[Paraneoplastic neuropathy with positive anti-Hu]. / Neuropatía paraneoplásica con anti-Hu positivo.

The case of a 72-year-old woman presenting sensory neuropathy and anti-Hu antibodies is reported. She was admitted in November 1995 with a one year history of sensory neuropathy. Her first symptoms were painful numbness and dysesthesias in both feet. She experienced progression of the sensory symptoms affecting upper limbs, and clumsiness of gait. One month before admission she complained of diminished strength in both hands. The neurologic examination showed anicocoric fixed pupils, with no reaction to light; convergence miosis was evident in the right eye (Argyll-Robertson pupil). In the lower limbs she had very mild distal weakness, and tendon reflexes were universally abolished. Pin and touch sensation, position sense and pallesthesia were absent in all four limbs. Romberg test was elicited, and a tabetic gait was patent. Pseudoathetotic movements were observed in hands and feet. An ulcer was present in the fifth finger of the right foot. Routine blood biochemistry and hematology showed a ESR of 105 and an increased IgG in the immune-electrophoretic run. Neurophysiologic evaluation disclosed a mild demyelinating neuropathy. Positive anti-Hu antibodies were found in the serum (Western blot - Athena Diagnostics); CSF was normal but not tested for anit-Hu. An abdominal CT scan disclosed multiple hypodense nodules in liver, right adrenal gland and peritoneum. A chest CT scan showed a hyperdense mass in the lower right pulmonary lobe and enlarged retrocava-pretracheal lymph nodes. A biopsy of the peritoneal nodule was performed, showing a metastatic small cell carcinoma. The patient died eight days after discharge. Although multiple organs were affected, she was independent until death, showing an indolent clinical course.

Familial bulbospinal neuronopathy with optic atrophy: a distinct entity.

A 61 year old woman and her 58 year old brother presented with the clinical picture of late onset progressive bulbar and spinal muscular atrophy with family history of involvement in successive generations. The sister also had optic neuropathy and the brother developed diabetes mellitus and sex hormone abnormalities. Neurophysiological and histopathological studies showed a pattern of motor and sensory neuronopathy. There was no abnormal expansion of CAG repeats in the androgen receptor gene. This family seems to have a previously unrecognised entity with the bulbospinal neuronopathy phenotype.

[What do adolescents think about their physician: a comparison between pediatricians and physicians for adults]. / Qué piensan los adolescentes de su médico: una comparación entre pediatras y médicos de adultos.

The objective of this study was to determine who is the primary care provider for adolescents and whether there are differences between pediatricians and general practitioners. To this end, 3,139 high school students from Barcelona, between 14 and 19 years of age, were surveyed between 1992 and 1993. We found that 14.3% were seen by pediatricians and 57% by general practitioners. Of those seen by pediatricians or general practitioners 72.3% and 76.1%, respectively, stated that they could not talk with their physician about any worry that they had. It was statistically more frequent (p < 0.01) that they could not do so with pediatricians because "they were always with an adult" or because "he would tell my parents". While it was significantly more frequent (p < 0.01) that they could not talk to general practitioners because of "lack of confidence" Adolescents attending a general practitioner office had seen their physician significantly more often (p < 0.01). We conclude that 1 in 4 adolescents does not know who is their primary care physician or does not have one. Although they have more confidence in pediatricians, they prefer to be seen alone and confidentially.

[Primary CNS lymphoma. Infratentorial localization and prolonged survival]. / Linfoma primario del sistema nervioso central. Localización infratentorial y prolongada sobrevida.

A case of a 35-year-old woman presenting infratentorial CNS lymphoma is reported. In 1990 she complained of diplopia, blurred vision and left horizontal nistagmus. An MRI disclosed a lesion in the medulla, pons, and cerebellar vermis and peduncles. Although no treatment was administered, a later RMI showed less extension of the tumor. One year after clinical diagnosis, she received corticosteroids; during the second year a stereotaxic biopsy of the cerebellar lesion was done showing a diffuse B cell non-Hodgkin's lymphoma. A whole brain irradiation was given (50 Gy). She did well for five years, and remains alive (79 months).

[Dolichoectatic intracranial arteries. Advances in images and therapeutics]. / Dolicoectasia de las arterias intracraneales. Adelantos en imágenes y terapéutica.

