[Nocturnal paroxysmal dystonia, movement disorder and epilepsy]. / Distonía paroxística nocturna, trastorno del movimiento y epilepsia.

INTRODUCTION: Nocturnal paroxysmal dystonia (NPD) is a disorder which appears during sleep. It is characterized by generally brief paroxysmal motor events which are complex when clinically expressed and are often repetitive. The origin of this disorder has been a matter for discussion for some time. Initially it was considered a specific movement disorder, but recently it has been suggested that it is epileptic in nature. CASE REPORT: In this study we present the case of a patient who is hospitalized in the Unit for epileptic surgery, suffering from epilepsy which does not respond to medical treatment and requires surgery. The patient is treated with conventional methodology, prolonged and continuous presurgical videoelectroencephalographic monitoring. EEG registers were used via electrodes placed in the scalp and skull, electrodes placed in the skull on adequate indication. Two types of perfectly defined electroclinical events occurred: some not epileptic and others clearly epileptic. On one occasion a convulsive tonic clonic epileptic crisis was recorded typical of frontal focal origin preceded by non epileptic motor phenomena. CONCLUSION: The presence of paroxysmal motor episodes during sleep, atypical as a form of epilepsy, the absence of unquestionable specific data in the EEG, and in the light of our discoveries, force us to consider the possibility that NPD is in fact a form of epilepsy caused in a reflex manner by a specific type of movement disorder during sleep, and whose origin should be more widely discussed.

[The association between post anoxic encephalopathy and startle epilepsy]. / Asociación entre encefalopatía postanóxica y epilepsia del sobresalto.

INTRODUCTION: The startle is a variant of primitive physiological reflex, present in developed animals. It could be defined as a sudden defensive reaction triggered by an adequate sensorial stimulus, most effective if auditory, consisting under a clinical basis on the sudden and sequential contraction of some muscular groups, in first place affecting the facial territory (orbicularis oculi bilaterally), followed by the rest of the cranial nerves, the axial musculature and finally the limbs, predominantly proximal and flexor regions of superior extremities. The pathological startle is that which is precipitated by inadequate stimulus and excessive in response to it (under a low level of intensity or duration). It appears on account of different causes and mechanisms, among them a peculiar form of reflex epilepsy, the startle epilepsy. This form of epilepsy commonly present on infant postanoxic encephalopathy, shares its clinical features with other causes of pathologic startle, even though it presents a very characteristic electroencephalographic semiology. CASE REPORT: We present the case of a child with a startle epilepsy clinically presented as a form of subintrant status epilepticus under the context of a postanoxic encephalopathy secondary to drowsiness. With an adequate treatment it was possible to eliminate the epileptic phenomena, thus notoriously improving his quality of life (vigil and asleep). CONCLUSION: We review the characteristics and classifications of a variant of reflex epilepsy, the startle epilepsy.

[Surgery unit for epilepsy]. / La Unidad de Cirugía de la Epilepsia.

INTRODUCTION: Only few medically refractory epileptic patients are evaluated for surgical treatment, in spite of the good results obtained at the Centers where epilepsy surgery is performed. OBJECTIVE: We are presenting the way the Gregorio Marañón Hospital Epilepsy Surgery Unit functions and their casuistry. PATIENTS AND METHODS: This Unit is composed by members of the Services of Neurosurgery, Neurology, Neurophysiology, Neuroradiology, Psychology, Neuropediatry, Nuclear Medicine and Neuroanestesiology. Patients are studied by means of protocols. RESULTS: Twenty cases were operated upon. The procedures were temporal lobectomy in 9 cases, amygdalo-hipocampectomy in 4, frontal lobectomy in 3, frontal topectomy in 2, partial hemispherectomy in 1 and vagal nerve stimulation in 1. In 16 of the 19 surgery specimen there were some pathological abnormalities. In the short follow-up 13 patients are seizures free experience significant improvement.

Sleep apnea syndrome in an adult patient with mandibular hypoplasia.

Sleep apnea syndrome is a condition which may be related to mandibular hypoplasia of a congenital as well as developmental nature. It is extremely unusual in adults; however, a case of sleep apnea syndrome gradually developing in an adult patient is illustrated in this report. Correction by means of maxillary and mandibular osteotomies was indicated.

[Management of podalic delivery]. / Conduite lors de l'assistance de l'accouchement podalique.

A study has been made of obstetric assistance in 1 796 podalic version labors between 1966 and 1982, with 12.4% of cesarean sections, an extremely conservative criterion. The corrected intra-and postnatal mortalities were 141.1% and 26.6% for premature and mature deliveries respectively. 744 children were examined between 4 and 10 years after birth, using clinical studies, intelligence quotient measurements by the method of Wppsi or WISC (according to age), EEG observations if necessary and stimulatory, motor and behavioral neurological tests. The results are compared with those of a similar study using 418 control children whose cephalic version births were spontaneous and completely normal. Care should be taken in interpretation of births through the vaginal canal in order to exclude the 30%-50% of cases with a wide safety margin, in which the danger is essentially zero in mature fetuses and in premature fetuses of 2 to 2.5 kg. Cesarean section should be performed almost systematically when the fetus weighs less than 2 kg, so as to avoid any possible danger to the child.