Case series of HaNDL syndrome responding to valproic acid

Abstract

Objective: We report a case series of patients diagnosed with the syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) responding to high dose Valproic acid. Background: HaNDL syndrome is an infrequent entity, presenting with neurological impairment episodes and severe headache. A treatment to prevent the attacks has not been proposed yet. Results: We describe 6 patients with a definite diagnosis of HaNDL, responding to Valproic acid 1000–1500 mg/day. Conclusion: Although HaNDL’s self-limiting nature, episodes may cause important disability and can last up to 3 months. Valproic acid may be a good choice to prevent attacks due to its effect on cortical depression and fast titration.

Syndrome of Transient Headache and Neurologic Deficits with Cerebrospinal Fluid Lymphocytosis (HaNDL): HHV-7 Finding in Cerebrospinal Fluid Challenges Diagnostic Criteria

“…There are currently no treatment recommendations for HaNDL. However, symptomatic treatment has been applied previously in cases with severe symptomatology: acetazomide to reduce abnormally high intracranial pressure [ 14 ], steroidal treatment given against a possible immune-mediated reaction after an infection or other inflammatory triggers [ 1 , 15 ], and treatment with valproic acid thought to affect the possible pathophysiological mechanism of cortical spreading depression (CSD) similar to migraine [ 16 ]. Moreover, it is probable that less-known features of valproic acids, such as the capacity to diminish replication of enveloped viruses and anti-inflammatory action through the modulation of innate and adaptive immunity cells [ 17 ], could contribute to a beneficial outcome.…”