Dolichoectasia of intracranial arteries is an infrequent disease with an incidence less than 0.05% in general population. It represents 7% of all intracranial aneurysms. Commonly seen in middle age patients with severe atherosclerosis and hypertension, the affected arteries include the basilar artery, supraclinoid segment of the internal carotid artery, middle, anterior and posterior cerebral arteries; males are more frequently affected. The clinical features of these fusiform aneurysms are divided in three categories: ische-mic, cranial nerve compression and signs from mass effect. Hemorrhage may also occur. Nine patients with symptomatic cerebral blood vessel dolichoectasias are presented. Six of them were males with moderate or severe hypertension. Lesions were confined to the basilar artery in 3 cases, carotid arteries and the middle cerebral artery in 1 case, and both systems were affected in 4 patients. Middle cerebral arteries were affected in 5 cases and the anterior cerebral artery in one. An isolated fusiform aneurysm of the posterior cerebral artery is also presented (case 8) (Table 3). Motor or sensory deficits, ataxia, dementia, hemifacial spasm and parkinsonism were observed. One patient died from cerebro-meningeal hemorrhage (Table 2). All patients were studied with computerized axial tomography of the brain, 5 cases with four vessel cerebral angiography, 4 cases with magnetic resonance imaging (MRI) and case 5 with MRI angiography. Clinical symptoms depend on the affected vascular territory, size of the aneurysm and compression of adjacent structures. The histopathologic findings are atheromatous lesions, disruption of the internal elastic membrane and fibrosis of the muscular wall. The resultant is a diffuse deficiency of the muscular wall and the internal elastic membrane. Recent advances in neuroimaging such as better resolution of CT scan, magnetic resonance images (MRI) and MRI angiography increased the diagnosis of this pathology showing clearly the affected vessels. This avoids the use of conventional or digital subtraction angiography, reserved only for diagnosing suspected saccular aneurysm, evidence of subarachnoid hemorrhage or planning surgical treatment. The treatment of this entity may be medical or surgical. There is evidence suggesting a more favorable outcome with anticoagulation therapy, although antiaggregation is a reasonable alternative. In our experience no difference in clinical outcome was evident. Surgical treatment of this type of aneurysm includes intra- or extracranial occlusion of parent artery, clipping or aneurysm trapping, tourniquet occlusion, and circumferential wrapping with clip reinforcement. Endovascular occlusion has been accomplished with detachable balloon technique or coils. No surgical attempt was done in our cases. The prognosis is variable depending on the patients age, vessels involved and clinical complications.(ABSTRACT TRUNCATED AT 400 WORDS)

Dolicoectasia de las arterias intracraneales. Adelantos en imágenes y terapéutica. / [Dolichoectatic intracranial arteries. Advances in images and therapeutics]

Dolichoectasia of intracranial arteries is an infrequent disease with an incidence less than 0.05

in general population. It represents 7

of all intracranial aneurysms. Commonly seen in middle age patients with severe atherosclerosis and hypertension, the affected arteries include the basilar artery, supraclinoid segment of the internal carotid artery, middle, anterior and posterior cerebral arteries; males are more frequently affected. The clinical features of these fusiform aneurysms are divided in three categories: ische-mic, cranial nerve compression and signs from mass effect. Hemorrhage may also occur. Nine patients with symptomatic cerebral blood vessel dolichoectasias are presented. Six of them were males with moderate or severe hypertension. Lesions were confined to the basilar artery in 3 cases, carotid arteries and the middle cerebral artery in 1 case, and both systems were affected in 4 patients. Middle cerebral arteries were affected in 5 cases and the anterior cerebral artery in one. An isolated fusiform aneurysm of the posterior cerebral artery is also presented (case 8) (Table 3). Motor or sensory deficits, ataxia, dementia, hemifacial spasm and parkinsonism were observed. One patient died from cerebro-meningeal hemorrhage (Table 2). All patients were studied with computerized axial tomography of the brain, 5 cases with four vessel cerebral angiography, 4 cases with magnetic resonance imaging (MRI) and case 5 with MRI angiography. Clinical symptoms depend on the affected vascular territory, size of the aneurysm and compression of adjacent structures. The histopathologic findings are atheromatous lesions, disruption of the internal elastic membrane and fibrosis of the muscular wall. The resultant is a diffuse deficiency of the muscular wall and the internal elastic membrane. Recent advances in neuroimaging such as better resolution of CT scan, magnetic resonance images (MRI) and MRI angiography increased the diagnosis of this pathology showing clearly the affected vessels. This avoids the use of conventional or digital subtraction angiography, reserved only for diagnosing suspected saccular aneurysm, evidence of subarachnoid hemorrhage or planning surgical treatment. The treatment of this entity may be medical or surgical. There is evidence suggesting a more favorable outcome with anticoagulation therapy, although antiaggregation is a reasonable alternative. In our experience no difference in clinical outcome was evident. Surgical treatment of this type of aneurysm includes intra- or extracranial occlusion of parent artery, clipping or aneurysm trapping, tourniquet occlusion, and circumferential wrapping with clip reinforcement. Endovascular occlusion has been accomplished with detachable balloon technique or coils. No surgical attempt was done in our cases. The prognosis is variable depending on the patients age, vessels involved and clinical complications.(ABSTRACT TRUNCATED AT 400 WORDS)

