[Utility of the study of the vegetative nervous system in the differential diagnosis between Parkinson's disease and multiple system atrophy]. / Utilidad del estudio del sistema nervioso vegetativo en el diagnóstico diferencial entre la enfermedad de Parkinson y la atrofia multisistémica.

INTRODUCTION: The aim of this study is to show if the exploration of the autonomic nervous system is useful to improve the specificity of clinical criteria of Parkinson's Disease (PD) and Multiple System Atrophy (MSA). PATIENTS AND METHODS: 20 patients with PD and 13 patients with MSA were studied. After 12 hours in off medication, NE and GH were measured in supine position and NE after 5 minutes standing. Later, GH levels were recorded at 15, 30, 45 and 60 minutes after a dose of 0.005 mg/kg of apomorphine. Finally, analysis of the symptoms of autonomic dysfunction and levodopa test were carried out. RESULTS: Sympathetic response to postural changes was significantly higher in patients with PD (NE increase in relation to basal: PD: 170.90 +/- 110.08 pg/ml; MSA: 91.33 +/- 73.79 pg/ml; p = 0.029). No differences were found in the response of GH to apomorphine (GH peak at 45 minutes: PD: 2.37 +/- 2.7 ng/ml; MSA: 1.69 +/- 1.90 ng/ml; ns). The symptoms of autonomic dysfunction were more frequently in patients with MSA. The stridor was specific to MSA. Improvement in motor scores in the levodopa test was higher in patients with PD (PD: 39.7 %; MSA: 17.89; p = 0.019). DISCUSSION: Sympathetic response to postural changes, description of symptoms of autonomic dysfunction, and motor response to levodopa test are useful tools in order to improve specificity of the diagnostic criteria of PD and MSA. The GH test with apomorphine was not useful for a differential diagnosis.

Utilidad del estudio del sistema nervioso vegetativo en el diágnóstico diferencial entre la enfermedad de Parkinson y la atrofia multisistémica / Utility of the study of the vegetative nervous system inthe differential diagnosis between Parkinson´s disease and multiple system atrophy

Introduction. El objetivo de este estudio es valorar si mediante la exploración del sistema nervioso vegetativo se consigue mejorar la especificidad de los criterios diagnósticos de la enfermedad de parkinson (EP) y de la atrofia multisistémica (AMS). Pacientes y métodos. Se han estudiado 20pacientes con EP y 13 pacientes con AMS. En ausencia de medicación durante 12 horas se determinaron la noradrenalina (NA) y la hormona de crecimiento (GH) en decúbito y la NA tras 5 min en bipedestación. Después se determinó la GH a los 15, 30, 45 y 60 min de una dosis de 0,005 mg/kg de apomorfina. Se analizaron las manifestaciones clínicas de disfunción vegetativa. Finalmente se realizó una prueba aguada de levodopa. Resultados. La respuesta simpática al cambio postural (incremento de NA respecto a la basal) fue superior en los pacientes con EP (EP: 170,90+/-110,8 pg/ml; AMS: 91,33+/-73,79 pg/ml; p=0,029). No hubo diferencias en la respuesta de la GH a la apomorfina (GH 45 min: Ep: 2,37 +/-2,7 ng/ml;AMS: 1,69+/-1,90 ng/ml; ns). Los síntomas de difusión vegetativa fueron más frecuentes en los pacientes con AMS. El estridor fue específico de la AMS. El beneficio obtenido en la prueba de levodopa fue superior en los pacientes con EP (EP: 39,7%; AMS: 17,89; P=0,019). Discusión. La respuesta simpática a los cambios posturales, la descrpción de los síntomas de disfunción vegetativo y la respuesta motora en la prueba de levodopa son útiles para mejorar la especificidad de los criterios diagnósticos de la EP y de la AMS. El test de la GH con apomorfina no ha sido útil para el diagnóstico diferencial

Introduction. The aim of this study is to show if the exploration of the automatic nervous system is useful to improve the specificity of clinical criteria of Parkinson´s (PD) and Multiple System Atrophy (MSA). Patients and methods. 20 patients with PD and 13 patients with MSA were studied. After 12 hours in off medication, NE and GH were measured in supine position and NE after 5 minutes standing. Later, GH levels were recorder at 15, 30, 45 and 60 minutes after a dose of 0.005 mg/kg of apomorphine. Finally, analysis of the symptoms of autonomic dysfunction and levodopa test were carried out. Results. Sympathetic response to postural changes was significantly higher in patients with PD (NE increase in relation to basal: PD 170.90+/-110.08 pg/ml; MSA: 91.33+/-73.79 pg/ml; p=0.029). No differences were found in the response of GH to apomorphine (GH peak at 45 minutes: PD: 2.37+/-2.7 ng/ml; MSA: 1.69+/-1.90 ng/ml; ns). The symptoms of autonomic dysfunction were more frequently in patients with PD (PD: 39.7%; MSA: 17.89; p=0.019). Discussion. Sympathetic response to postural changes, description of symptoms of autonomic dysfunction, and motor response to levodopa test are useful tools in order to improve specificity of the diagnostic criteria of PD and MSA. The GH test with apomorphine was not useful for a differential diagnosis

[Unilateral optic neuropathy associated to a dural arterio-venous fistula]. / Neuropatía óptica unilateral asociada a fístula arteriovenosa dural.

