New onset of ataxic dysarthria with CSF overdrainage syndrome: A case report

Cerebrospinal fluid (CSF) overdrainage syndrome occurs when excessive CSF drainage from the cranial cavity results from a spinal CSF leak or overdrainage through a ventriculoperitoneal (VP) or lumboperitoneal (LP) shunt. Symptoms include severe orthostatic headache, worsening with sitting or standing but improving when recumbent. The headache is typically dull, throbbing, and bilateral and may be exacerbated by Valsalva maneuver, coughing, or straining. Additional symptoms can include dizziness, nausea, vomiting, gait disturbances, diplopia, back pain, and seizures. We present a case of overdrainage syndrome following LP shunt placement for idiopathic intracranial hypertension in a 38-year-old woman with Ehlers–Danlos syndrome (EDS). One year post-surgery, she reported worsening headaches and nausea after prolonged upright positioning. Neurological examination revealed cerebellar dysfunction, including dysdiadochokinesia, intention tremor, and ataxic dysarthria. This condition gradually emerged 6 months post-LP shunt placement and was attributed to chronic overdrainage. After 1 day of observation, the patient underwent surgery to clamp the LP shunt outflow, resulting in overnight symptom resolution, including ataxic speech. This case underscores the importance of recognizing ataxic dysarthria in conjunction with low intracranial pressure syndromes, particularly in patients with EDS. It emphasizes the need to be aware of the diverse clinical manifestations of EDS and their relationship to altered CSF pressure syndromes.