[Neurologic complications by cocaine abuse]. / Complicaciones neurológicas por abuso de cocáina.

Argentina is facing an increase in cocaine use by adolescents and young adults from every socioeconomic background. It is calculated that up to 10% of all cocaine passing through this country is locally sold and consumed. Nevertheless, local information describing common cocaine-related neurological events is scarce. From August 1988 to March 1993, 13 patients were evaluated with neurological disease associated with cocaine abuse. Among these 13 patients (Table 1), the mean age was 29; 70% were men. Patients most commonly used the nasal route (snorting). Concomitant abuse of other intoxicants, especially alcohol, was frequent (85%). The major neurological complications included one or more seizures (n = 7), ischemic stroke (n = 2) (Fig. 1-2), hemorrhagic stroke (n = 2) associated with arteriovenous malformation (Fig. 3a-b), memory disturbances (n = 1) and paroxysmal dystonia (n = 1). Psychiatric complaints were present in all patients. Mortality was not observed. There was no correlation between the appearance of complications and the amount of cocaine used, or prior experience with this drug. Only one of the 7 patients with seizures had a previous history of seizures. All had generalized tonic-clonic seizures, and one had concomitant absence episodes. Cocaine modulates central neurotransmitters and has direct cerebrovascular effects. The neurological complications appear to be related to cocaine hyperadrenergic effects, striatal dopaminergic receptor hypersensitivity and perhaps vasculitis. Structural changes in the brain of long-term cocaine abusers could explain the persistence of neurologic symptoms after drug withdrawl.

Complicaciones neurológicas por abuso de cocaína / Neurologic complications by cocaine abuse

El consumo de cocaina ha adquirido proporciones epidemiológicas en los EEUU. Actualmente en nuestro país, sitio de paso de la droga, un 10 por ciento es retenida para su consumo, calculandose que un 80 por ciento de los drogadictos utilizan este alcaloide. Presentamos 13 paciente, 9 hombres y 4 mujeres con edades que oscilan entre 19 y 43 años que presentaron sintomatología por consumo de cocaína. Siete pacientes presentaron convulsiones y en uno de ellos se asociaron ausencias. Cuatro casos sufrieron accidentes cerebrovasculares, 2 de ellos isquémico y los otros hemorrágico, secundario a ruptura de malformaciones vasculares. Un paciente presentó fallas mnésicas severas y otro crisis distónicas paroxísticas. Todo menos uno consumían la cocaína por vía nasal y salvo 2 pacientes, el resto utilizaba otras drogas. El consumo concomitante de etanol era una práctica frecuente. La discontinuidad en el consumo del acaloide provó mejoría en las crisis epilépicas durante el período de seguimiento, salvo en el paciente con ausencias. El paciente con fallas mnésicas evidenció una marcada mejoría al cabo de 4 años de suspendida la cocaína. La paciente con crisis distónicas paroxísticas, al cabo de 2 años de haber suprimido la droga, persistía con el cuadro distónico. En 11 casos hubo una relación temporal entre el consumo de cocaína y la sintomatología neurológica, mientras que en los 2 restantes se debería al consumo crónico. Esto se debe a cambios farmacológicos y eventualmente estructurales en el sistema nervioso central. La frecuencia de estos casos, otrora excepcionales en nuestro medio, ha aumentado sensiblemente, obligando a considerar el consumo de este alcaloide en el enfoque diagnóstico de pacientes jóvenes con eventos neurológicos

Distonías craneales: perfil clínico y neurofarmacológico en 123 casos / Cranial dystonias: clinical and neuropharmacological profile in 123 cases

