Satoyoshi's syndrome related muscle spasms: functional study.

Satoyoshi syndrome is a rare disease presumed to be immunologically mediated, characterized by muscle spasms, alopecia and diarrhea. We describe the case of a female in whom the muscle spasms were the predominant feature and we analyze the changes in cortical and in spinal excitability under the paired pulses paradigm. Hyperexcitability was present in the H-reflex study, thus suggesting that the spinal cord is the structure most likely responsible for the spasms. This is the first reported case in Spain.

Abnormalities induced in reciprocal inhibition between orbicularis oculi and levator palpebrae following peripheral facial palsy.

Peripheral Facial palsy (PFP) is generally considered a benign condition with good recovery and no sequelae. Yet, a distortion in the gesture and abnormal blinking, as those typically found in blepharospasm, can potentially develop early on. Such abnormal movements seem to be related to remodelling mechanisms that take place in the process of recovery. We report 2 cases where such clinical features became evident following an idiopathic PFP, as a result of reciprocal inhibition of orbicularis oculi and levator palpebrae. Hence, the neurophysiological study revealed an increased frequency in the blinking, with bilateral trigeminal-facial facilitation and, most notably, a disturbance that only became evident when the eyes were maintained wide open. Interestingly, those features were not reproduced in other tasks where the blinking conditions had not been altered. Our findings suggest that sensory inputs (reflex afferent pathways) are involved in such abnormal movements. The insufficient eyelid closure (lagophthalmus) in the context of PFP is likely to account for the exaggerated corneal vulnerability, thus resulting in abnormal mechanisms of adaptation.

[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.

[Intracranial tumors simulating transient ischemic attacks]. / Tumores intracraneales que se manifiestan simulando ataques isquémicos transitorios.

INTRODUCTION: Occasionally intracranial tumors present a clinical picture similar to that seen with transient ischemic attacks (TIA). The mechanism for this is not clear. The differential diagnosis of these two disorders is important since their treatment is quite different. CLINICAL CASES: Two men and two women aged between 53 and 72 years of age, with no known cardiovascular risk factors, complained of one or several episodes of neurological deficit (hemisensitive deficits, transient blindness and isolated aphasia), lasting between 3 minutes and 6 hours. Neuroimaging investigations (CT and MR) showed images compatible with space occupying lesions localized to the convexity (parietal, frontotemporal and frontoparietal) and in one case at the edge of the sphenoid bone. Three patients were operated on. The lesions were confirmed as tumors (two cases of meningiomas and one of glioblastoma). Symptoms did not recur after a mean follow up period of nine months. Various mechanisms have been suggested to explain this phenomenon: vascular compression due to pressure from the mass, changes in intracranial pressure, vascular steal phenomenon, negative focal seizures, Leao s cortical depression and others. The absence of risk factors which would justify a vascular cause, the correlation between localization of the tumour and the clinical signs, and the disappearance of symptoms following surgery would seem to support the hypothesis that in our cases the symptoms observed were due to intracranial space occupying lesions. CONCLUSION: The presence of an intracranial tumour should be ruled out by using neuroradiological investigations in patients complaining of transient episodes of neurological deficits.

A new median motor test: comparison with conventional motor studies in carpal tunnel syndrome.

INTRODUCTION: Carpal tunnel syndrome (CTS) is the most commonly operated of entrapment peripheral neuropathies, and its surgical outcome largely depends on the underlying mechanism involved. Early identification of CTS is essential because it is associated with a better prognosis. AIM: To identify CTS at an earlier stage and hence improve the potential future outcome, the authors propose incorporating a new method of using the second lumbricalis (2L-MC) to register the nerve conduction. METHODS: We prospectively evaluated 121 of 216 patients with confirmed carpal tunnel syndrome and 49 cases from 25 healthy subjects. According to the accepted criteria of Stevens, we divided our study into two groups of mild CTS and moderate CTS, respectively. Following our proposed method, we stimulated the palm, distal forearm, and elbow and measuring the difference in latency between the second lumbricalis and the second interosseous, in addition to the conventional neurophysiologic assessments. RESULTS: Seventeen of 109 (15.6%) hands showed a significant difference in latency using 2L-MC within the mild CTS group, while only 4 of 107 (3.7%) within the moderate CTS group remained undiagnosed using this method. In the mild CTS group, motor involvement was only shown to be slightly more sensitive when compared with 2LI-DML. DISCUSSION: Adding the proposed test to the conventional studies of CTS in our cases presented showed a 14% increased sensitivity in detecting motor dysfunction when compared with conventional studies alone. Therefore, this new 2L-MC study represents a useful technique for assessing early motor involvement in CTS and would only add a few extra minutes to the standard diagnostic procedures. To conclude, we propose that incorporating this study as part of the standard assessment of CTS is given further consideration.

