Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis in a 14-year-old girl probably associated with celiac disease

Pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (PMP) is a rare disease with unknown etiology predominantly diagnosed in male adults. Besides, its course is limited to about 3 months. PMP is differentiated clinically from typical migraine with aura by a longer duration of neurological deficits that usually manifest as sensory and aphasic but not as visual symptoms.

We report the case of a 14-year-old girl presenting with headache, nausea, vomiting, confusion, and left sided paresthesias. Cranial MRI revealed no pathologic results. In CSF, a lymphocytic pleocytosis and a disturbed blood-brain barrier without intrathecal IgG synthesis were detected. Two weeks after the beginning of the first event, she was admitted again with similar symptoms. After these two episodes, the girl stayed without any complaints for a follow-up of 6 months. Extensive investigations showed no signs for an infection or vasculitis but revealed high titers of anti-transglutaminase IgA antibodies. Celiac disease was confirmed by duodenal villous atrophy. The patient was started on a gluten-free diet.

In patients with migraine-like headache and transient neurological deficits with lymphocytic pleocytosis, the diagnosis of PMP should be considered if infections and defined vasculitis are excluded. Due to its good prognosis, PMP is an important diagnosis and ineffective therapies can be avoided. Association of neurological disorders with gluten sensitivity is well known, cerebellar ataxia has been most frequently described. So far, PMP associated with celiac disease was not reported. Binding of antibodies from sera of patients with celiac disease to blood-vessels structures of human brain preparations could be demonstrated. This observation could represent the link to immunological aseptic leptomeningeal vasculitis discussed as etiology for PMP.