The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis mimicking an acute stroke

Eur J Neurol. 2016 Jul;23(7):1235-40. doi: 10.1111/ene.13008. Epub 2016 Apr 23.

Abstract

Background and purpose: The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) can present as sudden onset of focal neurological deficits which are clinically and radiologically indistinguishable from an ischaemic stroke. Its diagnosis requires a lumbar puncture (LP), which contraindicates intravenous thrombolytic therapy (IV-tPA).

Methods: All patients referred to our stroke centre as a stroke code resulting in a final diagnosis of HaNDL syndrome from June 2005 to June 2015 were retrospectively analysed.

Results: Nine cases were identified: seven women and two men (mean age 27.6 years, range 15-51). Clinical onset consisted of isolated aphasia (two) and aphasia with right hemiparesis/hemiparaesthesia (seven). All patients had headache in the acute setting, lasting 2-12 h. Cranial computed tomography (CT) and CT angiography (CTA) were normal in all patients. Perfusion CT was performed in seven patients, showing left hemispheric focal hypoperfusion in five cases; the remaining two were normal. Five patients were initially diagnosed as stroke and treated uneventfully with IV-tPA. Cranial magnetic resonance imaging within 48 h was normal in all cases. LP performed in all patients showed pleocytosis (range 17-351 cells/mm(3) ), high protein levels (range 0.4-1.6 g/l) and normal glucose levels. All cases recovered within 12 h and suffered a second episode within 72 h. Patients were asymptomatic between episodes and after remission.

Conclusions: The decision to thrombolyse or perform an LP in HaNDL patients mimicking a stroke is difficult in the acute setting. Perfusion CT can provide misleading results and CTA may be useful in ruling out occlusion of a cerebral vessel.

Keywords: HaNDL; IV-tPA; multimodal CT; perfusion CT; pseudomigraine with pleocytosis; stroke diagnostics; stroke mimic; thrombolysis.

MeSH terms

  • Adolescent
  • Adult
  • Aphasia / diagnosis*
  • Aphasia / diagnostic imaging
  • Brain / diagnostic imaging
  • Diagnosis, Differential
  • Diagnostic Errors
  • Female
  • Fibrinolytic Agents / therapeutic use
  • Headache / diagnosis*
  • Headache / diagnostic imaging
  • Humans
  • Lymphocytosis / diagnosis*
  • Lymphocytosis / diagnostic imaging
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Retrospective Studies
  • Stroke / diagnosis*
  • Stroke / diagnostic imaging
  • Stroke / drug therapy
  • Syndrome
  • Thrombolytic Therapy
  • Tomography, X-Ray Computed
  • Young Adult

Substances

  • Fibrinolytic Agents