John Libbey Eurotext

Brainstem lesion causing paroxysmal ataxia, dysarthria, diplopia and hemifacial spasm (PADDHS) Volume 21, issue 4, August 2019

Video

  • Brainstem lesion causing paroxysmal ataxia, dysarthria, diplopia and hemifacial spasm (PADDHS)

Figures

  • Figure 1

A 67-year-old man developed paroxysmal ataxia, dysarthria (with a distorted voice that sounded like a 45-rpm record played at 33 rpm), diplopia, and hemifacial spasms (PADDHS) lasting for 15-40 seconds, ∼40 times/day. Ictal video-EEGs did not show paroxysmal discharges. Brain MRI revealed a right-midbrain lesion (figure 1A-C). Inflammatory/autoimmune/neoplastic work-up was normal. The patient took steroid and broad-spectrum antibiotic/antimycotic therapy empirically. PADDHS attacks lasted for five months and then disappeared, and brain MRI returned to normal (figure 1D-F). At 24 months of follow-up, the patient was still asymptomatic. The nature of the lesion was similar to that of a previously described case of Neuro-Behçet disease (Kontzialis and Guryildirim, 2017). The brainstem lesion might have caused PADDHS due to ephatic axonal activation within demyelinated crossed fibre tracts, with interruption of the cerebello-thalamo-cortical pathway. PADDHS is a new syndrome, expanding the clinical spectrum associated with paroxysmal ataxia and dysarthria (PAD) (Li et al., 2011). This non-epileptic paroxysmal event should be considered in the differential diagnosis of focal epileptic seizures (Lüders et al., 2019).

 

Supplementary data

Summary didactic slides are available on the www.epilepticdisorders.com website.

Disclosures

None of the authors have any conflict of interest to declare.