Objective:

To report a case of paroxysmal sympathetic hyperactivity (PSH) with severe generalized dystonia and rigidity, resulting from acute hydrocephalus, with a slow course of recovery following hydrocephalus treatment.

Background:

Hydrocephalus can lead to decreased levels of consciousness, cognitive decline, headache, and gait disturbances. PSH, generalized dystonia, and rigidity are less commonly reported sequelae, particularly in cases of ventriculoperitoneal shunt (VPS) malfunction.

Design/Methods:

Case report and review of literature.

Results:

A 47-year-old woman with a history of cerebellar hamartoma, status post resection 20 years ago, and a ventriculoperitoneal (VPS) shunt in place, was admitted for subacute worsening of lethargy, ataxia, upward gaze palsy, and cognitive decline, with an initially unremarkable work-up. A few days after discharge, she developed a decreased level of consciousness, generalized dystonic posturing, generalized rigidity, and was found to have paroxysmal sympathetic hyperactivity (PSH). Imaging revealed communicating hydrocephalus due to shunt malfunction. A few days after shunt revision, she began waking up, but her PSH, generalized dystonia, and rigidity persisted. She was hospitalized for 38 days with slow improvement in her neurological exam. Extensive workup, including continuous EEG, CSF studies, brain and spine MRI, whole-body PET, heavy metals screening, infection workup (including prion disease), and autoimmune panels, were all unrevealing. A few days after discharge, she had an acute worsening of her neurological symptoms, becoming lethargic, with worsening generalized dystonia and rigidity. A head CT revealed worsening hydrocephalus. Thirty (30) cc of CSF was drained from her shunt, and the shunt was revised again, leading to an improvement in her symptoms within less than 24 hours.

Conclusions:

Generalized rigidity and dystonia can be manifestations of hydrocephalus, and the presentation may be more dramatic, with a slower recovery, in patients with prior cerebellar or brainstem insults, such as in this patient with a history of cerebellar surgery.