Cluster-tic syndrome as the initial manifestation of multiple sclerosis.

We report the case of a patient diagnosed as having cluster-tic syndrome as the initial manifestation of multiple sclerosis (MS). The patient's headache bouts improved after treatment with antiepileptic drugs, steroids, and beta-interferon. Magnetic resonance imaging (MRI) scans showed a pontine demyelinating lesion involving the area of the trigeminal root inlet and main sensory nucleus. Neurophysiological studies correlated well with MRI lesions. The association between cluster-tic syndrome and MS is an exception, and the mechanism of the pain is still unknown; therefore, this case might suggest a pathophysiological relationship between the trigeminal main sensory nucleus and cluster-tic syndrome.

[The contribution made by neurophysiological and ultrasound imaging studies to the diagnosis of carpal tunnel syndrome. Surgical correlation]. / Aportación de los estudios neurofisiológico y ecográfico al diagnóstico del síndrome del túnel carpiano. Correlación quirúrgica.

INTRODUCTION: Electrophysiological study has been for long time the elected approach for the diagnosis and clinical evaluation of carpal tunnel syndrome (CTS). More recently, echography and other imaging techniques have been introduced in current medicine for their potential in the anatomical evaluation of the neural compression. To asses the usefulness of both diagnostic procedures we have compared the findings obtained by electrophysiological and echographic approaches in a group of 60 CTS patients with different degrees of the disease. PATIENTS AND METHODS: In all patients the conduction velocity was evaluated in the median and cubital nerves using surface electrodes. For echography lineal transductors of 5-10 Hz and 5-12.5 MHz were employed. RESULTS: The patients were distributed for each test on a scale depending of the severity of the alterations detected by the corresponding technique and both files were subsequently compared by regression analysis, Pearson test and paired-test. No correlation was detected in any of the statistical test. CONCLUSIONS: The lack of correlation between the results of both proofs emphasizes the usefulness of the two diagnostic approaches in CTS. While electrophysiological study provides information about nerve function, ecography unravels the morphological alterations accounting for the syndrome, therefore being non-excluding complementary approaches.

Trigeminal somatosensory evoked potentials in multiple sclerosis: a case report.

Somatosensory evoked potentials elicited by trigeminal nerve stimulation (TSEPs) allow the study of the trigeminal system, its peripheral to its central portion. As a routine clinical testing procedure, however, this method has not yet received greater attention and still has to wait to become a part of the diagnostic workup in multiple sclerosis (MS). A patient diagnosed as MS was presented with selective involvement of the left trigeminal nerve. Neurophysiologically, the most prominent and persistent abnormalities were found in the TSEPs. The presumed diagnosis of MS was subsequently confirmed by MRI showing evidence of demyelinating disease. There has been a scarcity of references on TSEP. Our study emphasizes its usefulness and a plea is being made for the inclusion of TSEP in protocols for the diagnostic workup in patients with MS.

Friedreich ataxia with minimal GAA expansion presenting as adult-onset spastic ataxia.

Around a quarter of Friedreich ataxia (FA) patients, despite being homozygous for GAA expansion within the FRDA gene, show atypical presentations. Our aim is to describe the case of three brothers with long-term follow-up suffering from late onset FA manifested with spastic ataxia. The three patients belong to a family with occipital dysplasia (OD) and Chiari I malformation previously reported by us. We have carried out serial examinations since 1977. Electrophysiological and neuroimaging studies, and molecular genetic analyses of hereditary ataxias are available in all three patients. Onset of symptoms occurred between 25 and 35 years. The clinical picture consisted of progressive spastic gait, truncal and limb ataxia, dysarthria, nystagmus, hyperreflexia with knee and ankle clonus and extensor plantar response, and mild hypopallesthesia. Ages at present vary between 50 and 59. One patient is wheelchair-bound but the other two are able to walk with support. Leaving OD aside, skeletal anomalies are not prominent. All three patients showed cardiomyopathy. MR imaging revealed atrophy of the cerebellum and spinal cord. Motor and sensory nerve conduction velocities were normal. Central conduction time of both motor and sensory pathways was delayed or unobtainable. All three patients were homozygous for the GAA expansion, the smaller expanded allele ranging between 131 and 156 repeats. Four heterozygotic carriers were detected among non-ataxic relatives including one with OD; furthermore, an asymptomatic OD patient showed normal genotype. We conclude that adult onset spastic ataxia is a distinctive FA phenotype associated with minimal GAA expansion. This phenotype represents a new cause of selective distal degeneration of central sensory axons. The present concurrence of OD and FA reflects coincidental cosegregation of two different inherited disorders.

