[Non-convulsive epileptic status associated with Lafora disease: two case reports]. / Estado epiléptico no convulsivo asociado a enfermedad de Lafora: presentación de dos casos.

INTRODUCTION: Lafora s disease is a type of progressive myoclonic epilepsy with poor prognosis, is characterized by myoclonic crisis, tonic clonic seizures, absence or partial complex seizures and other neurological manifestations with a progressive course and a poor response to the treatment. It has not been considered as a cause of epileptic status. CASE REPORTS: Two women without important past medical history with normal psychomotor development before their suffering, with manifestations of 2 years of evolution the first one and 8 years on the second case characterized by myoclonic generalized, partial complex seizures and progressive deterioration of the mental functions that joined to our institution in a non convulsive epileptic status and they featured with a different evolution. The first patient with favorable control of the event with a single medication and functionality recover later, the second one with torpid evolution complicated with an epileptic status convulsive widespread condition and a prolonged permanency in the unit of intensive therapy. In both patients the diagnosis of Lafora s disease was established based in the findings of the skin axilar biopsy. DISCUSSION AND CONCLUSION: We believe that Lafora s disease must be suspected as a probable cause of non convulsive epileptic status in patients with myoclonic epilepsy associated with other neurological manifestations and a refractary response to the medical treatment. The evolution and clinical response will depend on the evolutionary stage of the disease.

Retinoic acid reduces chemotherapy-induced neuropathy in an animal model and patients with lung cancer.

OBJECTIVE: To evaluate the effect of all-trans retinoic acid (ATRA) as treatment for chemotherapy-induced peripheral neuropathy in an experimental animal model and in a randomized, double-blinded, controlled trial in patients with non-small-cell lung cancer (NSCLC). METHODS: Forty male Wistar rats were randomized in 5 groups: group A, control; groups B and C, treated with cisplatin; and groups D and E, treated with paclitaxel. ATRA (20 mg/kg PO) was administered for 15 days in groups C and E. We evaluated neuropathy and nerve regeneration-related morphologic changes in sciatic nerve, the concentration of nerve growth factor (NGF), and retinoic acid receptor (RAR)-α and RAR-ß expression. In addition, 95 patients with NSCLC under chemotherapy treatment were randomized to either ATRA (20 mg/m(2)/d) or placebo. Serum NGF, neurophysiologic tests, and clinical neurotoxicity were assessed. RESULTS: The experimental animals developed neuropathy and axonal degeneration, associated with decreased NGF levels in peripheral nerves. Treatment with ATRA reversed sensorial changes and nerve morphology; this was associated with increased NGF levels and RAR-ß expression. Patients treated with chemotherapy had clinical neuropathy and axonal loss assessed by neurophysiology, which was related to decreased NGF levels. ATRA reduced axonal degeneration demonstrated by nerve conduction velocity and clinical manifestations of neuropathy grades ≥2. CONCLUSIONS: ATRA reduced chemotherapy-induced experimental neuropathy, increased NGF levels, and induced RAR-ß expression in nerve. In patients, reduction of NGF in serum was associated with the severity of neuropathy; ATRA treatment reduced the electrophysiologic alterations. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that ATRA improves nerve conduction in patients with chemotherapy-induced peripheral neuropathy.