El blefaroespasmo aislado (BE) y/o asociado a distonía oromadibular (DOM) constituye una de las formas más frecuentes de distonía focal/segmentaria de inicio en la edad adulta. En los últimos 10 años evaluamos 123 pacientes con BE y BE + DOM. Observamos una predominancia de 3 a 1 en mujeres sobre varones, con una edad promedio de inicio de los síntomas de 52 años. Si bien algunos pacientes presentaron un comienzo unilateral, todos evolucionaron en forma bilateral. La remisión espontánea de los síntomas ocurrió en sólo 3 pacientes. El error diagnóstico más frecuente consistió en interpretar este cuadro como psicógeno u ocular. Los estudios por imágenes (TC y MRI) demostraron anormalidades en 12 casos que correspondieron a lesiones en ganglios basales en 6 de los mismos. En nuestra serie, 4 pacientes presentaron historia familiar de distonía craneal. El tratamiento de elección fue la administración local por vía subcutánea de toxina botulínica. Estos hallazgos son homologables a otras series internacionales. Creemos que el reconocimiento clínico-epidemiológico de esta afección, a menudo incapacitante, facilitará un mejor y más rápido diagnóstico y ofrecerá una posibilidad terapéutica sencilla que demuestra un alto grado de seguridad y eficacia

Complicaciones neurológicas por abuso de cocáina. / [Neurologic complications by cocaine abuse]

Argentina is facing an increase in cocaine use by adolescents and young adults from every socioeconomic background. It is calculated that up to 10

of all cocaine passing through this country is locally sold and consumed. Nevertheless, local information describing common cocaine-related neurological events is scarce. From August 1988 to March 1993, 13 patients were evaluated with neurological disease associated with cocaine abuse. Among these 13 patients (Table 1), the mean age was 29; 70

were men. Patients most commonly used the nasal route (snorting). Concomitant abuse of other intoxicants, especially alcohol, was frequent (85

). The major neurological complications included one or more seizures (n = 7), ischemic stroke (n = 2) (Fig. 1-2), hemorrhagic stroke (n = 2) associated with arteriovenous malformation (Fig. 3a-b), memory disturbances (n = 1) and paroxysmal dystonia (n = 1). Psychiatric complaints were present in all patients. Mortality was not observed. There was no correlation between the appearance of complications and the amount of cocaine used, or prior experience with this drug. Only one of the 7 patients with seizures had a previous history of seizures. All had generalized tonic-clonic seizures, and one had concomitant absence episodes. Cocaine modulates central neurotransmitters and has direct cerebrovascular effects. The neurological complications appear to be related to cocaine hyperadrenergic effects, striatal dopaminergic receptor hypersensitivity and perhaps vasculitis. Structural changes in the brain of long-term cocaine abusers could explain the persistence of neurologic symptoms after drug withdrawl.

Complicaciones neurológicas por abuso de cocaína / Neurologic complications by cocaine abuse

El consumo de cocaina ha adquirido proporciones epidemiológicas en los EEUU. Actualmente en nuestro país, sitio de paso de la droga, un 10 por ciento es retenida para su consumo, calculandose que un 80 por ciento de los drogadictos utilizan este alcaloide. Presentamos 13 paciente, 9 hombres y 4 mujeres con edades que oscilan entre 19 y 43 años que presentaron sintomatología por consumo de cocaína. Siete pacientes presentaron convulsiones y en uno de ellos se asociaron ausencias. Cuatro casos sufrieron accidentes cerebrovasculares, 2 de ellos isquémico y los otros hemorrágico, secundario a ruptura de malformaciones vasculares. Un paciente presentó fallas mnésicas severas y otro crisis distónicas paroxísticas. Todo menos uno consumían la cocaína por vía nasal y salvo 2 pacientes, el resto utilizaba otras drogas. El consumo concomitante de etanol era una práctica frecuente. La discontinuidad en el consumo del acaloide provó mejoría en las crisis epilépicas durante el período de seguimiento, salvo en el paciente con ausencias. El paciente con fallas mnésicas evidenció una marcada mejoría al cabo de 4 años de suspendida la cocaína. La paciente con crisis distónicas paroxísticas, al cabo de 2 años de haber suprimido la droga, persistía con el cuadro distónico. En 11 casos hubo una relación temporal entre el consumo de cocaína y la sintomatología neurológica, mientras que en los 2 restantes se debería al consumo crónico. Esto se debe a cambios farmacológicos y eventualmente estructurales en el sistema nervioso central. La frecuencia de estos casos, otrora excepcionales en nuestro medio, ha aumentado sensiblemente, obligando a considerar el consumo de este alcaloide en el enfoque diagnóstico de pacientes jóvenes con eventos neurológicos (AU)

