| IHS | Diagnosis | ICD-10 |
|---|---|---|
| 7.8 | Syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) | G44.82 |
| Previously used terms | Migraine with cerebrospinal pleocytosis; pseudomigraine with lymphocytic pleocytosis | |
Diagnostic criteria:
- Episodes of moderate or severe headache lasting hours before resolving fully and fulfilling criteria C and D
- Cerebrospinal fluid pleocytosis with lymphocytic predominance (>15 cells/µl) and normal neuroimaging, CSF culture and other tests for aetiology
- Episodes of headache are accompanied by or shortly follow transient neurological deficits and commence in close temporal relation to the development of CSF pleocytosis
- Episodes of headache and neurological deficits recur over <3 months
Comments:
This syndrome, first clearly delineated by Bartleson et al (1981), has also been referred to in the literature as a migrainous syndrome with cerebrospinal pleocytosis and as pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis. The clinical picture is of one to >20 discrete episodes of neurological deficits accompanied or followed by moderate to severe headache. Most of the episodes last hours. The neurological manifestations, involving either cerebral hemisphere and/or the brainstem/cerebellum, are most commonly sensory symptoms (78% of reported cases), aphasia (66%) and motor deficits (56%). Migraine-aura-like visual symptoms are relatively uncommon (18%). Some individuals report a "march" of symptoms similar to that reported in typical migraine aura. Patients are asymptomatic between episodes.
In addition to CSF lymphocytosis (10-760 cells/µl), there are elevations of CSF total protein (20-250 mg/dl) in >90% of cases and of the CSF opening pressure (100-400 mm H20] in >50% of cases. Papilloedema is occasionally present. Routine CT and MRI scans (with or without intravenous contrast) and angiography are virtually always normal. Microbiological studies have been uniformly normal. EEG and SPECT scans may show focally abnormal areas consistent with the focal neurological deficits.
The CSF pleocytosis eventually normalises on repeat sampling. Although no large systematic long-term follow-up studies have been reported, it appears that some patients with this syndrome may experience recurrence of it.
Most patients with this syndrome have no prior history of migraine. The clinician must consider other diagnoses that may share some of its clinical features, including familial hemiplegic migraine, neuroborreliosis, neurosyphilis, neurobrucellosis, mycoplasma, meningitis, granulomatous and neoplastic arachnoiditis, encephalitis and CNS vasculitis.
