An unusual case of anti-basal ganglia encephalitis showing polyradiculoneuritis features
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LETTER TO THE EDITOR
An unusual case of anti-basal ganglia encephalitis showing polyradiculoneuritis features Valentina Favoni 1,2 & Michela Crisci 3 & Annamaria Borghi 3 & Anna Zaniboni 3 & Marianna Nicodemo 4 & Cristina Cappelletti 5 & Pia Bernasconi 5 & Federico Oppi 2 & Stefania Testoni 3 & Anna Gabellini 3 & Roberto Michelucci 6 Received: 30 July 2019 / Accepted: 13 April 2020 # Fondazione Società Italiana di Neurologia 2020
Dear Editor, Basal ganglia encephalitis is a rare autoimmune encephalitis associated with the demonstration of anti-basal ganglia autoantibodies (ABGA). Historical clinical descriptions include sleep disorder (somnolence, sleep inversion, or insomnia), lethargy, parkinsonism, dyskinesias, and neuropsychiatric symptoms [1]. The co-existence of polyradiculoneuritis symptoms has not been previously reported. We describe an 18-year-old patient with an unremarkable medical history admitted to our Neurology Unit due to a newonset worsening headache, fever up to 38 °C, asthenia, and acute onset of limb weakness. There was no upper respiratory tract infection before disease onset. Neurological examination disclosed slowing of mentation, and hyporeflexia associated with mild upper and lower limb weakness. On admission, the cerebrospinal fluid (CSF) analysis showed a leukocyte count of 86 mmc, with a lymphocytic predominance (88%), and protein elevation (91 mg/dL). PCR of CSF for neurotropic Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10072-020-04423-4) contains supplementary material, which is available to authorized users. * Valentina Favoni [email protected] 1
Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna, Italy
2
IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
3
Neurology Unit, Maggiore Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
4
Neurology Unit, S.Orsola-Malpighi Hospital, Bologna, Italy
5
Neurology IV – Neuroimmunology and Neuromuscular Diseases Unit, Fondazione IRCCS Istituto Neurologico Carlo Besta, Milan, Italy
6
Neurology Unit, Bellaria Hospital, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy
viruses was negative. Bacterial as well as fungal cultures of the CSF were negative. Serologic testing for West Nile virus and Epstein-Barr virus was negative. Electroencephalogram (EEG) revealed generalized slowing; brain 1.5 T MRI was normal and electromyography (EMG) examination was also normal. In the following days, the patient’s symptoms worsened with increasing lower limb weakness with areflexia, global bradykinesia, and aphasia. A repeat EMG showed slowing of F waves in the lower limbs, which was interpreted as demyelination in the proximal nerve segments. A brain MRI performed 3 weeks after the initial symptoms showed bilateral high signal lesions on T2 imaging in the basal ganglia and thalamus (Fig. 1a). A positron emission tomographic scan of the head and body found bilateral striatal hypometabolism and no
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