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

Authors

  • Romina Moavero MD,

    1. Child Neurology Unit, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital, Headache Center, IRCCS, Rome, Italy
    2. Child Neurology and Psychiatry Unit, Tor Vergata University of Rome, Rome, Italy
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  • Laura Papetti MD,

    1. Child Neurology Unit, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital, Headache Center, IRCCS, Rome, Italy
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  • Samuela Tarantino PsyD,

    1. Child Neurology Unit, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital, Headache Center, IRCCS, Rome, Italy
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  • Barbara Battan MD,

    1. Child Neurology Unit, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital, Headache Center, IRCCS, Rome, Italy
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  • Irene Salfa MD,

    1. DPUO, Tor Vergata University of Rome - Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
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  • Annalisa Deodati MD,

    1. Diabetology Center, DPUO, Tor Vergata University of Rome – Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
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  • Riccardo Schiaffini MD,

    1. Diabetology Center, DPUO, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
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  • Federico Vigevano MD,

    1. Child Neurology Unit, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital, Headache Center, IRCCS, Rome, Italy
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  • Massimiliano Valeriani MD, PhD

    Corresponding author
    1. Child Neurology Unit, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital, Headache Center, IRCCS, Rome, Italy
    2. Diabetology Center, DPUO, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy
    3. Center for Sensory Motor Interaction Aalborg University, Aalborg, Denmark
    • Address all correspondence to M. Valeriani, Child Neurology Unit, Neuroscience and Neurorehabilitation Department, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy, Piazza S. Onofrio 4, 00165 Rome, Italy, email: valeriani@opbg.net

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  • Conflict of Interest: The authors report no conflicts of interest.

  • Funding: No funding was received for this study.

Abstract

Background

Transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) is benign and self-limited, with neurologic deficits including sensory disturbance of one body side, aphasia, nausea/vomiting, weakness, decreased vision, homonymous hemianopsia, photophobia. Acute confusional state can rarely occur. Papilledema and intracranial hypertension have also been described. It is a rare entity mainly affecting adults; however, it has been sporadically described in children and adolescents.

Main findings

In this clinical observational study, we describe a clinical series of three consecutive pediatric patients being diagnosed with HaNDL after presenting with altered consciousness, papilledema, and increased intracranial pressure. They all recovered without relapses.

Conclusion

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.

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