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Diagnostic test results in patients with HaNDL syndrome

Diagnostic test results in patients with HaNDL syndrome
Test Results consistent with HaNDL Results inconsistent with HaNDL
CSF
  • Lymphocytic pleocytosis (>15 cells/mm3)
  • Normal (<5 cells) or elevated with nonlymphocytic predominance
  • Normal or elevated (eg, >25 cm H2O) opening pressure
  • Normal or elevated protein (eg, >38 mg/dL)
  • Normal glucose concentration
  • Positive results on bacterial, viral, fungal cultures
  • Positive cytology
Brain MRI with contrast
  • Normal
  • Leptomeningeal enhancement (in some cases)
  • Restricted diffusion consistent with acute infarction
  • Acute hemorrhage
Brain CTV or MRV
  • Normal
  • Venous occlusion (CVT)
EEG
  • Normal
  • Diffuse or focal slowing
  • Epileptiform abnormalities
  • Seizures/status epilepticus
Laboratory testing*
  • Normal
  • Abnormal
Diagnostic testing in patients with clinical features consistent with HaNDL syndrome is performed to identify an aseptic lymphocytic CSF pleocytosis and to exclude alternative conditions that may also present with acute neurologic deficits along with headache. These include stroke, CVT, other structural brain conditions, meningitis, and seizures.

CSF: cerebrospinal fluid; CTV: computed tomographic venogram; CVT: cerebral venous thrombosis; EEG: electroencephalogram; HaNDL: headache and neurologic deficits with cerebrospinal fluid lymphocytosis; MRI: magnetic resonance imaging; MRV: magnetic resonance venogram; WBC: white blood cell.

* Typical laboratory testing in patients with suspected HaNDL includes assays for Lyme disease and syphilis for those either in endemic areas or with associated risk factors. For patients with encephalopathy, additional testing includes thyroid function tests, vitamin B12 level, and antineuronal antibodies. Refer to UpToDate topic for additional information.
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