Massive vasculitic cerebellar infarction in patient with systemic lupus erythematosus

Kim, Young Seo; Park, Bek-San; Baek, Won-Ki; Kim, Seung Hyun

doi:10.4103/0028-3886.93603

Cited by 3 publications

(8 citation statements)

References 3 publications

(4 reference statements)

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“…This difference may be explained because multiple factors (T and B lymphocytes, cytokines profile, autoantibodies, etc.) are involved in SCNSV in comparison with PCNSV [37][38][39][40]. In support of these findings, the Kaplan-Meier curve after 36 months of follow-up showed a significant difference in the relapse-free survival following 10 months of therapy.…”

Section: Discussionsupporting

confidence: 72%

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

et al. 2014

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The objectives of this study are to compare the initial clinical, laboratory, and imaging features in primary central nervous system vasculitis (PCNSV) vs secondary central nervous system vasculitis (SCNSV) and follow up after treatment with intravenous cyclophosphamide (IV-CYC) plus glucocorticosteroids (GCS): methylprednisolone (MP). Neurological, laboratory, and neuroimaging findings were analyzed in PCNSV and SCNSV patients. Cerebral biopsy (CB) was performed in nine patients. Both groups received at onset MP plus IV-CYC for 6 months, followed by bimonthly IV-CYC plus prednisone (PND) for 12 months. All patients were followed during 36 months. Thirty patients were included (12 PCNSV and 18 SCNSV). Focal and non-focal neurological manifestations were similar in both groups, headache being the most frequent manifestation in both groups. Fatigue, myalgias, arthralgias, neuropathy, low leukocytes and platelets, elevated erythrocyte sedimentation rate, positive antinuclear antibodies (ANA), anti-double-stranded DNA (dsDNA), antineutrophil cytoplasmic antibodies (ANCA), low complement, and rheumatoid factor were more frequent in SCNSV (p < 0.05). In cerebrospinal fluid, pleocytosis and proteins were higher in PCNSV (p < 0.05). Periventricular and subcortical hyperintense lesions were observed in cranial magnetic resonance imaging in both vasculitides. Cerebral angiography and angioresonance showed narrowing of vasculature in all patients in both groups. CB showed gliosis and lymphocytic infiltration within and around the walls in four patients and granulomatous infiltration in the other patients. After treatment, the Kaplan-Meier survival curve showed a higher relapse-free survival in PCNSV (p < 0.05). Neurological manifestations and neuroimaging findings were similar in both groups of vasculitides, but general symptoms, joint, musculoskeletal, and peripheral neuropathy were preponderant in SCNSV. After treatment with IV-CYC and GCS, patients with PCNSV had a higher relapse-free survival than those with SCNSV.

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Section: Discussionsupporting

confidence: 72%

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

et al. 2014

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“…El LESNP puede tener una presentación focal o difusa en el SNC. Sin embargo, la afección cerebelosa es muy rara y menor al 2% [4][5][6] , pero suele ser grave; los hallazgos histológicos incluyen hemorragias perivasculares múltiples con cambios vasculíticos clásicos y conducen a un peor resultado si existe demora terapéutica, según citan algunos autores [5] .…”

Section: Discussionunclassified

“…Este supuesto se deduce de los siguientes hallazgos: la aparición de vasculitis en el LES (vasculitis secundaria); la pertenencia a la categoría inflamatoria en las vasculopatías por LES (diferenciándose de la categoría no inflamatoria o trombótica); la definición de vasculitis de vasos medianos que implica a la lesión de aquellas arterias encefálicas principales y de sus ramas (distinta a la lesión predominante de arterias intraparenquimales, capilares y vénulas, denominada vasculitis de vasos pequeños); la presentación focal de la lesión (cerebelosa izquierda); la frecuencia de afección cerebelosa-troncoencefálica por arteritis intracraneal de vasos medianos (60% de las arteritis intracraneales de vasos medianos) y la frecuente lesión específica de la ACAS y ACPI (el 30% de las arteritis intracraneales ocurren en las arterias cerebelosas) [7][8][9] . Asimismo, el mayor número de casos, según los estudios publicados, implican al sexo femenino y al grupo etario de 17 a 40 años, como los predominantes [4,6,8] ; grupos en donde se ubicó al paciente estudiado. Además, algunos sugieren que las vasculitis en el LES tienen relación con la actividad de esta, y ambos eventos están presentes en este caso; sin embargo, la ocurrencia de vasculitis en las arterias intracraneales y su posible relación con la actividad de la enfermedad es desconocida [8] .…”

