[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.

[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)

[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

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.

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

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.

Treatment of Tourette's syndrome with calcium antagonists.

Six males and one female with chronic tic disorders, whose ages ranged from 12 to 31 years, were evaluated before treatment, after 1 month on placebo, after a single 10 mg nifedipine dose (three patients), and monthly while on flunarizine 10-15 mg (mean dose of 13 mg). None of the patients receiving nifedipine improved, but treatment with flunarizine significantly decreased both motor and phonic tic severity and frequency in all but one patient. Side effects included mild transient headaches in one patient, depression in one, and bradykinesia in two. Although a double-blind study is essential to validate our findings, results suggest that flunarizine is a useful drug in the treatment of Gilles de la Tourette syndrome.

[Multifocal demyelinating neuropathy after tetanus vaccine]. / Neuropatía desmielinizante multifocal siguiendo vacunación antitetánica.

A 39 year old man presenting multifocal demyelinating neuropathy (MFDN) is reported. Fifteen days before onset he had been vaccinated with 75 IU of tetanus toxoid. Although recent histories of vaccination or viral infection support the immunological hypothesis proposed for chronic acquired demyelinating neuropathies (CADN) it is reported for the first time in MFDN, suggesting that MFDN and CADN may share common pathophysiological mechanisms.

[Treatment of blepharospasm with botulinum toxin]. / Tratamiento del blefaroespasmo con toxina botulínica.

Blepharospasm is a relatively frequent cranial dystonia which may be seen either alone or related to orofacial-mandibular dystonia (Meige's syndrome). In its maximum degree it can cause functional blindness.Twelve patients with blepharospasm (4 essential and 8 Meige's syndrome) who had been previously treated unsuccessfully with drugs (trihexyphenidyl, biperiden, carbamazepine, lithium, baclofen, lisuride, imipramine, clonazepam and butyrophenones) were treated for 12 months with periocular injections of botulinum toxin (BOTOX). A "low" dose of 12,5 U per eye was employed. With this dose, eleven out of twelve patients experienced significant improvement which lasted from five to fifteen weeks. The only nonresponder obtained complete relief upon duplicating the dose. The only side effect was uni or bilateral ptosis in six patients which improved completely in seven to twenty one days. One patient developed a peripheral facial palsy with complete remission in nineteen days. No systemic side effects were noted. There was only one desertion from this study due to depression enhanced by prolonged (21 days) ptosis. All patients (including the deserter) agreed that treatment with BOTOX provided more relief than any other previous therapeutic method. Our results confirm those obtained by others but a more prolonged study is needed to better evaluate long term effects.

Neuropatía desmielinizante multifocal siguiendo vacunación antitetánica / Multifocal demyelinating neuropathy after tetanus vaccine

Se comunica el caso de un varón de 39 años de edad presentando un cuadro típico de neuropatía desmielinizante multifocal (NDM). Quince días antes del comienzo habia sido vacunado con 75 UI de toxoide tetánico. Aunque la historia reciente de vacunación o infección viral apoya la hipótesis inmunológica de las neuropatías desmielinizantes adquiridas crónicas (NDAC), se comunica por primera vez en la NDM. Nuestro hallazgos sugieren que la NDM y las NDACs compartirían un mismo mecanismo fisiopatológico

Tratamiento del blefaroespasmo con toxina botulínica / Treatment of blepharospam with botulinum toxin

El blefaroespasmo, ya sea aislado o en el contexto de una disquinesia oro-facio-mandibular (síndrome de Meige) es una distonía cranial relativamente frecuente. En su máxima expresión puede dar origen a marcada minusvalía e incluso ceguera funcional. Doce pacientes con respuesta poco satisfactoria a tratamientos medicamentosos (trihexifenidilo, hiperideno, imipramina, carbamazepina, baclofén, litio, lisuride, clonazepam, butirofenonas) fueron tratados con inyecciones perioculares de toxina botulínica (Botox), utilizando una dosis "baja" de 12,5 UI por ojo. Once de los doce pacientes obtuvieron mejoria significativa que duró entre cinco y quince semanas. Una sola paciente no respondió y lo hizo al duplicar la dosis de toxina inyectada. Los únicos efectos secundarios observados fueron ptosis uni o bilateral en 6 pacientes, reversible antes de los 21 días de la inyección y no se observaron efectos secundarios sistemáticos. Una paciente tuvo una parálisis facial periférica de 19 días de duración con remisión completa. Hubo una sola deserción del estudio en una paciente depresiva con ptosis prolongada (21 días). Todos los pacientes (inclusive la desertora) coincidieron en que el tratamiento con Botox fue más eficaz que cualquier ensayo medicamentoso previo. A pesar que estos resultados son similares a comunicaciones previas, creemos aconsejable acumular experiencia para evaluar los resultados a largo plazo

