Neurology
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Traditional side-by-side visual interpretation of ictal and interictal single-photon emission computed tomography (SPECT) scans can be difficult in identifying the surgical focus, particularly in patients with extratemporal or otherwise unlocalized intractable epilepsy. Computer-aided subtraction ictal SPECT co-registered to MRI (SISCOM) may improve the clinical usefulness of SPECT in localizing the surgical seizure focus. We studied 51 consecutive intractable partial epilepsy patients who had interictal and ictal scans. ⋯ On the other hand, seizure localization by the traditional method of SPECT inspection had no significant association with postsurgical outcome. We conclude that SISCOM improves the sensitivity and the specificity of SPECT in localizing the seizure focus for epilepsy surgery. Concordance between SISCOM localization and site of surgery is predictive of postsurgical improvement in seizure outcome.
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Review Case Reports
Rhabdomyolysis and hypoxia associated with prolonged propofol infusion in children.
Propofol, a new anesthetic, is now used more commonly to sedate patients in the intensive care unit. Propofol's rapid elimination has popularized its use to induce and maintain hypnosis in patients with refractory status epilepticus. ⋯ We report a child and an adolescent who developed severe metabolic acidosis, progressive hypoxia, and rhabdomyolysis during maintenance infusion of propofol for the treatment of refractory status epilepticus. We suggest that propofol should not be used for prolonged sedation in children until its safety can be ensured.
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Case Reports
Stiff-man syndrome in a woman with breast cancer: an uncommon central nervous system paraneoplastic syndrome.
We report a patient who developed stiff-man syndrome, including disabling shoulder subluxation and wrist ankylosis, in association with breast cancer. Immunologic investigations disclosed autoimmunity directed against not only glutamic acid decarboxylase but also amphiphysin, a 128-kd protein located in the presynaptic compartment of neurons. The patient improved after surgery and corticosteroid treatment and has been stable for nearly 4 years on only anti-estrogenics. The triad of stiff-man syndrome, breast cancer, and autoantibodies against amphiphysin identifies a new autoimmune paraneoplastic syndrome of the CNS.