[Isolated posterior cord syndrome in Lyme s disease: a clinico neurophysiological study]. / Síndrome cordonal posterior aislado en la enfermedad de Lyme: estudio cliniconeurofisiológico.

INTRODUCTION: Lyme disease, caused by spirochete Borrelia burgdorferi, is a multisystemic infectious disorder with prominent neurologic involvement, affecting both the peripheral and the central nervous system. Meningitis, cranial neuritis and radiculoneuritis are the usual manifestations in the acute phase, and peripheral neuropathy in the chronic phase. Other less common manifestations have been also described. Here we report one case of Lyme disease confirmed by PCR, with a previously undescribed neurological manifestation, and the neurophysiological studies performed before and after treatment. CLINICAL CASE: Our patient showed a chronic and progressive clinical picture consisting of instability on walking and distal paresthesia of lower limbs, suggestive of posterior column disfunction. The neurophysiological exam performed prior to treatment with ceftriaxone revealed bilateral absence of lower limbs somatosensory evoked potentials (SEPs), without alterations in the distal nervous conduction or in upper limbs SEPs. The exam performed after treatment revealed a partial recovery of lower limb SEP with presence of an evoked response in SEP of left lower limb, coincident with a transitory clinical improvement of paresthesia in the same extremity. CONCLUSIONS: Our findings reveal that posterior column disfunction can be a neurological manifestation of Lyme disease. Furthermore the neurophysiological study shows that this manifestation is partially reversible following treatment. Our study emphasize the importance of the neurophysiological tests for the diagnosis and follow up of neurological manifestations of Lyme disease.

¿Son la ocupación y la educación intercambiables como indicadores sociales en salud comunitaria? Un estudio en población ocupada / ¿Are education and occupation interchangeable as social indicators in community health? a study on employed people

Objetivo. Los estudios epidemiológicos y las intervenciones para reducir las desigualdades en salud en la comunidad requieren la asignación de indicadores socioeconómicos exhaustivos y fáciles de obtener. Dos de los más utilizados son la ocupación y la educación. En este estudio pretendemos evaluar la asociación entre educación y ocupación en población adulta ocupada, por si, en ausencia de una variable, fuera posible utilizar la otra con la menor pérdida posible de información sobre nivel socioeconómico. Diseño. A partir de una muestra representativa (n = 3.091) de población general (18-65 años), tomada de una encuesta de prevalencia sobre factores de riesgo de enfermedades crónicas realizada en la Región de Murcia, se ha realizado un análisis (log)lineal entre educación y ocupación en la población ocupada (65,8 por ciento varones y 34,2 por ciento mujeres de la muestra total). Resultados. Los varones presentan una asociación significativa entre las posiciones directivas y la educación universitaria. La asociación se reduce entre la ocupación en tareas administrativas y la categoría de estudios secundarios, para incrementarse en los trabajadores manuales con estudios primarios o sin estudios. En las mujeres, con un menor número de efectivos, la asociación educación-ocupación describe el mismo patrón, incluso con mayor magnitud en las asociaciones entre las clases directivas y el nivel educativo universitario. En varones y mujeres las mayores asociaciones se encuentran en ambas diagonales de las tablas de educación por ocupación, indicando: a mejor ocupación, mayor educación, y lo contrario. Conclusión. La clasificación de la gente con trabajo de acuerdo con la educación y la ocupación presenta: asociación, consistencia interna y gradiente. En ausencia de información exhaustiva sobre ocupación, la educación puede ser una alternativa como indicador de nivel socioeconómico (AU)

[Electrophysiological study during the initial stage of the Guillain- Barré syndrome]. / Estudio electrofisiológico en la fase inicial del síndrome de Guillain-Barré.

