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Syndrome of Headache With Neurological Deficits and CSF Lymphocytosis: A Spreading Depression Mechanism? The Role of SPECT

The syndrome of headache with neurological deficits and cerebrospinal. fluid (CSF) lymphocytosis (HaNDL) is a benign and self-limited syndrome characterized by migrainous headaches and transient neurological deficits with inflammatory changes in the CSF. Berg and Williams’ have published diagnostic criteria based on the clinical and laboratory data in the reported cases in the literature. Its pathogenesis remains unknown. An inflammatory or infectious origin has been proposed, but there is not yet a pathophysiologic explanation for the neurological deficits,’ although it has been postulated that it could be an expression of spreading depression. However, studies of brain blood flow in this syndrome are limited. We performed a single photon emission computed tomography (SPECT) study in a patient who fulfilled the diagnostic criteria of HaNDL. A 25year-old man without a personal or family history of migraine, 2 weeks after an upper respiratory viral infection, experienced a severe left-sided hemicranial headache accompanied by three episodes of right-sided hemiparesis starting in his right foot, ascending to the right arm and perioral zone, each lasting 3 or 4 minutes. Five days later, he suffered a similar episode with left-sided hemicranial headache, right-sided hemiparesis, and dysarthria. An EEG performed the next day showed focal delta slowing in the left temporal and occipital lobes. A brain CT was normal. Four days later, he experienced five episodes of right-sided hemiparesis and numbness with the same progression as the previous episodes, this time without headache. He was admitted to hospital. Three days later, he experienced a severe frontal headache with five episodes of right-sided numbness and hemiparesis and a similar episode on the left side. A lumbar puncture showed CSF with 280 cells/mm3 (72% lymphocytes), a glucose level of 62 mg/dL (serum glucose 84 mg/dL), and a total protein of 81 mg/dL. An EEG demonstrated delta slowing affecting the left hemisphere, mainly over the temporal lobe. A cranial MRI was normal. Another lumbar puncture was performed 14 days after the last episode: the CSF revealed 90 cells/mm3 (86% lymphocytes), a glucose level of 60 mg/dL, and a total protein of 30 mg/dL. Cerebral arteriography showed no abnormalities. A SPECT scan demonstrated reduced bitemporooccipital blood flow. The demonstration of a functional disorder by SPECT, the absence of vascular pathology on arteriography, and the cortical progression of the neurological deficits, suggest that this syndrome might be produced by a spreading depression mechanism like the aura of migraine: probably triggered by a leptomeningeal inflammation. Stimulation of trigeminal nerve fibers would be the origin of headache as Moskowitz proposes in migraine.4 We found a bilateral reduction of brain blood flow, probably because the SPECT scan was obtained after the last episode, which involved both hemispheres. It would be interesting to perform a SPECT scan during the acute episode to evaluate changes of regional brain blood flow. We are currently pursuing this line of research, that could provide answers to the pathophysiologic cause of the neurological deficits in this syndrome.