Headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) syndrome

Last revised by Rohit Sharma on 14 Jun 2023

Headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL) syndrome is a rare condition characterized by recurrent headaches associated with focal neurological deficits.

The condition typically occurs in young adults aged 30-40 years, and has a slight male predilection 8.

The core clinical features include 8:

  • migraine-like headache which is often severe in intensity, throbbing in character, and lasting hours to short days

    • often associated with nausea, vomiting, and photophobia

  • transient neurological deficits such as hemiparesis, hemisensory disturbances, aphasia, and visual symptoms

    • these either accompany the headache or shortly precede it

CSF analysis, by definition, reveals a lymphocytic pleocytosis (>15 white cells/mL) 8. The degree of pleocytosis correlates with the severity of the symptoms 8.

The pathophysiology remains unclear although changes in the neurovascular resemble those found in migraine, which may be triggered by an infectious process 8.

MRI brain is normal in approximately 75% of cases 8. One case report of abnormalities included grey matter swelling, sulcal leptomeningeal enhancement, and hypoperfusion involving unilateral temporal and occipital regions 7. Another case report described reduced venous susceptibility artifact in the symptomatic hemisphere, the opposite to what may be seen in some patients with migraine 9.

Treatment is symptomatic and the prognosis is considered excellent 8.

Clinically can mimic various serious, including life-threatening, diseases, such as stroke or meningoencephalitis.

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