Demencia autoinmune


Neuroimaging in patients with an immunotherapy-responsive cognitive disorder.
Magnetic resonance imaging: yellow arrows indicate areas of abnormality on fluid-attenuated inversion recovery (FLAIR). A, 36-year-old woman (patient 10 in Appendix 1) had fluctuating memory problems and was seropositive for glutamic acid decarboxylase-65 autoantibody. Bilateral hippocampal axial FLAIR abnormality, shown in A1, almost completely resolved after treatment with intravenous (IV) methylprednisolone (A2). B, 51-year-old woman (patient 20 in Appendix 1) had subacute fluctuating memory problems, multifocal neurologic examination findings, and evidence of autoimmunity (IgM antiphospholipid antibody). Symmetric confluent T2 signal abnormality in the white matter of both hemispheres (B1) decreased after treatment with IV methylprednisolone (B2). C, 60-year-old man (patient 41 in Appendix 1) had memory, language, and gait problems and was seropositive for both striational and glutamic acid decarboxylase-65 antibodies. Axial T1 magnetic resonance imaging with contrast demonstrated periventricular vessel enhancement (C1) and resolution after treatment (C2). D, 53-year-old woman (patient 29 in Appendix 1) had memory loss, hallucinations, and subsequent seizure; cerebrospinal fluid protein was elevated (>100 mg/dL), and she was seropositive for thyroid peroxidase antibodies and neuronal and muscle acetylcholine receptor antibodies. Axial FLAIR images show diffusely increased T2 signal in the midbrain (D1), which improved after treatment with IV methylprednisolone (D2). Multiple myeloma was diagnosed 18 months after neurologic presentation.
Positron emission tomographic imaging: Brain reconstructions (brighter color represents regions of hypometabolism) in a 58-year-old man (patient 21 in Appendix 1) who presented with personality change and memory problems and had elevated cerebrospinal fluid protein (>100 mg/dL). Hypometabolism, predominantly frontal and temporal (E1), improved after treatment with IV methylprednisolone (E2).
Single-photon emission computed tomographic brain imaging: Brain neuroimaging in a 35-year-old man (patient 22 in Appendix 1) who presented with vertigo and memory problems, had multiple coexisting autoimmune conditions, and was seropositive for muscle acetylcholine receptor and striational antibodies. Diffuse decrease in uptake in frontotemporoparietal regions (F1) was markedly improved globally after treatment with IV methylprednisolone (F2).


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