Mystery of CNS Invasion in HaNDL Syndrome: A First Case Report with Positive PCR EBV in CSF

Headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL) syndrome is a rare and ambivalent condition that mimics certain diseases like stroke, primary cerebral vasculitis, meningoencephalitis, and migraine with aura. The cause is believed to be viral invasion. However, none has demonstrated such a hypothesis. Here, we present the first case of this syndrome with a presentation of ataxia along with a clear evidence of the Epstein-Bar virus in the CSF. A 28-year-old female presented with a right-sided headache followed by a transient episode of ataxia for 2 weeks. Five days later, she developed right-sided ataxia with a severe headache that did not subside despite paracetamol intake. At the hospital, she developed right-sided hemianesthesia involving both the body and face. An emergency brain magnetic resonance imaging was done. However, it did not reveal any vascular or parenchymal lesion. She was diagnosed with stroke in the young and was admitted. Lumbar puncture was performed for further evaluation showing 200 white cells per μL, normal glucose and protein level. Polymerase chain reaction of the viral panel was done showing positive for Epstein-Barr virus. Serum workups were within normal range. The EEG revealed the presence of occasional intermittent bursts of polymorphic slow predominantly over the left cerebral hemisphere. Therefore, the diagnosis of the syndrome of transient headache and HaNDL was made. Gabapentin was given with significant improvement. In this study, we presented the first case of HaNDL with evidence of CNS invasion. We found that EBV might be a trigger factor to the development of cortical spreading depression which is responsible for the focal neurological deficit seen in the patient. This finding is significant since no other report has proven the existence of the virus in the CNS and had led to a further understanding of the pathophysiology of HaNDL.