INTRODUCTION: In typical Guillain-Barré syndrome the clinical symptomatology is more pronounced in lower extremities. So, this is the territory of election to demonstrate electrophysiological abnormalities early in the disease. PATIENTS AND METHODS: We presented the results of the electrophysiological study of 37 patients, who showed abnormalities in peroneal nerve territory as soon as 15 days from the onset of the disorder, and were maintained at least up to 30 days later. Since Guillain-Barré syndrome is an heterogeneous syndrome, which can differ in extension and severity in the different patients, is necessary a more extensive electrophysiological study. Furthermore, subtypes of the disorder are recognized in relation to different electrophysiological patterns. Therefore, it is important to analyze data of each patient individually and to make control evolutive studies. For these reasons we also describe in detail the electrophysiological study of a selected patient affected by Guillain-Barré syndrome. In this case motor and sensory abnormalities were localized in distal nerve segments, and there was not denervation potentials in EMG. RESULTS: We considered these findings indicative of a mainly distal sub-pattern with a good prognosis. The occurrence of A-waves was an early finding in this case of Guillain-Barré syndrome. During the course of the disease the behavior of these A-waves was variable in the different nerves of the lower extremities, and they disappeared in parallel with the clinical improvement of the patient.

[Are occupation and education interchangeable as social indicators in community health? A study in an employed population]. / Son la ocupación y la educación intercambiables como indicadores sociales en salud comunitaria? Un estudio en población ocupada.

OBJECTIVE: Epidemiological studies and interventions to reduce inequalities in community health require the assignation of exhaustive and easy-to-obtain social indicators. Occupation and education are two of the most often used. In this study we attempt to evaluate the association between education and occupation among adult working population because if, in the absence of one of these two variables, it will be feasible to use the remaining with the lesser lost of socio-economic information. DESIGN: From a representative sample (n = 3091) of general population (18-65 years old) drawn out from a prevalence survey on chronic disease risk factors performed in the Region of Murcia, a log-lineal analysis has been made between education and occupation among working people (65.8% of males and 34.2% of females from the original sample). RESULTS: Men present significant association between managerial positions and university education. The association drops between clerks and high school graduates to increase among all manual workers with or without primary schooling education. Among women--with a lesser number of participants--the education/occupation association describes the same pattern but with higher magnitudes in the positive associations between managerial positions and university education. For both genders, the greatest associations are found in both diagonals of the education by occupation tables indicating: better occupation, more education, and the opposite. CONCLUSIONS: The classification of working people according to education and occupation presents association, internal consistency and gradient. In absence of comprehensive information regarding occupation, education could be an alternative as socio-economic indicator.

[Miller-Fisher syndrome: electrophysiological serial study of five patients]. / Síndrome de Miller Fisher: estudio electrofisiológico evolutivo en cinco pacientes.

OBJECTIVE: The object of the present study is to show the spectrum of neurophysiological findings during clinical course of Miller Fisher syndrome (MFS), avoiding the controversy over a combined central and peripheral pathology. PATIENTS AND METHODS: We report five patients with a syndrome of ophthalmoplegia, ataxia and areflexia. A similar episode had been suffered previously by 2 of these patients, 14 and 13 years before. In one of them the second episode evolved to a typical Guillain-Barré syndrome (GBS). Motor and sensory conduction velocity of upper and lower limbs, F waves, blink-reflex and somatosensory evoked potentials were recorded in all cases. Needle electromyography in four and brainstem evoked potentials in three of them and the jaw-reflex in another one. RESULTS: In all patients there was a markedly reduced amplitude of the distal sensory evoked response, and no signs of denervation in the EMG. The other results were variable both interindividually and intraindividually. The severity of abnormalities was also different between different patients. The clinical recovery was always accompanied by a improvement of neurophysiological parameters. CONCLUSIONS: The electrophysiological findings in MFS can be variable but they appear always related with the clinical symptomatology. We report for the first time a case in whom the jaw-reflex was abnormal whereas the blink-reflex was normal.

