Journal of Movement Disorders

Case Reports

Progressive Supranuclear Gaze Palsy with Predominant Cerebellar Ataxia: A Case Series with Videos: Zheyu Xu, Tchoyoson C.C. Lim, Wing Lok Au, Louis C.S. Tan; J Mov Disord. 2017;10(2):87-91. Published online April 18, 2017; DOI: https://doi.org/10.14802/jmd.16059

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Progressive supranuclear palsy (PSP) with predominant cerebellar ataxia (PSP-C) is a rare phenotype of PSP. The clinical and radiological features of this disorder remain poorly characterized. Through a retrospective case series, we aim to characterize the clinical and radiological features of PSP-C. Four patients with PSP-C were identified: patients who presented with prominent cerebellar dysfunction that disappeared with the progression of the disease. Supranuclear gaze palsy occurred at a mean of 2.0 ± 2.3 years after the onset of ataxia. Mild cerebellar volume loss and midbrain atrophy were detected on brain imaging, which are supportive of a diagnosis of PSP. Videos are presented illustrating the co-existence of cerebellar signs and supranuclear gaze palsy and the disappearance of cerebellar signs with disease progression. Better recognition and the development of validated diagnostic criteria would aid in the antemortem recognition of this rare condition.

Citations

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Central nystagmus in progressive supranuclear palsy: A neglected clinical feature?
Maja Klarendic, Manja Hribar, Nina Bozanic Urbancic, Nina Zupancic, Milica G. Kramberger, Maja Trost, Saba Battelino, Diego Kaski, Maja Kojovic
Parkinsonism & Related Disorders.2021; 84: 15. CrossRef
“Parkinson’s disease” on the way to progressive supranuclear palsy: a review on PSP-parkinsonism
Ján Necpál, Miroslav Borsek, Bibiána Jeleňová
Neurological Sciences.2021; 42(12): 4927. CrossRef
Progressive Supranuclear Palsy with Predominant Cerebellar Ataxia
Shoichiro Ando, Masato Kanazawa, Osamu Onodera
Journal of Movement Disorders.2020; 13(1): 20. CrossRef

Progressive Encephalomyelitis with Rigidity and Myoclonus in an Intellectually Disabled Patient Mimicking Neuroleptic Malignant Syndrome: Zheyu Xu, Kalpana Prasad, Tianrong Yeo; J Mov Disord. 2017;10(2):99-101. Published online March 24, 2017; DOI: https://doi.org/10.14802/jmd.16058

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Abstract PDF

We present a case of 32-year-old male with profound mental retardation and autism spectrum disorder who had presented with seizures, rigidity and elevated creatine kinase and was initially diagnosed as neuroleptic malignant syndrome (NMS). The patient subsequently had a complicated clinical course, developing refractory status epilepticus, which lead to the eventual diagnosis of progressive encephalomyelitis with rigidity and myoclonus (PERM). We discuss the clinical similarities and differences between NMS and PERM, and highlight the need to consider alternative diagnoses when the clinical picture of NMS is atypical, particularly in this patient group where the history and clinical examination may be challenging.

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Progressive Encephalomyelitis with Rigidity and Myoclonus (PERM)-like Symptoms Associated with Anti-ganglionic Acetylcholine Receptor Antibodies
Yuki Kitazaki, Masamichi Ikawa, Toru Kishitani, Tomoko Kamisawa, Shunya Nakane, Yasunari Nakamoto, Tadanori Hamano
Internal Medicine.2021; 60(14): 2307. CrossRef
A Systematic Review and Meta-Analysis of Immunoglobulin G Abnormalities and the Therapeutic Use of Intravenous Immunoglobulins (IVIG) in Autism Spectrum Disorder
Daniel A Rossignol, Richard E Frye
Journal of Personalized Medicine.2021; 11(6): 488. CrossRef
Progressive encephalomyelitis with rigidity: A Taiwanese case and review of literature
Anna Chang, Kuan-yu Lin, Kai-Ju Chuang, Patrick Waters, Sarosh Irani, Victor Mgbachi, Hsu-Ling Yeh, Li-Ming Lien, Hou-Chang Chiu, Wei-Hung Chen
Clinical Neurology and Neurosurgery.2021; 208: 106807. CrossRef
Neuroleptic Malignant Syndrome in Children with Autism Spectrum Disorder (ASD): A Case Report and Brief Review of Recent Literature
Stefano Berloffa, Claudia Dosi, Benedetta Tascini, Beatrice Fossati, Ilaria Lupetti, Gabriele Masi
Children.2021; 8(12): 1201. CrossRef
A case report of rigidity and recurrent lower limb myoclonus: progressive encephalomyelitis rigidity and myoclonus syndrome, a chameleon
Aurélie Degeneffe, Marie Dagonnier, Alain D’hondt, Jose Antonio Elosegi
BMC Neurology.2018;[Epub] CrossRef

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