Neurología : publicación oficial de la Sociedad Española de Neurología
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We review the present status of surgery for Parkinson's disease. Surgical options for Parkinson's disease are rapidly spanding. The main objectives of surgical techniques are to restore the dopaminergic deficit in the striatum (transplantation) and to normalize the neuronal activity of the subthalamic-pallidal circuit (pallidotomy and deep brain stimulation). ⋯ Both types of surgical procedures are supported by strong scientific data. However, much work remains to be done in order to understand several aspects not clearly elucidated at present. The results and current indications for pallidotomy and deep brain stimulation are analyzed.
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Fluctuating stiffness and paroxysmal spasms of the trunk and legs are the primary features of stiff-man syndrome and it's variants, progressive encephalomyelopathy with rigidity and myoclonus (PERM) and stiff-leg syndrome. The spasms characterized by hyperextension of the back and legs are both spontaneous as well as stimulus-sensitive. They can be excruciatingly painful and are frequently accompanied by symptoms of autonomic dysregulation. ⋯ It consists of well-reproduced reflex EMG-activity commencing 50-80 ms after medial or tibial nerve stimulation and lasting several seconds thereafter. The activity is first myoclonic then spasmodic in nature, and commonly begins in the muscles most severely affected before spreading bidirectionally along the neuraxis. Spasmodic reflex myoclonus and the high incidence of antibodies against GAD are suggestive of an autoimmune disorder affecting GABAergic neurons in the spinal cord, but the precise locus of dysfunction remains to be elucidated.
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Non convulsive status epilepticus (NCSE) is defined by prolonged loss of awareness and epileptiform activity on electroencephalogram. There are several types of NCSE depending of the clinical and electroencephalographic manifestations: generalized (typical or atypical absence status, status epilepticus during sleep) and partial (complex partial and simple partial status epilepticus). NCSE diagnosis and treatment should be performed early due to the potential brain damage associated to it, specially in complex partial NCSE. Accurate differential diagnosis from other imitators of NCSE (psychogenic seizures, metabolic encephalopaties or antiepileptic drug intoxication) is necessary for early treatment.