Degeneración hepatocerebral adquirida / Acquired hepatocerebral degeneration

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[Frontal cortical infarction and contralateral postural and international tremor]. / Infarto cortical frontal y temblor postural e intencional contralateral.

INTRODUCTION: Movement disorders, when caused by ischemic stroke, may appear as initial manifestation or after a variable interval of time. Among postictal movement disorders tremor is an uncommon manifestation. Holmes' tremor, which is a mixed tremor, is the most frequent type of tremor after stroke. It is associated to infarcts of various localizations like mesencephalon, pons, cerebellum, and thalamus. Cortical infarct is exceptional as a cause of tremor. CASE REPORT: A sixty-three-year-old woman, with hypertension, who, 2 weeks after a cortical infarct located in the precentral circunvolution of the left frontal lobe, presented with a postural and intention tremor of low frequency (4 Hz) and high amplitude at the right arm. Tremor is still present 2 years after stroke and it has improved partially after treatment with clonazepam. DISCUSSION: Frontal cortical infarcts may cause a contralateral postural and intentional tremor. It usually appears after a time interval. Tremor physiopathology may be related, in these cases, to interruption of the inhibitory fronto-subcortical motor circuit.

Infarto cortical frontal y temblor postural e intencional contralateral / Frontal cortical infarction and contralateral postural and international tremor

Introducción. El ictus isquémico es una causa infrecuente de trastornos del movimiento que pueden formar parte de la clínica inicial o presentarse de forma diferida. Entre los movimientos involuntarios postictales el temblor es excepcional. El temblor más frecuente tras un ictus es un temblor mixto, de reposo, postural e intencional, denominado temblor de Holmes, que puede presentarse con infartos de topografía muy diversa (mesencéfalo, protuberancia, cerebelo y tálamo). La aparición de temblor tras infartos corticales es excepcional. Caso clínico. Mujer de 63 años, hipertensa, que 2 semanas después de un infarto cortical en la circunvolución precentral del lóbulo frontal izquierdo comenzó con un temblor postural e intencional, de gran amplitud y baja frecuencia (4 Hz) en el miembro superior derecho. El temblor persistía 2 años después del ictus y mejoró discretamente con clonazepam. Discusión. Los infartos corticales frontales son una causa infrecuente de temblor postural e intencional contralateral que suele presentarse de forma diferida. En estos casos la fisiopatología del temblor podría estar relacionada con la interrupción del circuito motor inhibidor frontosubcortical

Introduction. Movement disorders, when caused by ischemic stroke, may appear as initial manifestation or after a variable interval of time. Among postictal movement disorders tremor is an uncommon manifestation. Holmes’ tremor, which is a mixed tremor, is the most frequent type of tremor after stroke. It is associated to infarcts of various localizations like mesencephalon, pons, cerebellum, and thalamus. Cortical infarct is exceptional as a cause of tremor. Case report. A sixty-three-year-old woman, with hypertension, who, 2 weeks after a cortical infarct located in the precentral circunvolution of the left frontal lobe, presented with a postural and intention tremor of low frequency (4 Hz) and high amplitude at the right arm. Tremor is still present 2 years after stroke and it has improved partially after treatment with clonazepam. Discussion. Frontal cortical infarcts may cause a contralateral postural and intentional tremor. It usually appears after a time interval. Tremor physiopathology may be related, in these cases, to interruption of the inhibitory fronto-subcortical motor circuit

[A descriptive analysis of the demand for ambulatory neurological care in the health district of Huelva]. / Análisis descriptivo de la demanda de asistencia neurológica ambulatoria en un área sanitaria de Huelva.

INTRODUCTION: In recent years the demand for ambulatory neurological care has risen. Studying this situation can help to improve health care planning and the quality of the referrals from primary care. AIM: To analyse a number of variables involved in the first visits referred from primary care to neurology services. PATIENTS AND METHODS: We conducted a prospective, descriptive study in which data was consecutively collected about 500 new patients from primary care who had appointments for a visit to general neurology services in the health district of the province of Huelva. Demographic features, the reason for referral, initial diagnoses and the case resolution index were analysed. RESULTS: Mean age was 51 years old, and patients were predominantly female (63.4%). The most frequent diagnoses were headaches (42.8%) and cognitive impairment (12%). In 8.2% of cases the referred patients had no neurological pathology. Data showed that 40.2% were discharged after the first visit. CONCLUSIONS: The demographic characteristics and reasons for the visit were similar to those previously published on other regions in Spain. The high percentage of direct discharges translates into a poor selection of the patients referred from primary care. Improving the quality of referrals would make it possible to enhance the efficiency of ambulatory neurological care.

Análisis descriptivo de la demanda de asistencia neurológica ambulatoria en un área sanitaria de Huelva / A descriptive analysis of the demand for ambulatory neurological care in the health district of Huelva

Introducción. En los últimos años se ha incrementado la demanda de asistencia neurológica ambulatoria. Su estudio puede contribuir a mejorar la planificación sanitaria y la calidad de las derivaciones desde atención primaria. Objetivo. Analizar diversas variables de las primeras visitas remitidas desde atención primaria a las consultas de neurología. Pacientes y métodos. Estudio prospectivo descriptivo en el que se recogieron de forma consecutiva los datos de 500 pacientes nuevos procedentes de atención primaria citados en una consulta de neurología general en el área sanitaria de la provincia de Huelva. Se analizaron las características demográficas, el motivo de derivación, los diagnósticos iniciales y el índice de resolución de los casos. Resultados. La media de edad fue de 51 años, con un predominio femenino (63,4%). Los diagnósticos más frecuentes fueron las cefaleas (42,8%) y el deterioro cognitivo (12%). El 8,2% de los pacientes remitidos no tenía ninguna patología neurológica. El 40,2% fue dado de alta tras la primera visita. Conclusiones. Las características demográficas y los motivos de consulta son similares a los publicados previamente en otras comunidades españolas. El elevado porcentaje de altas directas traduce una escasa selección de los pacientes remitidos desde atención primaria. La mejora en la calidad de las derivaciones permitiría mejorar la eficiencia de la asistencia neurológica ambulatoria

