Syndrome of Transient Headache and Neurologic Deficits With Cerebrospinal Fluid Lymphocitosis Should Be Considered in Children Presenting With Acute Confusional State

Abstract

Presentation during childhood and adolescence is rare; the majority of pediatric cases presented with altered consciousness, which is infrequent in HaNDL. This may suggest that in childhood this symptom might be more common than in adults. All three patients presented with increased intracranial pressure and papilledema, thus suggesting that these aspects should be investigated in all patients presenting with this clinical pattern. Finally, all our patients began to suffer from migraine. This feature, together with the benign course of the disease, could favor the hypothesis of a migrainous pathophysiology of this syndrome, although this remains a speculative.

HaNDL Syndrome: Case Report and Literature Review

“…40,41 An underlying genetic predisposition has not been rigorously studied, but patients often do not have a personal or family history of migraine. 12,15,17,20,23,25…”

“…Treatment is supportive and includes analgesics, antiemetics, and, when papilledema is present, carbonic anhydrase inhibitors. 7,23,33 Though the prognosis is excellent with a full return to neurologic baseline, several pediatric patients experienced prolonged visual impairments due to increased intracranial pressure. 7,10…”

“…2 –20 The remaining 7 fulfilled all but 1 of the criteria (infectious organism detected in 2, cerebrospinal fluid lymphocyte count less than 15 cells/µL in 1, syndrome duration longer than 3 months in 2, episode duration less than 4 hours in 2) and were classified as probable. 8,16,21 –24 At least 7 cases from 4 articles were excluded from analysis as insufficient clinical data was reported to confirm the diagnosis or more than 1 diagnostic criterion were not met. 25 –28 One additional case was excluded given the absence of cerebrospinal fluid lymphocytosis.…”

“…3,5,7,10,14,17,19 Of the 18 patients who had an opening pressure documented, 72% were elevated (28 mmHg), with papilledema noted in more than three-quarters of these (Table 1). 5,7,23,29 Cerebrospinal fluid lymphocytosis averaged 95%, with 1 outlier of 67%. 3 More than half had a cerebrospinal fluid white blood cell count greater than 100 cells/m 3 , with one-third greater than 200 cells/m 3 .…”

“…40,41 An underlying genetic predisposition has not been rigorously studied, but patients often do not have a personal or family history of migraine. 12,15,17,20,23,25 Literature review revealed a strong female predominance in pediatric cases of HaNDL syndrome. 15,33,35 Migraine incidence peaks around late adolescence to early adulthood and is greater in females than in males.…”