Thursday, December 24, 2009

Clinical spectrum of disease of VGKC

Voltage gated calcium channels are seen in a variety of neurologic diseases. They include

1. Autoimmune neuromyotonia (formerly Isaac's syndrome)
2. Morvan's syndrome (encephalopathy and myotonia). Augustus Morvan (1870) "la choree fibrillaire." see separate post on Morvan's in this blog
3. Encephalopathy without neuromuscular excitability--clinical syndrome consists of a) clinically indistinguishable from paraneoplastic limbic encephalitis (PLE) b) subacute cognitive impairment with behavioral changes and temporal lobe seizures c) FLAIR and T2 changes in mesial temporal lobes on MRI d) temporal lobe eeg abnormalities e) association with hyponatremia f) male predominance g) dramatic response to IVIG or steroids
4. Are occassional cases with associated cancer, especially lung and thymus carcinoma, but these are typically associated with "other" paraneoplastic markers and symptoms and are minority
5. A similar presentation and responsiveness to treatment occurs in VGKC negative patients who have anti hippocampal neuropil antibodies.

No comments: