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autoimmune-glial-fibrillary-acid-protein-gfap-astrocytopathy

Strong List Colon Position

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"T2/FLAIR:"

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"DWI: normal ^6,17"

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"SWI: normal ¹⁷"

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"T1 C+ (Gd):"

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"MR angiogram: normal ^6,17"

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"T2: hyperintense longitudinally extensive spinal cord lesion(s), often pericentrally located, present in half of all patients ^{1-6,13,14,17,19}"

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"T1 C+ (Gd): may have contrast-enhancement, which is usually leptomeningeal or of the central canal ^1-6,13,14,17"

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Optional Plurals

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"T2: hyperintense longitudinally extensive spinal cord lesion(s), often pericentrally located, present in half of all patients ^{1-6,13,14,17,19}"

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Acronyms

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"The key marker is the GFAP antibody, which has a higher positive predictive value when present in the CSF than in the serum ⁵. Additionally, CSF will often have an inflammatory phenotype, with high protein and a pleocytosis ^1-6. Co-existing autoantibodies (e.g. NMDAR antibody, AQP4 antibody) may also be present, especially in patients with ovarian teratoma ^1-6,11,17."

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Headings Valid

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Expected headings

H1 Terminology
H1 Usage
H1 Epidemiology
H2 Risk factors
H2 Associations
H1 Clinical presentation
H2 Complications
H1 Diagnosis
H2 Diagnostic criteria
H2 Diagnostic clues
H1 Pathology
H2 Aetiology
H2 Location
H2 Classification
H2 Macroscopic appearance
H2 Microscopic appearance
H2 Immunophenotype
H2 Markers
H2 Genetics
H1 Radiographic features
H2 Plain radiograph
H2 Mammography
H2 Antenatal ultrasound
H2 Transoesophageal echocardiography
H2 Ultrasound
H2 CT
H3 Dual-energy CT
H2 Angiography (DSA)
H2 MRI
H2 CT/MRI
H2 Nuclear medicine
H3 PET-CT
H3 PET-MRI
H1 Radiology report
H1 Treatment and prognosis
H2 Complications
H1 History and etymology
H1 Differential diagnosis
H2 Clinical differential diagnosis
H1 Practical points
H1 See also

"Associations"

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"Brain"

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"Spinal cord"

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Commas

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"Given the rarity of the condition, epidemiological data pertaining to autoimmune GFAP astrocytopathy are not well established. Based on existing case series-level data, it tends to manifest in middle-aged adults, but can also affect paediatric patients ¹², and there are conflicting reports regarding any sex preponderance ^1,13,17,21."

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"Autoimmune GFAP astrocytopathy has a broad clinical neuropsychiatric and temporal spectrum ^1-6,8. The most common phenotypical syndromes are those of meningoencephalitis or meningoencephalomyelitis ^1-6,8,21, which can manifest over an acute, subacute, or less commonly, a chronic time-frame ^4,8. It is usually (~80%) monophasic ¹⁷."

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"Within those syndromes, a wide range of clinical features may be present, the most common of which include ^{1-6,8,17,21,22}:"

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"hyperintense, diffuse, confluent, periventricular white matter lesions (present in up to 75% of cases) ^1-6,11,17, sometimes extending into or otherwise involving the cortex, centrum semiovale, thalami (especially posteriorly), basal ganglia, and/or brainstem ^1,3,11,13,17"

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"characteristic (present in up to ~50% of cases) linear, perivascular pattern of contrast-enhancement extending radially from the periventricular surface and through the cerebral white matter, although can also be seen extending through the thalami and basal ganglia ^{1-6,11,12,18,19,21}"

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"other less characteristic, but possible, patterns of enhancement include leptomeningeal, punctate, nodular, or periependymal ^{1,2,4-6,11,13,17}"

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"T2: hyperintense longitudinally extensive spinal cord lesion(s), often pericentrally located, present in half of all patients ^{1-6,13,14,17,19}"

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"Autoimmune GFAP astrocytopathy is typically steroid-responsive ^1-6. In cases that are steroid-refractory, often in the setting of concurrent underlying malignancy or another co-existing autoantibody, there may be response to other immunosuppressive agents (e.g. rituximab, mycophenolate mofetil, azathioprine, cyclophosphamide, intravenous immunoglobulin) ⁵."

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Inline EG

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"movement disorders (e.g. tremor, chorea, myoclonus)"

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"bulbar symptoms (e.g. dysphagia)"

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"psychiatric symptoms (e.g. psychosis)"

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Oxford Comma

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"in paediatric patients, the pons, thalami and basal ganglia may be more likely to be affected ^12,16"