Distonías craneales: perfil clínico y neurofarmacológico en 123 casos / Cranial dystonias: clinical and neuropharmacological profile in 123 cases

El blefaroespasmo aislado (BE) y/o asociado a distonía oromadibular (DOM) constituye una de las formas más frecuentes de distonía focal/segmentaria de inicio en la edad adulta. En los últimos 10 años evaluamos 123 pacientes con BE y BE + DOM. Observamos una predominancia de 3 a 1 en mujeres sobre varones, con una edad promedio de inicio de los síntomas de 52 años. Si bien algunos pacientes presentaron un comienzo unilateral, todos evolucionaron en forma bilateral. La remisión espontánea de los síntomas ocurrió en sólo 3 pacientes. El error diagnóstico más frecuente consistió en interpretar este cuadro como psicógeno u ocular. Los estudios por imágenes (TC y MRI) demostraron anormalidades en 12 casos que correspondieron a lesiones en ganglios basales en 6 de los mismos. En nuestra serie, 4 pacientes presentaron historia familiar de distonía craneal. El tratamiento de elección fue la administración local por vía subcutánea de toxina botulínica. Estos hallazgos son homologables a otras series internacionales. Creemos que el reconocimiento clínico-epidemiológico de esta afección, a menudo incapacitante, facilitará un mejor y más rápido diagnóstico y ofrecerá una posibilidad terapéutica sencilla que demuestra un alto grado de seguridad y eficacia (AU)

Bruxism secondary to chronic antidopaminergic drug exposure.

Eight cases of diurnal bruxism (DB) secondary to long-term antidopaminergic drug exposure are reported. Five exhibited a grinding pattern, one a clenching form, and two a mixed type. An odontological etiology was absent throughout. EMG recordings disclosed two distinct patterns of muscle activity, one with brief rhythmic, forceful contractions and the other featuring sustained prolonged contractions. Surface EMG and EEG monitoring during a 24-h period confirmed the absence of bruxism during sleep. Several drug trials failed to provide relief. Our findings support DB as a focal tardive dystonia syndrome.

Chronic Histoplasma capsulatum infection of the central nervous system successfully treated with fluconazole.

A 48-year-old man with a long-standing history of communicating hydrocephalus is reported. Ventriculoperitoneal shunting led to clinical improvement, but symptoms recurred despite surgical re-exploration switching the shunt to an atrial drainage. Ten months after the last surgical procedure, an acute myelopathy developed. Concomitant pharyngeal granuloma examination identified Histoplasma capsulatum (Hc) yeasts. Despite initial response to amphotericin B, Hc was isolated from cerebrospinal fluid (CSF), valve reservoir and distal catheter after two courses of therapy. Fluconazole successfully sterilized CSF, but transverse myelopathy persisted unchanged, and shunting was needed to control hydrocephalus.

[A new levodopa benserazide preparation for Parkinson's disease with motor fluctuations refractory to standard L-dopa]. / Un nuevo preparado de levodopa benserazida en parkinsonianos con fluctuaciones motoras refractarias a tratamientos con L-dopa standard.

As Parkinson's disease worsens, many patients develop motor fluctuations which usually correlate directly or indirectly with L-dopa plasma levels. A new L-dopa-benserazide HBS preparation (Madopar) a control release pharmaceutical formulation which is activated when it contacts gastric fluid thus providing more stable L-dopa plasma levels, was assayed. Ten patients with a diagnosis of idiopathic Parkinson's disease and motor fluctuations otherwise unresponsive to conventional therapy were selected. The average age was 62 years and the duration of the disease 9 years. The motor status was evaluated on an hourly basis with the King's College Parkinson's disease rating scale; in addition, a nocturnal disability scale (Lees) was used. Out of the 10 patients, 2 dropped out within the first month due to worsening of parkinsonian signs, while 7 of the remainders preferred HBS preparation to the previous treatment. The number of off hours in this group was reduced by 58% and motor fluctuation became less severe. In only 3 cases was it possible to use HBS as monotherapy while in the rest standard L-dopa had to be added, specially as morning doses. The average L-dopa daily dose was increased by 36%. Unwanted effects included psychiatric disturbances in two (in one L-dopa dose had to be reduced) and epigastralgia in one. Our findings suggest that this L-dopa-benserazide control release may be considered an able therapeutic formulation in the control of motor fluctuations in Parkinson's disease.