series

J Neurosurg Case Lessons. 2021 Nov 29;2(22):CASE21177. doi: 10.3171/CASE21177.

Abstract

Background: As the proportion of elderly people continues to increase, the number of patients with dropped head syndrome (DHS) also grows. However, the relationship between onset and clinical course of DHS has hardly been studied, particularly, that of sudden-onset DHS has not been reported and remains unclear.

Observations: Sudden-onset DHS was defined as presenting with chin on chest deformity within 3 days from the time of awareness of cervical weakness. Sixty-six patients with DHS visited our facility. Among them, 8 of the total cases (12.1%) had experienced sudden onset DHS (6 females and 2 males; average age: 71.9 ± 10.9 years). Six of 8 cases showed recovery by conservative treatment, whose first interventions were from 0.1 to 12 months, but 3 experienced recurrence. Diffuse spinal kyphotic-type DHS was seen in 2 cases, and both had recurring horizontal gaze disturbance after initial recovery. Two unimproved cases underwent surgery of combined anterior and posterior cervical fixation, and their first interventions were at 5 and 24 months. After surgery, cervical sagittal alignment was improved, and they could walk maintaining horizontal gaze.

Lessons: Sudden-onset DHS can be expected to have a better outcome, but recurrence is possible in global imbalanced-type DHS.

Keywords: DHS = dropped head syndrome; INEM = isolated neck extensor myopathy; MRI = magnetic resonance imaging; STIR = short-time inversion recovery; dropped head syndrome; echography; histology; magnetic resonance imaging; sudden onset.

Publication types

  • Case Reports