Section: Discussionunclassified

“…El stroke es parte de esta entidad y, de ocurrir en el cerebelo y debido a una vasculitis, las que son muy infrecuentes, ensombrecen el pronóstico. Así, la vasculitis cerebelosa, que es una vasculopatía inflamatoria de las arterias cerebelosas, podría ser secundaria al LES, caracterizándose por la elevada mortalidad [1][2][3][4][5][6]8] . El manejo médico es crucial en las vasculitis del SNC por LES; sin embargo, se desconoce el beneficio del manejo quirúrgico en estos casos.…”

Section: Introductionunclassified

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Vasculitis cerebelosa por lupus eritematoso sistémico y descompresión suboccipital: reporte de caso

Castro-Castillo,

González-Quinteros

2024

Acta Med Peru

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Las vasculitis de las arterias o vasculitis cerebelosas en el lupus eritematoso sistémico (LES) son extremadamente infrecuentes y podrían estar inmersas o formar parte de los casos de LES neuropsiquiátrico (LESNP), stroke cerebeloso en el LES y grupo de vasculitis del sistema nervioso central (SNC ) por LES. La adición del manejo quirúrgico a esta entidad, mediante descompresión suboccipital, ha sido reportada en muy pocas publicaciones. Se presenta el caso de una paciente mujer de 18 años con LES, LESNP, stroke cerebeloso, con alta sospecha de vasculitis inflamatoria de las arterias cerebelosas a quien, a pesar del manejo médico, se le brindó descompresión suboccipital. La vasculitis inflamatoria de las arterias cerebelosas en el LES es una causa infrecuente de stroke cerebeloso de elevada mortalidad. Existe limitada evidencia científica en la realización de la descompresión suboccipital de esta entidad.

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“…[ 5 ] In rare instances, a biopsy of cerebellum revealed dense microglial infiltration and perivascular neutrophilic invasion. [ 6 ] As specific markers of cerebellar affection in SLE have not been validated, a response to therapy can be accepted as a surrogate marker of immunopathogenesis. However, therapy with mycophenolate mofetil in such a situation has not been reported.…”

Section: Discussionmentioning

confidence: 99%

Cerebellar ataxia in a young patient: A rare path to lupus

Ghosh

Chatterjee

Ghosh

et al. 2014

Journal of Neurosciences in Rural Practice

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Cerebellar ataxia is a rare manifestation of neuropsychiatric systemic lupus erythematosus (SLE). Development of vasculitic infarcts in the cerebellum is the most plausible reason of this manifestation. We report the case of a patient who presented with characteristic skin rashes of lupus along with cerebellar signs. Imaging of brain in this patient revealed prominent cerebellar atrophy. She was treated with mycophenolate mofetil and oral corticosteroid, and there was no further progression of her neurological signs after the initiation of therapy. In the clinical context of varied presentations of neurolupus, this is one of the rare sightings and our treatment protocol holds promise as first-line therapy in future.

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Massive vasculitic cerebellar infarction in patient with systemic lupus erythematosus

Cited by 3 publications

References 3 publications

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

Primary and secondary central nervous system vasculitis: clinical manifestations, laboratory findings, neuroimaging, and treatment analysis

Vasculitis cerebelosa por lupus eritematoso sistémico y descompresión suboccipital: reporte de caso

Cerebellar ataxia in a young patient: A rare path to lupus

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