Esclerodermia lineal en coup de sabre. Manifestaciones neurológicas, imágenes y revisión / Linear scleroderma en coup de sabre. Neurological symptoms images and review

Introducción. La esclerodermia lineal en ‘golpe de sable’, mejor conocida como esclerodermia lineal en coup de sabre (ELCS), es una enfermedad rara de causa incierta, caracterizada por atrofia focal progresiva craneofacial y, en parte, es diferente al síndrome de Parry-Romberg (SPR). Casos clínicos. Comunicamos tres pacientes con ELCS (2 mujeres y 1 hombre, con un promedio de edad de 40 años). Las manifestaciones neurológicas principales fueron cefalea y crisis convulsivas. Los alteraciones radiológicas, aunque diversas, fueron todas ipsilaterales al ‘golpe de sable’. En una paciente sometida a biopsia se obtuvo evidencia histopatológica de gliosis e infiltrado inflamatorio mixto perivascular. Destacamos en otro paciente la afectación cerebrovascular, dado el hallazgo de un infarto cerebeloso antiguo subclínico y oclusión de la arteria cerebelosa superior en ausencia de otra posible causa. Conclusiones. La ELCS, cuando afecta al sistema nervioso central es heterogénea en su presentación clínica y radiológica. Los estudios de imagen durante el control clínico y los hallazgos histopatológicos apoyan un proceso inflamatorio focal que puede ser progresivo. La afectación arterial se debe probablemente a una vasculopatía inflamatoria no ateroesclerosa, oclusiva y crónica (AU)

Introduction. ‘Sword stroke’ linear scleroderma, which is better known as linear scleroderma en coup de sabre (LSCS), is a rare disease with an uncertain causation that is characterised by progressive craniofacial focal atrophy and is, at least in part, different from Parry-Romberg syndrome (PRS). Case reports. Here, we report on the cases of 3 patients with LSCS (2 females and 1 male, with a mean age of 40 years). The main neurological symptoms were headache and seizures. Although different alterations were observed in the X-ray images, they were all ipsilateral to the coup de sabre. Histopathological evidence for gliosis and mixed perivascular inflammatory infiltrate was found in the study of a biopsy specimen taken from one female. Cerebrovascular involvement was seen in another patient, as highlighted by the observation of an earlier subclinical cerebellar infarct and occlusion of the superior cerebellar artery in the absence of any other possible causation. Conclusions. When it affects the central nervous system, the clinical and radiological presentation of LSCS is heterogeneous. Both the imaging studies carried out during the clinical control and the histopathological findings suggest a focal inflammatory process that can be progressive. The arterial involvement is probably due to a non-atherosclerotic, occlusive and chronic inflammatory disease of the peripheral vessels (AU)

[Linear scleroderma en coup de sabre: neurological symptoms, images and review]. / Esclerodermia lineal en coup de sabre. Manifestaciones neurologicas, imagenes y revision

INTRODUCTION: 'Sword stroke' linear scleroderma, which is better known as linear scleroderma en coup de sabre (LSCS), is a rare disease with an uncertain causation that is characterised by progressive craniofacial focal atrophy and is, at least in part, different from Parry-Romberg syndrome (PRS). CASE REPORTS: Here, we report on the cases of 3 patients with LSCS (2 females and 1 male, with a mean age of 40 years). The main neurological symptoms were headache and seizures. Although different alterations were observed in the X-ray images, they were all ipsilateral to the coup de sabre. Histopathological evidence for gliosis and mixed perivascular inflammatory infiltrate was found in the study of a biopsy specimen taken from one female. Cerebrovascular involvement was seen in another patient, as highlighted by the observation of an earlier subclinical cerebellar infarct and occlusion of the superior cerebellar artery in the absence of any other possible causation. CONCLUSIONS: When it affects the central nervous system, the clinical and radiological presentation of LSCS is heterogeneous. Both the imaging studies carried out during the clinical control and the histopathological findings suggest a focal inflammatory process that can be progressive. The arterial involvement is probably due to a non-atherosclerotic, occlusive and chronic inflammatory disease of the peripheral vessels.

[Intracerebral haemorrhage in a referral hospital in the central-western region of Mexico]. / Hemorragia intracerebral en un hospital de referencia de la region centro-occidente de Mexico.

