A case of clippers with spinal cord presentation

Aglave, V.; Ojha, Pawan; Ansari, R.; Barvalia, P.

doi:10.1016/j.jns.2019.10.1080

Cited by 3 publications

(2 citation statements)

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“…Lesions became fewer and smaller as distance from the pons increased [ 1 ]. However, CLIPPERS-like pathology and imaging patterns were identified in the spinal cord, midbrain, cerebellum, medulla and in supratentorial regions [ 3 , 4 , 5 , 6 ]. Additionally, multiple extrapontine inflammatory changes that were not seen with conventional 3-Tesla imaging were detected with 7-Tesla magnetic resonance imaging in four CLIPPERS patients, emphasizing that subclinical pathology may occur beyond the pons [ 7 ].…”

Section: Discussionmentioning

confidence: 99%

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?

et al. 2023

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Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS) is a rare variant of the CLIPPERS spectrum with less than ten reports published so far. There is ongoing discussion regarding whether SLIPPERS is a disease entity on its own or just an acronym encompassing many underlying diagnoses, such as sarcoidosis, vasculitis and anti-glial fibrillary acidic protein (GFAP)-associated disease. A 40-year-old woman presented with episodes of language and attention impairment. Magnetic resonance imaging (MRI) revealed T2/FLAIR hyperintense lesions in the subcortical white matter associated with a micronodular, curvilinear perivascular contrast-enhancement. Alternative diagnoses were excluded. There was a remarkable response to steroids. A relapse occurred after six years, and the biopsy showed perivascular T-cell lymphocytic infiltrate, without granulomas, vasculitis, or neoplasia. There was complete resolution of the relapse after steroids. This case represents the longest reported follow-up of a patient diagnosed with SLIPPERS, and brain biopsy after 6 years did not suggest alternative diagnoses. This report contributes to the discussion regarding the possibility that exclusive supratentorial CLIPPERS-like pathology might be an isolated disease entity, but more biopsy-proven cases with a longer follow-up are needed to support this hypothesis. Recently, GFAP astrocytopathy has been characterized and might correspond to a significant number of cases previously diagnosed as CLIPPERS or SLIPPERS.

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Section: Discussionmentioning

confidence: 99%

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?

et al. 2023

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“…HIV may precede, be coincident, or complicate MG. However, the management of MG in the setting of HIV infection is uncertain as data are limited to a small case series from South Africa and case reports (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19).…”

Section: Introductionmentioning

confidence: 99%

A Comparison of Clinical, Electro-Diagnostic, Laboratory, and Treatment Outcome Differences in a Cohort of HIV-Infected and HIV-Uninfected Patients With Myasthenia Gravis

2021

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There is limited literature comparing the clinical parameters and treatment outcomes in HIV-infected and HIV-uninfected myasthenia gravis (MG) patients. The aim of the study was to investigate the clinical differences and treatment outcomes in the two categories of patients, particularly the safe use of immunosuppressive therapy in immunocompromised patients. The study was a retrospective analysis of medical records of MG patients from the neuromuscular unit at Inkosi Albert Luthuli Central Hospital in Kwa-Zulu Natal between 2003 and 2019. One hundred and seventy-eight (178) patients fulfilled the clinical criteria for MG. Twenty-four (13.4%) were HIV-infected and 154 (86.5%) were HIV-uninfected. There were 116 (65%) females, median 45 years, (IQR 40–62), 90 (50.5%) black African, 66 (37%) Indian, 20 (11.2%) white, and 2 (1.1%) of mixed ancestry. In the HIV-infected cohort, 20 (87%) had generalized MG, 12 (50%) bulbar, and 14 (60.9%) respiratory onset MG, 12 (50%) presented with MG Foundation of America (MGFA) class five diseases at diagnosis, six (25%) presented with MG crisis during the 5-year follow-up. Thirteen (54%) of the HIV-infected group required rescue therapy using (plasma exchange or IV immunoglobulin) combined with pulse cyclophosphamide compared with 17 (11%) in the HIV-uninfected cohort, respectively. At 5 years, 8 (33%) of the HIV-infected group remained refractory to treatment compared with 10 (6.5%) HIV-uninfected cohort, respectively. No adverse events were documented in HIV-infected patients receiving combination rescue therapy (PLEX or IVIG combined with IV cyclophosphamide). In conclusion HIV-infected MG patients are more likely to require combination rescue therapy with PE/IVIG and IV cyclophosphamide compared with those who were HIV-uninfected. No side effects were documented in the HIV-infected group receiving the above therapy.

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Unusual CLIPPERS presentation with a predominant spinal cord involvement: case report and review of the literature

Cruciani,

Motolese,

Tortorella

et al. 2024

Neurol Sci

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A case of clippers with spinal cord presentation

Cited by 3 publications

References 0 publications

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?

Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)—Does it Really Exist?

A Comparison of Clinical, Electro-Diagnostic, Laboratory, and Treatment Outcome Differences in a Cohort of HIV-Infected and HIV-Uninfected Patients With Myasthenia Gravis

Unusual CLIPPERS presentation with a predominant spinal cord involvement: case report and review of the literature

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