[Factors related with compliance with tuberculosis chemoprophylaxis]. / Factores relacionados con el cumplimiento de la quimioprofilaxis contra la tuberculosis.

OBJECTIVES: To determine therapeutic tolerance and compliance level with antituberculous chemoprophylaxis. PATIENTS AND METHODS: 587 patients were proposed for antituberculous chemoprophylaxis with isoniazid from January 1995 to June 1996 in Murcia (Spain). RESULTS: 3.6% of the patients gave up treatment because of hepatic intolerance and 73% completed the prophylaxis. Non-compliance related factors were: social risk factors (odds ratio [OR] = 9.31), previous history of allergies (OR = 2.87), previous personal history of tuberculosis-related events (OR = 0.49) and interaction between sex and age. CONCLUSIONS: Support and intervention measures directed to risk groups of a social origin deem to be needed. Control measures and information provided to middle-aged males, with particular emphasis to the first weeks of prophylaxis, should also be implemented.

[The contribution of conventional EMG to the diagnosis of amyotrophic lateral sclerosis with bulbar symptoms]. / Aportación del EMG convencional al diagnóstico de la esclerosis lateral amiotrófica con síntomas bulbares.

INTRODUCTION: We presented here the results of quantitative electromyographic (EMG) study from 61 patients affected by Amyotrophic Lateral Sclerosis (ALS) with bulbar symptomatology at the time of study. MATERIAL AND METHODS: Patients were divided into two groups depending on whether the illness started with bulbar manifestations alone or with predominant neuromuscular weakness. RESULTS: Electromyography was performed on the abductor pollicis brevis, deltoideus or biceps brachii and tibialis anterior muscles (target muscles). In the first study the EMG was abnormal in more than 90% of the patients regardless to the duration of the illness and muscular weakness distribution. In the follow-up study the EMG was abnormal in all the patients lacking significant differences between the two groups. CONCLUSIONS: Quantitative EMG appeared as a very useful tool for the early diagnosis of ALS with bulbar manifestations. We found a correlation between the alterations of the different EMG parameters with the natural evolution of the illness.

Ototoxicity of erythromycin in man: electrophysiologic approach.

Ototoxicity is probably the least acknowledged adverse reaction of erythromycin. The mechanism of erythromycin ototoxicity is still unknown. Here we report on two new cases of erythromycin-induced hearing loss. In both of them, serial evoked auditory brainstem potentials (EABPs) were obtained. The recorded EABPs showed absence of waves I to III during treatment with erythromycin, and normalization of all EABP waves after the administration of erythromycin had been stopped. Our findings support the hypothesis that erythromycin-induced hearing loss is attributable to a functional disorder in the peripheral parts of the auditory system.

Brain-stem auditory evoked potentials and blink reflex in Friedreich's ataxia.

The brain-stem involvement in Friedreich's ataxia (FA) was studied by using brain-stem auditory evoked potentials (BAEPs) and the blink reflex. Ten out of 18 patients had abnormal BAEPs, the main abnormality being complete absence of responses and disappearance of wave V. Combined degeneration of the peripheral and central acoustic pathways probably accounts for these findings. The blink reflex was abnormal in 50% of the cases. The outstanding abnormality was bilateral delay of late responses with normal early response, which could be correlated with the known pallor of the descending trigeminal tracts. In contrast with BAEP findings, blink reflex abnormalities did not correlate with either the age of patients or the severity and duration of the disease. These data suggest a difference in susceptibility to degeneration between the auditory system and neuronal system subserving the blink reflex. We conclude that systematic BAEP and blink reflex recording is useful in the electrophysiological evaluation of FA patients.

Spanish toxic oil syndrome neuropathy in three patients with hereditary motor and sensory neuropathy type I.

We describe three patients with hereditary motor and sensory neuropathy type I coming from a family who suffered from Spanish toxic oil syndrome with neuromuscular manifestations. Their clinical course neither differed from other kin only affected with the inherited neuropathy nor from other patients with Spanish toxic oil syndrome studied by us. These findings suggest that patients with hereditary motor and sensory neuropathy do not exhibit a special susceptibility to vasculitic neuropathy associated with Spanish toxic oil syndrome.