Autoimmune glial fibrillary acidic protein astrocytopathy manifesting as subacute meningoencephalitis with descending myelitis: a case report

Abstract Background Glial fibrillary acidic protein (GFAP) autoimmune astrocytopathy is characterized by GFAP autoantibody positive encephalitis, meningoencephalitis or meningoencephalomyelitis. The initial clinical presentation may be similar to central nervous system infections making early diagnosis challenging. Case presentation A Chinese female patient presented with subacute meningitis with symptoms of headache, vomiting, and fever. Cerebrospinal fluid (CSF) analysis showed monocytic pleocytosis, elevated protein level, low glucose level, and negative basic microbiological studies including Xpert MTB/RIF. Brain magnetic resonance imaging (MRI) showed bilateral cerebral cortical and white matter hyperintensities on FLAIR sequences. The patient was diagnosed with possible tuberculous meningitis and started on anti-tuberculosis therapy (ATT). Three months later, the patient developed cervical myelopathy and encephalopathy with persistent CSF pleocytosis. Five months later, tissue-based and cell-based assays demonstrated GFAP antibodies in blood and CSF. Her symptoms improved with repeated administration of intravenous immunoglobulin (IVIG) and corticosteroids. One-and-a-half -year follow-up showed neither clinical progression nor relapses. Conclusions Anti-GFAP astrocytopathy should be included in the differential diagnosis of patients who present with subacute meningitis with negative microbiological studies and a progressive clinical course including encephalitis and/or myelitis.

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Autoimmune glial fibrillary acidic protein astrocytopathy presented as ataxia, myoclonus and bulbar syndrome: a case report and review of the literature

BMJ Neurology Open ◽

10.1136/bmjno-2021-000142 ◽

2021 ◽

Vol 3 (2) ◽

pp. e000142

Author(s):

Azael C Novo ◽

Begoña Venegas Pérez

Keyword(s):

Glial Fibrillary Acidic Protein ◽

Acidic Protein ◽

Review Of The Literature ◽

Atypical Case ◽

Neurological Improvement ◽

Csf Analysis ◽

Elevated Protein ◽

Progressive Ataxia ◽

Atypical Manifestations ◽

Elevated Protein Level

ObjectiveTo describe an atypical case of autoimmune glial fibrillary acidic protein (GFAP) astrocytopathy.ResultsA patient in his 60s presented with 6 months of progressive ataxia, proximal myoclonus and bulbar symptomatology. Cerebrospinal fluid (CSF) analysis showed monocytic pleocytosis, elevated protein level and elevated adenosine deaminase (ADA) level. CSF microbiological studies were negative and brain and cervical MRI showed no significant findings. We tested for nuclear, cytoplasmatic and synaptic neural autoantibodies as well as anti-GFAP antibodies. While awaiting these results, the patient was commenced on methylprednisolone boluses (1 g/day for 5 days), noting rapid neurological improvement. Eventually, CSF tests were positive for anti-GFAP antibodies.ConclusionWe report atypical manifestations of GFAP astrocytopathy. Further research is needed to fully understand the spectrum of neurological manifestations of this autoimmune disease and facilitate timely diagnosis.

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079 Glial fibrillary acidic protein (GFAP) astrocytopathy associated with cerebral micro-infarction and poor therapeutic response

Journal of Neurology Neurosurgery & Psychiatry ◽

10.1136/jnnp-2019-anzan.67 ◽

2019 ◽

Vol 90 (e7) ◽

pp. A25.2-A25 ◽

Cited By ~ 1

Author(s):

Allycia MacDonald ◽

James Triplett ◽

Srimathy Vijayan ◽

Michael Bynevelt ◽

Rahul Lakshmanan ◽

...

