[Chorea as the presenting form of progressive systemic sclerosis]. / Corea como presentación de esclerosis sistémica progresiva.

We present an exceptional case of a patient with hemichorea of ischemic origin as a form of presentation of progressive systemic sclerosis. The neurologic complications, both central and peripheral, of this condition are considered infrequent and may be due to coexisting or iatrogenic factors, or may be a direct complication of progressive systemic sclerosis. There have been occasional reports of cerebrovascular disease in progressive systemic sclerosis, and this apparent rarity may be related to structural and morphological variations in brain arteries. Recently, there have been reports of primary cerebrovascular changes and vascular calcifications in progressive systemic sclerosis. There are no reported cases associated with movement disorders. The patient is a 66-year-old ex-smoker, and no other vascular risk factors, with chronic Raynaud's phenomenon affecting both hands, and polyarthralgias. Three months previously he began to complain of choreic movements affecting the right-sided limbs without any other neurological manifestations. The abnormal movements responded to haloperidol. There were skin changes suggestive of progressive systemic sclerosis, and capillaroscopy showed abundant avascular zones with dilated capillary loops. Anticentromere antibody titer was elevated to 1/1280. Brain arteriography was normal, and the brain SPECT showed bilateral hypoperfusion bilaterally, predominantly on the left side. Brain MR imaging confirmed an infarct in the left caudate and adjacent internal capsule. The study of this case supports the hypothesis of a direct primary cerebrovascular damage in progressive systemic sclerosis.

Corea como presentación de esclerosis sistémica progresiva / Chorea as the presentating manifestation of progressive systemic sclerosis

Presentamos un caso excepcional de un paciente con hemicorea aguda de origen isquémico como forma de inicio de una esclerosis sistémica progresiva. Las complicaciones neurológicas, tanto centrales como periféricas, de esta enfermedad se consideran inhabituales y se pueden deber a factores coexistentes, iatrogénicos o complicaciones directas de la propia esclerosis sistémica progresiva. La disfunción clínica cerebrovascular en pacientes con esclerosis sistémica progresiva ha sido referida de forma ocasional, y ésta aparente rareza puede ser debida a diversas variaciones estructurales y morfológicas de las arterias cerebrales. Recientemente se han descrito casos con cambios cerebrovasculares primarios y calcificaciones vasculares en la esclerosis sistémica progresiva. No hay casos de esta enfermedad asociados a trastornos de movimientos. El paciente es un varón, de 66 años de edad, ex fumador, sin otros factores de riesgo vasculares, con fenómeno de Raynaud crónico en ambas manos y poliartralgias. Tres meses antes comenzó a presentar movimientos coreicos en las extremidades derechas, sin otras manifestaciones neurológicas. Los movimientos involuntarios se controlaron con haloperidol. Existían cambios cutáneos sugerentes de esclerosis sistémica progresiva, y la capilaroscopia puso de manifiesto abundantes zonas avasculares con asas capilares dilatadas. Los anticuerpos anticentrómero estaban elevados a un título de 1/1.280. La arteriografía cerebral fue normal y la SPECT cerebral demostró hipoperfusión frontoparietal bilateral de predominio izquierdo. La RM cerebral confirmó un infarto en el núcleo caudado y cápsula interna izquierda. El estudio de este caso permite apoyar la hipótesis de un daño cerebrovascular primario ocasionado por la propia esclerosis sistémica progresiva (AU)

[Infections with cutaneous and nervous system alterations]. / Infecciones con afectación cutánea y del sistema nervioso.

OBJECTIVE: We review and update the clinical and diagnostic aspects in the most representative neurocutaneous infections, emphasizing the features of interdisciplinary interest. METHODS: Human skin is the primary host barrier against infection and his importance is critical in the immunocompromised population. The genetic hability of pathogen micro-organisms to bind the adhesion molecules of cellular membranes defines the anatomic affinity of each species. Cutaneous involvement can be crucial for diagnosis in infectious diseases. The characteristics of the elemental lesions and the accessible cytology, bacteriology and histopathology procedures, usually leads to a specific diagnosis. We highlight the cutaneous manifestations of the acute and subacute bacterial meningitides. We review the clinico-pathologic characteristics of the meningoencephalitidis associated to the viral exanthems. We describe the chronic bacterial entities with prominent cutaneous and neural affectation as lepra, syphilis and borreliosis, as well as the numerous clinical forms of presentation of herpesvirus hominis and varicellazoster. Finally, we stand out the transcendency of cutaneous findings in the HIV set. CONCLUSIONS: The appropriate interpretation of the infectious cutaneous semiology, supplemented with exams of direct samples, allow frequently to reach an ethiologic or orientated diagnosis, in a rapid, economic and non-invasive way. This information must be carefully incorporated to the study of high-morbidity infections, as there that concerns to the nervous system.

Milia en plaque.

A 59-year-old woman presented with milia grouped in plaques, in the preauricular areas, bilaterally. Follicle-damaging dermatoses, tumours and external agents, can lead to this peculiar clinical pattern. We outline the clinical and histological features which allow accurate diagnosis of this condition.