Tumores intracraneales que se manifiestan simulando ataques isquémicos transitorios / Intracranial tumors simulating transient ischemic attacks

Introducción. La literatura sólo observa la publicación de siete casos de placa móvil sobre carótida cuya descripción imprecisa dificulta que se diferencie de las lesiones móviles de tipo trombo. Por otra parte, la historia natural de este tipo de lesiones no es conocida y su tratamiento resulta controvertido por cuanto existe la tendencia de elegir el tipo de tratamiento a seguir de acuerdo con el `potencial embolígeno' de la afección. Casos clínicos. Se describen dos casos de placa móvil sobre carótida. El diagnóstico se ha practicado en ambos casos mediante ecografía Doppler. El primer caso es un paciente neurológicamente sintomático, cuya placa móvil está asociada a una estenosis superior al 70 por ciento. Se le se indica tratamiento quirúrgico. El segundo caso es neurológicamente asintomático. En el estudio mediante ecografía Doppler se observa el fragmento de placa móvil sobre la carótida común, en la zona del seno carotídeo, sobre una estenosis del 30-50 por ciento. En la zona distal de la misma, sobre el bulbo de la carótida interna, se encuentra una estenosis >70 por ciento. En este caso se indica tratamiento médico debido al deteriorado estado general del paciente. Ambos pacientes han evolucionado favorablemente. Conclusiones. Sumados al segundo caso aquí descrito, son tres los pacientes con placa móvil carotídea (neurológicamente asintomáticos) que han evolucionado favorablemente con tratamiento médico. Mientras no se disponga de más datos acerca de la historia natural de estas lesiones no deberían considerarse, por sí mismas, motivo suficiente para indicar la endarterectomía carotídea. La posible indicación de tratamiento quirúrgico estará en función del grado de estenosis carotídea. Introducción. Los tumores intracraneales se manifiestan ocasionalmente simulando accidentes isquémicos transitorios (AIT). El mecanismo por el que se produce dicho fenómeno no es bien conocido. El diagnóstico diferencial de ambos procesos es importante ya que el manejo terapéutico es radicalmente distinto. Casos clínicos. Dos varones y dos mujeres de entre 53 y 72 años de edad, sin factores de riesgo cardiovascular conocidos, refirieron uno o varios episodios de déficit neurológico (déficit hemisensitivos, amaurosis fugax y afasia aislada), que duraron entre 3 minutos y 6 horas. Las pruebas de neuroimagen (TC y RM) mostraron imágenes compatibles con lesiones ocupantes de espacio localizadas en la convexidad (parietal, frontotemporal y frontoparietal) y en el borde del esfenoides en un caso. Tres pacientes fueron intervenidos, y se confirmó la naturaleza tumoral de dichas lesiones (meningiomas en dos casos, glioblastoma en uno). Los síntomas no reaparecieron después de un seguimiento medio de nueve meses. Se han propuesto diversos mecanismos para explicar este fenómeno: compresión vascular por efecto de masa, cambios en la presión intracraneal, fenómeno de robo vascular, crisis focales negativas, depresión cortical de Léao, entre otros. La ausencia de factores de riesgo que justifiquen una causa vascular, la correlación entre la localización del tumor y la clínica y la desaparición de los síntomas tras la cirugía parece apoyar que en nuestros casos los síntomas observados han sido producidos por los procesos expansivos intracraneales. Conclusión. La presencia de un tumor intracraneal debe ser excluida mediante pruebas neurorradiológicas en aquellos pacientes que experimenten episodios deficitarios transitorios (AU)

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

Introducción. El sobresalto es un reflejo fisiológico primitivo presente en animales evolucionados. Puede definirse como aquella brusca reacción defensiva desencadenada por un estímulo sensorial adecuado, auditivo como más eficaz, que se traduce clínicamente por la brusca y secuencial contracción de grupos musculares, con inicio en territorios faciales (orbicularis oculii bilateralmente), y posteriormente en el resto de los craneales, axiales y de extremidades, con predominio en regiones proximales y flexoras de miembros superiores. El sobresalto patológico es aquel sobresalto exagerado y precipitado por estímulos inadecuados (escaso umbral de intensidad o duración). Aparece por diversas causas y mecanismos, entre ellos una forma peculiar de epilepsia refleja, la epilepsia sobresalto. Esta forma de epilepsia, habitualmente presente en afectados por encefalopatías postanóxicas infantiles perinatales, comparte la expresión clínica con las otras causas de sobresalto patológico, pero presenta una semiología electroencefalográfica muy característica. Caso clínico. Presentamos el caso de un niño con una epilepsia sobresalto presentada clínicamente en forma de estado subintrante en el contexto de una encefalopatía postanóxica por ahogamiento. Con un adecuado tratamiento se consiguió eliminar la fenomenología epiléptica, además de mejorar notablemente su calidad de vida (vigilia y sueño). Conclusión. En el presente artículo revisamos las características y clasificaciones de una forma de epilepsia refleja, la epilepsia del sobresalto (AU)

Emigrantes neurólogos / Emigrant neurologists

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