Keyword(s):

White Matter ◽

Glial Fibrillary Acidic Protein ◽

Early Recognition ◽

Oral Intake ◽

Brain Biopsy ◽

Acidic Protein ◽

Gait Disturbance ◽

High Dose ◽

Elevated Protein ◽

Upper Thoracic

IntroductionGlial fibrillary acidic protein (GFAP) astrocytopathy is a lesser recognised immune-mediated meningo-encephalomyelitis, which is steroid responsive in the majority of cases. Neuroimaging is unique with a distinctive symmetric white matter perivascular linear and punctate enhancement pattern. We present a case with classical phenotype but delayed clinical response, and highlight the importance of early recognition and treatment.CaseA 59-year-old Caucasian female presented with a two month history of headache, gait disturbance, insomnia, agitation, disorientation and reduced oral intake. Examination revealed a high frequency upper limb tremor, hypertonicity and pathologically brisk reflexes with impaired cognitive function. MRI brain and spinal cord demonstrated high T2 signal and striking perivascular and punctate enhancement in supratentorial white matter, cervical and upper thoracic cord. CSF examination revealed lymphocytic pleocytosis and elevated protein. Brain biopsy demonstrated reduced GFAP expression, perivascular T-lymphocytic infiltrate, and recent white matter microinfarction. CSF and serum GFAP antibodies were positive.Motor deterioration accompanied progression to a stuporous state. High dose corticosteroids were commenced, followed by intravenous immunoglobulin and mycophenolate. While there was marked improvement of perivascular contrast enhancement on imaging, the patient continued to demonstrate prominent tremor, gait disturbance and behavioural issues 9 months following symptom onset.ConclusionsThe persistence of disability in this case is likely the result of axonal loss from the initial insult, reflected by the biopsy evidence of microinfarction. Awareness of the unique pattern on MRI and the clinical phenotype will aid in early recognition and prompt treatment of this condition, thus preventing the potential long term morbidity.

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Primary Diffuse Leptomeningeal Gliomatosis

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s031716710004717x ◽

1985 ◽

Vol 12 (3) ◽

pp. 278-281 ◽

Cited By ~ 41

Author(s):

Peter Bailey ◽

Yves Robitaille

Keyword(s):

Cerebrospinal Fluid ◽

Glial Fibrillary Acidic Protein ◽

Review Of The Literature ◽

Chronic Meningitis ◽

Primary Diffuse Leptomeningeal Gliomatosis ◽

Fungal Meningitis ◽

Csf Cells ◽

Elevated Protein ◽

Cranial Nerve Palsies ◽

Low Glucose

ABSTRACT:A review of the literature on primary diffuse meningeal gliomatosis (DMG) yielded three cases and we report a fourth. DMG is a syndrome characterized by extensive basal and spinal chronic meningitis with mental confusion, headaches, diplopia, papilledema and cranial nerve palsies. The cerebrospinal fluid (CSF) has a markedly elevated protein content, moderate mononuclear pleocytosis and a normal or low glucose. This picture invariably leads to the diagnosis and treatment of tuberculous or fungal meningitis despite persistently negative cerebrospinal fluid (CSF) cultures. Reaction of exfoliated CSF cells with glial fibrillary acidic protein (GFAP) immunoperoxidase labelled antibody is suggested as a diagnostic tool. A basal meningeal biopsy appears to be the only alternative diagnostic approach.

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Magnetic Resonance Imaging Lesions in the Central Nervous System of a Dog with Canine Monocytic Ehrlichiosis

Case Reports in Veterinary Medicine ◽

10.1155/2011/379627 ◽

2011 ◽

Vol 2011 ◽

pp. 1-5

Author(s):

Linda G. Lang ◽

John F. Griffin ◽

Jonathan M. Levine ◽

Edward B. Breitschwerdt

Keyword(s):

Magnetic Resonance Imaging ◽

Central Nervous System ◽

Nervous System ◽

Magnetic Resonance ◽

Cervical Spinal Cord ◽

Resonance Imaging ◽

Csf Analysis ◽

Elevated Protein ◽

Canine Monocytic Ehrlichiosis ◽

Magnetic Resonance Imaging Mri

A ten-year-old neutered male dog was examined for tetraparesis, vestibular dysfunction, ataxia, and vertebral column hyperesthesia of 10 days duration. On magnetic resonance imaging (MRI), there were multifocal, punctate, T2-hyperintense lesions in the brain and cervical spinal cord and intracranial leptomeningeal contrast enhancement. Cerebrospinal fluid (CSF) analysis revealed a predominantly mononuclear pleocytosis and mildly elevated protein.Ehrlichia caniswas diagnosed by serum immunofluorescent antibody (IFA) testing. The dog improved with administration of doxycycline. This report describes MRI central nervous system findings in a dog with ehrlichiosis.

