Late onset multiple sclerosis.

INTRODUCTION: Late onset multiple sclerosis (LOMS) is an unusual entity, poorly characterised and difficult to diagnose. OBJECTIVE: To study a series of patients with LOMS (presentation of the first symptom of disease after the age of 50 years). PATIENTS AND METHODS: In this retrospective study we review demographic characteristics, first onset symptom, diagnostic delay, disability at the time of diagnosis (EDSS), disease course and findings in SCF, VEP and MRI studies. RESULTS: We included 18 patients (12 F and 6M) with LOMS (4.8% of the total). The most frequent first symptoms were motors deficits (33%), multisystem deficits (33%) and cerebellum disorder (16%). Clinical course (all the cases with a minimal follow-up of 5 years after the diagnosis): primary progressive-MS (62%), secondary progressive-MS (22%), relapsing-remitting-MS (16%). The initial EDSS score was higher than 4 points in one third of patients and diagnosis delay was over 5 years in two thirds of cases. The cerebral MRI study was abnormal and compatible with MS in all patients and fulfilled the Barkhof criteria in 12 (67% of cases). Oligoclonal IgG bands were positive in the 64% of patients in the CSF study and VEP were abnormal in the 73%. The most frequent wrong diagnoses were cerebrovascular disorders and spondyloarthritic cervical myelopathy. CONCLUSIONS: LOMS course is often primary, progressive and motor and multisystem symptoms are the most frequent. The diagnosis is usually delayed and when it is made patients have a high disability score. The findings of cerebral and spinal MRI, CSF and VEP studies are of high diagnostic yield. Cerebrovascular disorders and spondyloarthritic cervical myelopathy are the most important entities in the differential diagnosis of LOMS.

[Computerized cerebral tomography in tuberous sclerosis. Study of 3 patients]. / Tomografía computarizada cerebral en la esclerosis tuberosa. Estudio de tres pacientes.

The findings on computerized axial tomography of the brain in three patients with tuberous sclerosis are reported. Numerous intracranial calcified nodules varying in size from a few millimeters to one centimeter, located preferentially below the ependyma of the lateral ventricles and determining filling defects in its lumen were found in all cases. Other calcified lesions were found in a subcortical location. One patient presented areas of low density throughout the hemispheric white matter, and another showed ventricular dilatation of the ventricles and basal cisternas. Computerized axial tomography is useful in the evaluation of patients with tuberous sclerosis because of its early demonstration of the characteristic intracranial lesions and detection of complications such as calcification of basal and cerebellar ganglia, hydrocephalus, and development of gliomas. Low density areas may correspond to foci of demyelinization. Furthermore, computerized axial tomography allows the demonstration of cerebral tuberosities in asymptomatic relatives, thus offsetting the inconveniences of pneumoencephalography.

[Pentoxifylline: is it useful in multiple sclerosis?]. / Pentoxifilina: es útil en la esclerosis múltiple ?

INTRODUCTION: Pentoxifylline (PTX) is a phosphodiesterase inhibitor which has been found in studies in vitro to inhibit the production of Th-1 cytokines. It has been postulated that it might be used as a possible coadjuvant treatment for interferon in patients with multiple sclerosis. This would also reduce the potential side effects of interferon. OBJECTIVE. To show the efficacy of PTX in reducing the side effects of interferon, and in the functional improvement of these patients. PATIENTS AND METHODS: We studied 18 patients with remitting-relapsing multiple sclerosis over a period of 18 months; nine patients were given PTX and interferon 800 mg/day simultaneously, and nine patients were treated with interferon alone. The clinical condition was evaluated every three months using the Expanded Disability Status Scale (EDSS) and the Neurological Score (NRS) scales. RESULTS: We found no statistical improvement in the clinical course of EDSS and NRS in either group of patients after treatment for 18 months. The patients treated with PTX have fewer secondary effects due to interferon (fever and myalgia) during the first three months, but these differences between the groups subsequently disappear. In two patients PTX caused transient gastralgias and nauseas. CONCLUSIONS: PTX may be useful as a coadjuvant drug with interferon during the first three months of treatment since some of the side effects of interferon may thus be reduced. However, there seems no justification for using PTX for a longer period since there is no functional improvement.

[Headache secondary to carbon monoxide poisoning]. / Cefalea secundaria a intoxicación por monóxido de carbono.

INTRODUCTION: Chronic intoxication due to carbon monoxide (CO) is a condition in which the frequency is underestimated since the clinical features are nonspecific. It is important since serious neurological sequelae may result: an extrapyramidal syndrome, dementia or a vegetative state. The diagnosis is established when there are venous blood levels of carboxyhemoglobin greater than 10%. Early treatment with hyperbaric O2 rapidly improves the symptoms and avoids the development of neurological sequelae. CLINICAL CASE: We report the case of a 30 year old woman who was admitted to hospital for investigation into two episodes of loss of consciousness with vomiting and subsequent somnolence not accompanied by other neurological sequelae. For the previous eight months she had had daily pulsatile biparietal headaches, sometimes accompanied by a fainting feeling, nausea and vomiting. During her admission she remained asymptomatic and all neurophysiological, cardiological and neuroimaging studies were normal. The day after medical discharge she returned with the same clinical condition. A woman who lived with her and accompanied her also complained of similar symptoms. Venous gasometry showed raised levels of carboxyhemoglobin in both the patient (30.4%) and her companion (31.2%). Treatment with hyperbaric 100% O2 reverted the symptoms within a few hours. Later studies showed that the domestic gas burner was faulty. CONCLUSIONS: Occult CO intoxication causes headache which often requires differential diagnosis from psychiatric disorders and episodes of migraine. In patients with refractory nonspecific headache, irregular course and systemic symptoms, usually in winter, CO intoxication should be considered to be a possible cause. Diagnosis is based on finding venous blood levels of carboxyhemoglobin of over 10%. Early treatment avoids lesion of the globus pallidus and irreversible extrapyramidal sequelae.