Estimulación dopaminérgica continua en la enfermedad de Parkinson. Estudio a largo plazo / Steady dopaminergic stimulation in parkinson disease. A long term study

Cinco pacientes (2 hombres y 3 mujeres) con enfermedad de parkinson idiopático (PI) y severas fluctuaciones motoras refractarias a L_Dopa y agonistas dopaminérgicos orales (Bromocriptina o lisuride) fueron seleccionados para este estudio. Sus edades oscilaron entre 56 y 75 años (X 62,6) y la duración de los síntomas variaron entre 7 y 18 años. Cuatro casos presentaban PI con estadio IV según Hoehn y Yahr, y uno se hallaba en el estadio III, presentando todos ellos fluctuaciones predecibles. El estado clínico fue evaluado empleando la escala para enfermedad de parkinson del king's college, las diskinesias fueron valoradas de acuerdo al grado de interferencia con movimientos voluntarios, su distribución, tipo y relación con la administración de L-Dopa. La administración de L-Dopa fue mantenida constante (300-875 mg/d) durante el estudio. La infusión dubcutánea de lisuride (ISL) fue realizada empleando bombas portátiles TECENSA AIP-62 (Madrid-España) o una BETATRON II CPI lilly (USA) en dosis gradualmente crecientes hasta estabilizarlas entre 41 ug/h y 104 ug/h. Todos los pacientes mejoraron marcadamente en las primeras semanas manteniéndose este beneficio durante 12 meses. Las diskinesias bifásicas y distonías del período off respondieron cediendo en ISL. Los efectos colaterales fueron fundamentalmente psiquiátricos en 2 casos, lo que motivó en uno la supresión de ISL y en otro la reducción de la dosis (con regímenes de infusión menores durante la noche). En todos los casos se observaron nódulos en el sitio de infusión los que en 2 pacientes tuvieron características hemorrágicas, en un caso se produjo infección de un nódulo. Nuestros resultados sugieren que la ISL puede constituir un valioso suplemento terapéutico de la L-Dopa en PI con fluctuaciones motoras incapacitantes, desafortunadamente efectos colaterales locales y psiquiátricos pueden limitar su aplicación sobre todo para su empleo prolongado.

[Clinical and neurophysiologic tests in the normal elderly]. / Examen clínico y neurofisiológico en gerontes normales.

Clinical and neurophysiological examination was performed in 35 normal aged subjects (72 +/- 6.4; range 65-86 years). Seventeen showed abnormal ankle reflex (48.6%), one case had patellar areflexia as well (2.9%), six had malleolar appalesthesia (17%), and five revealed both areflexia and apallesthesia (14%). Electromyography and motor conduction were normal in every case. Sural nerve conduction velocity and the amplitude of the sensory evoked potential showed no differences between subjects with and without clinical abnormalities. It is concluded that a) reflex and vibration sensibility changes in the elderly are not consequence of peripheral nerve involvement and b) there is no reason to believe there is an "aging neuropathy".

Unusual manifestations of basilar artery ectasia.

Three patients with unusual presentations of basilar artery ectasia are described: subarachnoid hemorrhage was manifest in one and autonomic dysfunction developed in the other two. Aneurysmal rupture was the cause in the first patient, while compression of the brainstem and/or of the baroreceptor afferences of the IXth and Xth cranial nerves is postulated to be responsible for the symptoms found in the other two. Basilar artery aneurysms should be considered in cases with subarachnoid hemorrhage or autonomic dysfunction, particularly when diagnostic procedures fail to disclose other possible etiologies.