Introduction. In recent years the demand for ambulatory neurological care has risen. Studying this situation can help to improve health care planning and the quality of the referrals from primary care. Aim.To analyse a number of variables involved in the first visits referred from primary care to neurology services. Patients and methods. We conducted a prospective, descriptive study in which data was consecutively collected about 500 new patients from primary care who had appointments for a visit to general neurology services in the health district of the province of Huelva. Demographic features, the reason for referral, initial diagnoses and the case resolution index were analysed. Results. Mean age was 51 years old, and patients were predominantly female (63.4%). The most frequent diagnoses were headaches (42.8%) and cognitive impairment (12%). In 8.2% of cases the referred patients had no neurological pathology. Data showed that 40.2% were discharged after the first visit. Conclusions. The demographic characteristics and reasons for the visit were similar to those previously published on other regions in Spain. The high percentage of direct discharges translates into a poor selection of the patients referred from primary care. Improving the quality of referrals would make it possible to enhance the efficiency of ambulatory neurological care

[Lumbosacral plexopathy as a form of presentation of an aneurysm of the iliac artery]. / Plexopatía lumbosacra como forma de presentación de un aneurisma de la arteria ilíaca.

INTRODUCTION: The etiology of lumbosacral plexopathy is often due to compression. One of the less common causes of this is aneurysm of the iliac artery. However, 13% of the patients with this disorder initially have symptoms of plexus irritation or deficit. CLINICAL CASE: We describe the case of a 42 year-old-man, with no previous medical history, who complained of right-sided sciatica for the previous three months. On examination there were clinical signs of a lesion of the right lumbosacral plexus. On CT of the pelvis and MR of the lumbar spine there were images compatible with an aneurysm of the right iliac artery. This diagnosis was confirmed on arteriography. Treatment was surgical (aneurysmography and right iliofemoral by-pass). The pain disappeared and the motor deficit improved considerably. CONCLUSION: When a patient presents with lumbosacral plexopathy, an iliac aneurysm should be considered as a possible etiological factor. In such cases early diagnosis is essential, since surgical treatment will usually resolve the clinical problem.

[Pourfour du Petit syndrome]. / Síndrome de Pourfour du Petit.

The Pourfour du Petit's syndrome is characterized by the unilateral appearance of mydriasis, lid retraction and exophthalmos. It suggests the existence of a localized oculosympathetic hyperactivity. It tends be to caused by injuries that suppose a stimulus of the sympathetics fibers at level of the proximal portion of the first dorsal root or in the cervical sympathetic chain. We report the clinical case of a young patient who developed a Pourfour du Petit's syndrome secondary to a small condrosarcoma of the proximal portion of the first rib. The observation of this syndrome is exceptional but its knowledge permits, by the great topographic value that possesses, a rapid identification of the causative injury.

[Bilateral hypoacusia and basilar dolichoectasia]. / Hipoacusia bilateral y dolicoectasia basilar.

INTRODUCTION: The clinical findings of basilar dolichoectasia (DB) are caused by compression of the cranial nerves, vertebrobasilar ischaemia, hydrocephaly and compression of the brainstem. Hypoacusia as an isolated finding in DB is very rare. CLINICAL CASE: We describe the case of a 48-year-old hypertensive man with bilateral hypoacusia as an isolated finding in DB. Diagnosis was made on MR, and arteriography showed changes characteristic of an anomalous blood vessel. CONCLUSIONS: In patients with hypoacusia of uncertain aetiology, specially if they are also hypertensive, one should rule out the possibility of DB using cerebral magnetic resonance or angioresonance.

[The use of FD-6 monoclonal antibody in diagnosing and detecting the carriers of familial amyloidotic polyneuropathy type I]. / Utilidad del anticuerpo monoclonal FD-6 para el diagnóstico y la detección de portadores en la polineuropatía amiloidótica familiar tipo I.

Familial amyloidotic polyneuropathy type I (FAF-I) is caused by a specific genetic mutation that gives rise to a transthyretin anomaly whose presence in serum constitutes the biochemical marker for this disease. We studied the serum of 7 patients and 16 asymptomatic members of their immediate families using ELISA with FD-6 monoclonal antibody to detect the transthyretin anomaly. Positive results were found for the 7 patients, including the 2 patients whose disease was apparently sporadic, and 12 carriers were detected among the family members. This technique makes sural nerve biopsy unnecessary for establishing a diagnosis in patients whose clinical signs are consistent with FAP-I. Asymptomatic carriers are also detected, facilitating appropriate genetic counseling.

[Peripheral neuropathy in progressive systemic sclerosis]. / Neuropatía periférica en la esclerosis sistémica progresiva.

We report a patient that developed a sensorimotor polyneuropathy more than a year before the appearance of the typical clinical signs of progressive systemic sclerosis. A sural nerve biopsy showed epineural vasculitis with involvement of the basal membrane of the endoneural vessels, without proliferation of the connective tissue.