Transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) syndrome in children: case report and narrative review

TL;DR

HaNDL syndrome is a rare, self-limiting condition in children marked by headaches, neurological symptoms, and lymphocytosis in cerebrospinal fluid.

Contribution

This paper presents a pediatric HaNDL case and reviews 44 pediatric cases, highlighting clinical features and diagnostic challenges.

Findings

59.1% of pediatric HaNDL cases were female, with sensory symptoms affecting 71.4% of patients.

CSF analysis showed elevated white blood cells and protein in most cases, with negative neuroimaging results.

Only 31.8% of patients received symptomatic treatment, and the condition typically resolves within 3 months.

Abstract

The transient Headache and Neurological Deficit with cerebrospinal fluid Lymphocytosis (HaNDL) Syndrome is a rare form of primary headache, with few cases reported in children.

We report the case of a 15-year-old female with HaNDL syndrome showing paresthesia to the right side of her face and upper limbs, asthenia, dysarthria and aphasia, followed by left periorbital pulsating headache with moderate nausea, lasting about four hours. Forty-four cases of pediatric HaNDL syndrome are reported in literature (ours included), but only 25 fulfilled all diagnostic criteria according to ICHD-3. Overall, 59.1% were females. Sensory symptoms affected 71.4% of patients, followed by impaired speech (69.0%) and motor symptoms (52.4%). At CSF analysis, the mean value of white blood cells was 201.1/µl; proteinorrhachia was reported in 31 patients (70.5%). When asked, neuroimaging was negative. Symptomatic treatment was reported only in 14 patients (31.8%).

HaNDL is a rare self-limiting syndrome affecting both adults and children. The etiology is unknown, but autoimmune mechanisms have been proposed. HaNDL is a diagnosis of exclusion: differential diagnoses include stroke, tumors, epilepsia, neuro-infective disorders, autoimmune encephalitis, vasculitis, hemiplegic migraine and migraine with aura. Usually, HaNDL episodes last less than 3 months; therapy is symptomatic. The diagnostic work out includes CSF analysis, neuroimaging and EEG. The treatment is symptomatic, and the course is self-limiting, usually resolving within 3 months.

The online version contains supplementary material available at 10.1186/s13052-025-02139-9.

Figures