1 - Pediatric Infectious Diseases Unit, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisbon
2 - Pediatric Neurology Unit, Hospital Dona Estefânia, Centro Hospitalar Universitário de Lisboa Central, Lisbon
3 - National Institute of Health Dr. Ricardo Jorge, Lisbon
- Oral presentation - Excellence in Pediatrics Conference
Introduction: Headache with neurological deficits and cerebrospinal fluid lymphocytosis (HaNDL) is an uncommon headache syndrome described as “non-infectious inflammatory intracranial disease”, presumably related to a transient immune-mediated central nervous system inflammation. Probably post infectious, etiology and pathophysiology are incompletely understood, with cerebrospinal fluid (CSF) investigations for an infectious agent almost consistently negative.
Case Report: Fifteen-year old male with a previous history of an episodic headache with clinical features of migraine was referred with a suspected diagnosis of acute confusional state. He presented with severe periorbital pain and left-sided frontal headache associated with hypoesthesia and paraesthesia in his right upper limb and difficulty to speak. The patient had a paraphasic speech consistent with motor aphasia and no problems of language comprehension. He had a questionable deviation of the tongue to the left and slight gait imbalance without any preponderant direction. He had no other abnormal findings on examination. MRI was normal and the CSF demonstrated a pleocytosis (194/μL) with predominant lymphocytes, normal glucose levels and mildly elevated protein levels (53 mg/dL). Unexpectedly, PCR was positive for Borrelia burgdorferis sensu lato (s.l.) and the sequence revealed the presence of DNA of Borrelia lusitaniae. The patient was treated with 21-day course of ceftriaxone with a full recovery from headache, aphasia and sensory symptoms within less than 24 h and remains asymptomatic with normal neurological examination.
Discussion: The isolation of Borrelia lusitaniae was concomitant with CSF lymphocytosis and clinical presentation compatible with HaNDL. Borrelia lusitaniae is prevalent in Mediterranean countries, with only few cases reported implicated human disease. But would untreated disease continue to meet HaNDL criteria? Because the favorable outcome and consequently symptomatic treatment of HaNDL, it is imperious to consider other diagnosis, especially serious and/or treatable entities.
Key words: headache, neurological deficits, cerebrospinal fluid lymphocytosis, Borrelia lusitaniae.