We report the case of a 43-year-old female patient that had a subacute loss of visual acuity on her left eye, initially lacking any additional symptom or sign of intracranial hypertension. She was diagnosed and studied as an optic neuropathy. The cranial MR was normal and it did not show changes on the signal of the optic nerve. The patient did not improve with steroidal treatment. She was re-admitted three months later due to cephalalgia without any modification of the visual symptomatology. On this occasion a high intracranial pressure (400 mmH20) was recorder on lumbar tap. A thrombosis of the right transverse sinus with an associated complex dural arterio-venous fistula, was visualized at the Angio-MRI and cerebral arteriography. We suggest a relationship between optical neuropathy and dural arterio-venous fistula. We also discuss the attitude with regard to patients suffering from optic neuropathies and endocranial hypertension of uncertain origin.

Neuropatía óptica unilateral asociada a fístula arteriovenosa dural / Unilateral optic neuropathy associated to a dural arterio-venous fistula

Presentamos el caso de una paciente de 43 años con pérdida subaguda de agudeza visual del ojo izquierdo, con borramiento papilar y sin síntomas de hipertensión endocraneal, compatible con el diagnóstico inicial de neuropatía óptica. La resonancia magnética nuclear (RMN) craneal fue normal, sin alteración en la señal del nervio óptico. La paciente no mejoró con tratamiento esteroide y a los tres meses reingresó por cefalea, sin modificación de los síntomas visuales. En esta ocasión se detectó una presión intracraneal elevada (400 mmH20), y en la angio-RMN y la arteriografía cerebral se visualizó una trombosis del seno transverso del lado derecho, junto con el desarrollo de una fístula arteriovenosa dural (FAVD). Se plantea la relación entre la neuropatía óptica y la FAVD y se comenta la actitud a seguir ante pacientes con neuropatías ópticas e hipertensión endocraneal de origen incierto (AU)

[Spontaneous internal carotid artery dissection as a cause of unilateral lower cranial nerve palsies]. / Disección espontánea de la arteria carótida interna como causa de parálisis de pares craneales bulbares.

We present a 60-year-old man with a complete right IX-XII nerve palsy (Collet-Sicard syndrome) due to spontaneous right internal carotid artery (ICA) dissection. Magnetic resonance imaging (MRI) and MR angiography (MRA) showed signs of subadventitial dissection of the right ICA with a mural haematoma that expanded the circumference of the vessel at the level of the retrostyloid space, adjacent to the IX-XII nerves. No narrowing of the lumen or aneurysms was found. Clinical recovery was excellent after treatment with only antiplatelet drugs. Cervical internal carotid artery dissection should be included in the differential diagnosis of lower cranial nerve palsies. MRI and MRA are noninvasive, reliable methods for diagnosis and follow-up, especially in subadventitial dissections.

[The practice of neurology by regional specialists in Vizcaya (Spain)]. / Actividad asistencial de los facultativos especialistas de área en neurología: experiencia en Vizcaya.

We describe the practice of 3 regional specialists in neurology in 1993 and 1994 at Hospital de Cruces in Vizcaya. Each neurologist alternately spent 1 day working in the hospital ward and another day working in an external clinic in the region, thus allowing clinic patients to be seen consistently by the same neurologist, while responsibility for inpatients could be shared by 2 physicians. All clinic patients in the region and the majority of hospital patients were covered by these specialists. A yearly average of 683.05 new patients were seen; 177.72 (26.01%) as inpatients, 82 (12%) at the hospital-based outpatient clinic and 423.33 (61.98%) at other clinics in the region. The wait time for new patients in the regional clinics was 119.71 days; 21.97% of these patients were classified as having no neurological disease (with diagnoses of syncope and peripheral vertigo included in this group), 25.74% had headache and 40.12% had cerebrovascular, neuromuscular, degenerative disease or epilepsy. Hospitalized patients released with diagnoses of non-neurological disease or cephalea represented 12.91% of the total; 50.6% of hospital patients had cerebrovascular disease.

[Posterior cortical atrophy with progressive visual agnosia]. / Atrofia cortical posterior con agnosia visual progresiva.

Interest in progressive focal cerebral syndromes associated with classical degenerative diseases has increased in recent years. Descriptions of posterior cortical atrophy with progressive visual agnosia are relatively rare. We present 5 patients (2 women) ranging in age between 57 and 72 years old. In all cases symptoms began and progressed with no known etiology. All cases were sporadic. The main clinical signs are difficulty in recognizing objects, colors, persons or places; topographical disorientation and visual memory alterations; alexia, simultagnosia, loss of ocular fixing and optic ataxia. Some patients presented other disturbances of praxis or memory and 2 progressed to global dementia. Language function was preserved and behavioral disturbances did not develop. The amplitude of the P100 visual evoked potential was low but latency was normal in 4 patients and prolonged in 1. Brain images showed atrophy and hypoperfusion in the parieto-occipital area. The neuropathology status of these patients is unknown.

[Progressive multifocal leukoencephalopathy associated with leukopenia of unknown (iatrogenic?) origin]. / Leucoencefalopatía multifocal progresiva asociada a leucopenia de origen desconocido (yatrógena?).

Progressive multifocal leukoencephalopathy is a virtually always opportunistic infection of central nervous system caused by papova viruses, which clinically presents with symptoms and signs of involvement of different encephalic levels. We report a case with a double interest: on the one hand, both clinical features and lesions were limited to the brainstem and cerebellum; on the other hand, the disease developed in a previously healthy female in whom laboratory evidence of immunodeficiency of unknown origin was demonstrated. A reason for immunodeficiency was also not found at autopsy, being speculated that it could have been iatrogenically associated with antidepressant drugs.