Chorea and the effectiveness of steroids in a patient with the syndrome of transient headache with neurologic deficits and cerebrospinal fluid lymphocytosis: a case report

Lu Zhao,1 Ruihao Wang,1,2 Hui Fang,1 Bo Song,1 Dongyi Liang,3 Yuming Xu11Department of Neurology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, People’s Republic of China; 2Department of Neurology, University of Erlangen-Nuremberg, D-91054 Erlangen, Germany; 3Department of NICU, Zhengzhou Central Hospital, Zhengzhou, Henan, People’s Republic of ChinaBackground: The clinical manifestations of the transient headache and neurologic deficits with cerebrospinal fluid lymphocytosis (HaNDL) syndrome are variable, and involuntary movements have not been reported. The etiology and treatment of the syndrome are not entirely clarified.Case: A 25-year-old female presented with recurrent transient headache, involuntary movements (left upper extremity chorea) and paralysis. Lumbar punctures showed intracranial hypertension and cerebrospinal fluid pleocytosis. Symptoms and intracranial hypertension were relieved after administration of steroids.Discussion/conclusion: Chorea may also present in the HaNDL syndrome. Steroids might relieve the symptoms of the HaNDL syndrome, which indicates the possible treatment strategy for HaNDL and supports the infectious or postinfectious autoimmune etiology hypothesis.Keywords: syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis (HaNDL), involuntary movement, steroids, intracranial hypertension