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Leptomeningeal carcinomatosis can be presenting manifestation of breast carcinoma

Vojnosanitetski pregled ◽

10.2298/vsp120814118m ◽

2016 ◽

Vol 73 (3) ◽

pp. 293-295 ◽

Cited By ~ 2

Author(s):

Gorana Mandic-Stojmenovic ◽

Aleksandra Pavlovic ◽

Milica Skender-Gazibara ◽

Danica Grujicic ◽

Aleksandra Radojicic ◽

...

Keyword(s):

Breast Carcinoma ◽

Clinical Status ◽

Brain Magnetic Resonance Imaging ◽

High Volume ◽

Leptomeningeal Carcinomatosis ◽

Solid Cancer ◽

Csf Analysis ◽

Negative Findings ◽

Malignant Breast ◽

Magnetic Resonance Imaging Mri

Introduction. Leptomeningeal carcinomatosis (LC) is a serious complication occuring in solid cancer patients with rather poor prognosis. Case report. We presented a 47-yearold woman with the 6-month history of diffuse headache, nausea and visual obscuration. Initially, clinical status and brain magnetic resonance imaging (MRI) indicated syndrome of idiopathic intracranial hypertension. Due to clinical progression and high papillary stasis, cerebrospinal fluid (CSF) examination was performed only after ventriculoperitoneal shunt was implanted. This led to a significant although transient clinical improvement. Futher investigations led to the diagnosis of invasive lobular breast carcinoma and repeated CSF analysis revealed malignant breast carcinoma cells. In this case LC was an initial presentation of a malignant disease. Conclusion. In the presence of a high clinical suspicion of LC, in spite of initially negative findings, a clinician should persist in repeating relevant tests, such are MRI with larger amounts of gadolinium and high-volume cytological CSF analyses in order to make the diagnosis.

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CSF β-amyloid predicts prognosis in patients with multiple sclerosis

Multiple Sclerosis Journal ◽

10.1177/1352458518791709 ◽

2018 ◽

Vol 25 (9) ◽

pp. 1223-1231 ◽

Cited By ~ 4

Author(s):

Anna M Pietroboni ◽

Michela Caprioli ◽

Tiziana Carandini ◽

Marta Scarioni ◽

Laura Ghezzi ◽

...

Keyword(s):

Multiple Sclerosis ◽

Disease Progression ◽

Characteristic Curve ◽

Brain Magnetic Resonance Imaging ◽

Predictive Biomarker ◽

Neurofilament Light Chain ◽

Neurofilament Light ◽

Csf Analysis ◽

Magnetic Resonance Imaging Mri ◽

Disease Stages

Background: The importance of predicting disease progression in multiple sclerosis (MS) has increasingly been recognized, and hence reliable biomarkers are needed. Objectives: To investigate the prognostic role of cerebrospinal fluid (CSF) amyloid beta1–42 (Aβ) levels by the determination of a cut-off value to classify patients in slow and fast progressors. To evaluate possible association with white matter (WM) and grey matter (GM) damage at early disease stages. Methods: Sixty patients were recruited and followed up for 3–5 years. Patients underwent clinical assessment, brain magnetic resonance imaging (MRI; at baseline and after 1 year), and CSF analysis to determine Aβ levels. T1-weighted volumes were calculated. T2-weighted scans were used to quantify WM lesion loads. Results: Lower CSF Aβ levels were observed in patients with a worse follow-up Expanded Disability Status Scale (EDSS; r = −0.65, p < 0.001). The multiple regression analysis confirmed CSF Aβ concentration as a predictor of patients’ EDSS increase ( r = −0.59, p < 0.0001). Generating a receiver operating characteristic curve, a cut-off value of 813 pg/mL was determined as the threshold able to identify patients with worse prognosis (95% confidence interval (CI): 0.690–0.933, p = 0.0001). No differences in CSF tau and neurofilament light chain (NfL) levels were observed ( p > 0.05). Conclusion: Low CSF Aβ levels may represent a predictive biomarker of disease progression in MS.

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