Brain and nerve = Shinkei kenkyū no shinpo
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X-linked adrenoluekodystrophy (X-ALD) is a clinically heterogenous disorder ranging from the rapidly progressive childhood cerebral form to the milder adrenomyeloneuropathy in adults, and some persons with ALD protein (ALDP) defects may remain asymptomatic for life. Hematopoietic cell transplantation (HCT) is currently the only effective therapy for cerebral X-ALD. Stem cell sources for transplantation are bone marrow, peripheral blood and cord blood. ⋯ More than 90% of early stage patients have survived with good quality of life for a long term, whereas patients with advanced disease had the higher mortality rate during transplant procedures and the neurological abnormalities progressed in most of them. Patients with a parietal-occipital lobe pattern of demyelination demonstrated a greater mean loss of performance IQ (PIQ) points than patients with a frontal lobe pattern of demyelination. Thus, boys with early-stage disease benefit from HCT, whereas boys with advanced disease may be candidates for experimental therapies.
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Vagus nerve stimulation (VNS) and stereotactic radiosurgery (SRS) represent novel and less invasive therapeutics for medically intractable epilepsy. VNS ushered in the recent advancement in clinical application of electrical stimulation therapy for epilepsy. Chronic stimulation of the left vagus nerve with implanted generator and electrodes inhibits seizure susceptibility of the cerebral cortices. ⋯ A randomized clinical trial using the dose of 20 or 24 Gy is ongoing in the United States. SRS for intractable epilepsy associated with hypothalamic hamartoma has been advocated because of a high surgical morbidity, but further study is needed for standardization of the treatment. Substitute use of SRS for other surgical technique like callosotomy or disconnection of epileptic focus seems to be another direction worth pursuing.
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Case Reports
[A case of neuralgic amyotrophy in the lower extremity (lumbosacral radiculoplexus neuropathy)].
We report a 76-year-old man who suddenly had pain in the right buttock that radiated down the back of the leg. He underwent nerve block therapy, but subsequently noted weakness of the right leg. On examination, there was moderate weakness of the right gastrocnemius, flexor digitorum longus and extensor hallcis longus, and mild weakness of the tibialis anterior, quadriceps and hamstrings muscles. ⋯ Treatment with corticosteroids was effective for alleviating his pain, and muscle strength improved gradually. One year later, he showed no neurological deficits. Neuralgic amyotrophy in the lower extremities is rare, but awareness of this disorder helps us avoid unnecessary surgical investigations and leads to possible treatment with immunomodulative therapy.