Spinocerebellar ataxia type 28 (SCA 28) is characterized by young-adult onset, very slowly progressive gait and limb ataxia, dysarthria, nystagmus, ptosis, and ophthalmoplegia. It is caused by a heterozygous pathogenic mutation in the AFG3L2. So far, approximately 80 cases with genetically-confirmed SCA 28 have been reported in the literature. We report a patient with mild gait ataxia and dysarthria who carried a known pathogenic mutation in the AFG3L2. This is the first report of genetically-confirmed SCA 28 in Korea.
Scrub typhus is an infective acute febrile disorder caused by the intracellular parasite Orientia tsutsugamushi. Neurological manifestations of scrub typhus are meningoencephalitis, cerebellitis, transverse myelitis, papilledema, and cranial nerve palsy. However, opsoclonus-myoclonus syndrome associated with scrub typhus has been rarely reported. A 59-year-old man developed vertigo, nausea, vomiting, and imbalance following scrub typhus infection for eight days. Examination showed eschar at the axilla, decreased mentality, and opsoclonus- myoclonus syndrome. Video-oculography disclosed opsoclonus with an amplitude of 15°–20° and a frequency of 6–8 Hz. The serum antibody titers to Orientia tsutsugamushi were 1:5,120, and cerebrospinal fluid analysis revealed pleocytosis. Brain magnetic resonance imaging was normal. Neurological symptoms and signs completely improved by systemic steroid and antibiotics treatment. Various mechanisms including direct disseminating inflammation or indirect immune modulation may give rise to neurological complications following scrub typhus.
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Scrub typhus meningoencephalitis presenting as opsoclonus myoclonus syndrome: A video-based case Laxmikant Ramkumarsingh Tomar, Dhrumil Jatinbhai Shah, Utkarsh Agarwal, Atul Gogia, Anshu Rohatgi, CS Agrawal Tropical Doctor.2022; 52(1): 192. CrossRef
Neurological Manifestations of Scrub Typhus Sagar Basu, Ambar Chakravarty Current Neurology and Neuroscience Reports.2022; 22(8): 491. CrossRef
Neurological facets of scrub typhus: A comprehensive narrative review Divyani Garg, Abi Manesh Annals of Indian Academy of Neurology.2021; 24(6): 849. CrossRef
Scrub typhus is an infective acute febrile disorder caused by the intracellular parasite Orientia tsutsugamushi. Neurological manifestations of scrub typhus are meningoencephalitis, cerebellitis, transverse myelitis, papilledema, and cranial nerve palsy. However, opsoclonus-myoclonus syndrome associated with scrub typhus has been rarely reported. A 59-year-old man developed vertigo, nausea, vomiting, and imbalance following scrub typhus infection for eight days. Examination showed eschar at the axilla, decreased mentality, and opsoclonus-myoclonus syndrome. Video-oculography disclosed opsoclonus with an amplitude of 15-20° and a frequency of 6-8Hz. The serum antibody titers to Orientia tsutsugamushi were 1:5,120, and CSF analysis revealed pleocytosis. Brain MRI was normal. Neurological symptoms and signs completely improved by systemic steroid and antibiotics treatment. Various mechanisms including direct disseminating inflammation or indirect immune modulation may give rise to neurological complications following scrub typhus.