HaNDL Syndrome: Case Report and Literature Review

Abstract

Headache and Neurologic Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) syndrome is a rare stroke mimicker characterized by moderate to severe headache temporally associated with transient neurologic deficits, typically hemiparesis, hemisensory disturbance, and/or aphasia. Cerebrospinal fluid studies reveal a lymphocytosis and elevated protein. Episodes recur over a period no longer than 3 months. Here we describe the case of a 16-year-old boy who presented with 3 episodes of selfresolving neurologic deficits, papilledema on fundoscopic examination, and leptomeningeal enhancement on magnetic resonance imaging (MRI). We additionally review the 30 previously reported pediatric cases of HaNDL syndrome, with a focus on possible etiologic and pathophysiologic mechanisms of disease. The reported case and literature review highlight the benign episodic nature of this likely underrecognized syndrome as well as the higher than expected frequency of abnormal neuroimaging findings.

Vestibular Migraine With Brainstem Auras: A Review of Pathogenesis, Clinical Varieties, Abortive and Prophylactic Treatment

“…Leptomeningeal enhancement on MRI was found. Spreading depression (CSD) triggered by a preceding or concurrent viral illness and leading to transient vasomotor changes has been suggested [71].…”

HaNDL syndrome: a reversible cerebral vasoconstriction triggered by an infection? A case report and a case-based review

“…The syndrome of transient Headache and Neurological Deficits with cerebrospinal fluid Lymphocytosis (HaNDL) is characterized by migraine-like headaches accompanied by transient neurological deficits, most frequently hemi-paraesthesia, hemiparesis, and dysphasia; unlike classical migraine with aura, visual symptoms are uncommon [ 1 ]. The clinical course is benign and self-limiting, with 1–12 attacks of several hours’ duration (usually > 4 h), [ 2 , 3 ] recurring over a one-to-three-month period. Diagnosis relies on the exclusion of alternative causes; thus, MRI is claimed as normal.…”

HaNDL syndrome after COVID-19

“… 1 In fact, the mechanism believed to cause the neurological symptoms is cortical spreading depression, similarly to what occurs in migraine aura. 9 In older individuals, the condition may present as a stroke mimic, which, when in doubt, is managed with fibrinolytic treatment. 1 Differential diagnosis of HaNDL syndrome includes a wide range of diseases; in addition to the conditions mentioned previously, it also includes CNS vasculitis, infectious or inflammatory encephalitis, neoplastic arachnoiditis, Mollaret meningitis, and posterior reversible encephalopathy.…”

“… 1 Differential diagnosis of HaNDL syndrome includes a wide range of diseases; in addition to the conditions mentioned previously, it also includes CNS vasculitis, infectious or inflammatory encephalitis, neoplastic arachnoiditis, Mollaret meningitis, and posterior reversible encephalopathy. 9 …”

“…Although diagnosis is established by exclusion, other complementary findings have been identified in recent years, including leptomeningeal contrast uptake on brain MRI, focal or diffuse slowing of EEG activity, 2 , 7 , 9 cerebral hypoperfusion predominantly in the temporal lobe, 10 , 11 and vasospasm in transcranial Doppler ultrasound studies. 7 Given its excellent prognosis without specific treatment and the monophasic course of the disease, 1 , 2 recurrence should lead us to reconsider the diagnosis of HaNDL syndrome.…”