INTRODUCTION: Intracerebral haemorrhage (ICH) has received little attention in studies in Mexico. Isolated reports talk of high frequency, its importance as a disorder among young people, its being mainly located in the lobar regions and a high rate of recurrence. AIMS: The objective of this study was to characterise the clinical, radiological, therapeutic and prognostic spectrum of ICH in a general hospital in the central-western region of Mexico. PATIENTS AND METHODS: The study involved 270 consecutive patients over the age of 15 years with spontaneous ICH who were hospitalised in the Neurology and Neurosurgical Service in the Antiguo Hospital Civil de Guadalajara between the years 2000 and 2002. Their clinical history and progression was known at least on discharge from the hospital. RESULTS: The mean age was 63 years (12% under 40 years old) with no predominance according to gender (53% males). Arterial hypertension was the main risk factor in 69%, followed by obesity in 38%. There were no differences in the Glasgow administered on admission in three pre-established subgroups. The ICH was ganglionic in 64% of cases and lobar in 24%. Arterial hypertension was the principal cause of ICH in 76%. Ventricular aperture was noted in 53%. All the patients were treated in a general ward. Mortality in the acute phase occurred in 49%, although a poor progression was observed in 83%. Overall recurrence was 13%. Outpatient follow-up was poor. CONCLUSIONS: ICH shares most of the features reported in Anglo-Saxon series including aetiology and location. In our population, mortality and recurrence are high with important sequelae. The high frequency of ICH (40%) may represent a bias in the selection of hospitals.

Hemorragia intracerebral en un hospital de referencia de la región centro-occidente de México / Intracerebral haemorrhage in a referral Hospital in the central-Western región of Mexico

Introducción. La hemorragia intracerebral (HIC) se ha estudiado escasamente en México. Algunos comunicados aislados señalan una elevada frecuencia, importante afectación en jóvenes, mayor localización lobar y alta recurrencia. Objetivos. Caracterizar el espectro clínico, radiológico, terapéutico y pronóstico de la HIC en un hospital general de la región centro-occidente de México. Pacientes y métodos. Se incluyeron 270 pacientes consecutivos mayores de 15 años con HIC espontánea ingresados en el Servicio de Neurología y Neurocirugía del Antiguo Hospital Civil de Guadalajara, entre los años 2000 y 2002. Todos tenían historia clínica y una evolución conocida, al menos, hasta el alta hospitalaria. Resultados. La edad promedio fue de 63 años (12% menores de 40 años) sin predominio de sexo (53% hombres). La hipertensión arterial fue el principal factor de riesgo (69%), seguida de la obesidad (38%). No hubo diferencia en el Glasgow al ingreso en tres subgrupos preestablecidos. La localización de la HIC fue ganglionar en el 64% y lobar en el 24%. La hipertensión arterial fue la principal causa de HIC en el 76%. La apertura ventricular se consignó en un 53%. Todos los pacientes se trataron en la sala general. La mortalidad en fase aguda se dio en el 49%, aunque se consignó una mala evolución en el 83%. La recurrencia global fue de 13%. El seguimiento por consulta externa es pobre. Conclusiones. La HIC comparte la mayoría de las características comunicadas en series anglosajonas con inclusión de etiología y localización. En nuestra población, la mortalidad y la recurrencia son altas, con secuelas importantes. La elevada frecuencia de HIC (40%) puede representar un sesgo de selección hospitalaria (AU)

Introduction. Intracerebral haemorrhage (ICH) has received little attention in studies in Mexico. Isolated reports talk of high frequency, its importance as a disorder among young people, its being mainly located in the lobar regions and a high rate of recurrence. Aims. The objective of this study was to characterise the clinical, radiological, therapeutic and prognostic spectrum of ICH in a general hospital in the central-western region of Mexico. Patients and methods. The study involved 270 consecutive patients over the age of 15 years with spontaneous ICH who were hospitalised in the Neurology and Neurosurgical Service in the Antiguo Hospital Civil de Guadalajara between the years 2000 and 2002. Their clinical history and progression was known at least on discharge from the hospital. Results. The mean age was 63 years (12% under 40 years old) with no predominance according to gender (53% males). Arterial hypertension was the main risk factor in 69%, followed by obesity in 38%. There were no differences in the Glasgow administered on admission in three pre-established subgroups. The ICH was ganglionic in 64% of cases and lobar in 24%. Arterial hypertension was the principal cause of ICH in 76%. Ventricular aperture was noted in 53%. All the patients were treated in a general ward. Mortality in the acute phase occurred in 49%, although a poor progression was observed in 83%. Overall recurrence was 13%. Outpatient follow-up was poor. Conclusions. ICH shares most of the features reported in Anglo-Saxon series including aetiology and location. In our population, mortality and recurrence are high with important sequelae. The high frequency of ICH (40%) may represent a bias in the selection of hospitals (AU)

Hemorragia intracerebral asociada a una pesadilla. Comunicación de un caso / Intracerebral hemorrhage associated with a nightmare. Case report

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