Neurology at the Bedside by Daniel Kondziella & Gunhild Waldemar

Author:Daniel Kondziella & Gunhild Waldemar
Language: eng
Format: epub
Publisher: Springer International Publishing, Cham

Yo (breast, ovarian)

Hu (SCLC)

Ma2 (testis)

Amphiphysin (breast, lung)

CV2 (SCLC, thymoma)

Antibodies against glutamic acid decarboxylase (GAD), which is also an intracellular protein, are only occasionally associated with paraneoplastic limbic encephalitis, and their pathogenic properties remain unclear. Of note, anti-GAD antibodies are seen with stiff person syndrome.

AIE associated with antibodies against neuronal surface or synaptic antigens. These conditions are relatively common. They occur more often than not without malignancy, although searching for an underlying neoplastic process is always mandatory.Voltage-gated potassium channel (VGKC) complex:Leucine-rich glioma-inactivated 1 (LGI1). This is one of the two most frequent AIE. It is typically seen in middle-aged people and the elderly (>40 years). Men are twice as often affected as women. Less than 5% of cases are associated with malignancies. Characteristically, anti-LGI1 encephalitis is associated with hyponatremia and faciobrachial dystonic seizures, an epileptic syndrome preceding the onset of limbic encephalitis. Anti-LGI1 encephalitis is a classical limbic encephalitis, leading to mesial temporal lobe inflammation and related neurological symptoms, including amnesia, neuropsychiatric features, and epilepsy. Increased signal activity on MR T2-weighted and fluid-attenuated inversion recovery (FLAIR) sequences may be seen in the temporal lobes corresponding to inflammatory infiltrates (although these imaging changes can be subtle or even missing in the early and late stages of the disease), and FDG PET of the brain typically shows focal hypermetabolism in one or both temporal lobes. EEG reveals focal slowing and/or epileptic activity involving the temporal lobes. CSF is usually slightly inflamed with pleocytosis, increased protein levels and/or oligoclonal bands, and IgG index, but occasionally can be normal (Case 4.11).

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