[Familial strio-pallido++-dentate calcification]. / Calcificación estriopalidodentada familiar.

INTRODUCTION: Calcification of the dentate nucleus of the cerebellum may be seen on cerebral CT in 0.3-0.5% of patients with no symptoms or extra-pyramidal signs. Although there are many causes, some cases show a family incidence. Clinical cases. We present two cases, mother and son, in whom the predominant clinical findings were poor language and a bilateral extrapyramidal syndrome. Laboratory tests, hormone and immunological studies were normal. In both patients cerebral CT showed bilateral calcification of the basal nuclei and dentate nuclei of the cerebellum. DISCUSSION: Our patients fulfilled the clinical and neuro-imaging criteria described in familial strio-pallido-dentate calcification. There is no relationship between the duration and intensity of symptoms, and the extension of the calcification. However, all patients with calcification show clinical features. The predominant features are poor language and a bilateral extra-pyramidal syndrome. These observations indicate that in patients with calcification of the basal ganglia and dentate nuclei of the cerebellum it is necessary to study first degree relatives in order to identify the condition.

Recurrent leukoencephalopathy with microhemorrhages: gradient-echo MRI study diagnostic value in CNS primary angiitis.

Diagnosis of primary angiitis of the central nervous system (PACNS) is difficult in relation to variability in its clinical manifestations and absence of specific signs in neuroimaging. A young patient presented with a recurrent encephalopathic clinical course. T2 and fluid-attenuated inversion recovery-magnetic resonance imaging (FLAIR-MRI) showed hyperintense lesions in the cerebral white matter suggesting demyelination. Those lesions decreased or even disappeared after treatment with steroids and immunoglobulins. In echo gradient MRI (T2*-MRI), there were permanent cortical-subcortical petechial hypointense lesions (microhemorrhages). Definite diagnosis was established after cerebral biopsy. Intravenous cyclophosphamide was administrated with no new relapses in more than 18 months of follow-up. In a compatible clinical course, the finding of petechial hemorrhages in T2*-WI could play an important role in early diagnosis of PACNS.

[Widespread brainstem abscess resolved with medical therapy]. / Extenso absceso de tronco resuelto con tratamiento médico.

The brainstem is an uncommon site of a brain abscess. Such lesions, which were invariably fatal, changed with the arrival of computed tomography and magnetic resonance imaging (MRI). These not only helped in the diagnosis but also in treatment management. A 51-year old patient was diagnosed of widespread pontomesencephalic abscess. He was admitted with a clinical picture of dizziness, headache and involvement of multiple cranial nerves with near complete ophthalmoplegia and cerebellar syndrome in the side of the lesion and contralateral hemiplegia. Microbiologic investigations were negative and the medical management decided was broad spectrum antibiotic and periodic MRI controls. The length of the lesion decreased (from 4 cm in diameter to 0.5 cm) with progressive improvement of the neurological deficits. Treatment of large brainstem abscesses includes primary antibiotic therapy combined with stereotaxic drainage, but in individual cases empirical medical therapy can be effective. Sequential MRI examinations are very important for monitoring treatment efficacy.

[Past hepatitis B virus infection and demyelinating multiphasic disease: casual or causal relationship?]. / Infección remota por virus de la hepatitis B y enfermedad desmielinizante multifásica: ¿casualidad o causalidad?

INTRODUCTION: The relationship between hepatitis B virus and hepatitis B vaccine with central nervous system demyelinating diseases is controversial. CASE REPORTS: We describe two male patients, who in their 70's developed recurrent pictures of acute demyelinating diseases. The first one had recurrent acute disseminated encephalomyelitis (diplopia, paraparesis and urinary retention) and the second one recurrent transverse myelitis (paraparesis and urinary retention). RESULTS: The cerebrospinal fluid test showed mononuclear pleocytosis with negative oligoclonal bands in both patients. Visual evocated potentials were normal. Magnetic resonance imaging (T2-WI and FLAIR) showed hyperintense lesions located in the brain and spinal cord in the first case and only in the spinal cord in the second. With negative antigenemia, antibodies against hepatitis B core and hepatitis B surface antigens were positive in both patients. No patient had been vaccinated for nor had suffered symptomatic hepatic disease. In the second patient, there was an almost total remission of the symptoms with periodic treatment with immunoglobulins. CONCLUSIONS: We recommend hepatitis B virus infection investigation in all patients with central nervous system demyelinating disease.

Prevalence of multiple sclerosis in Santiago de Compostela (Galicia, Spain).

INTRODUCTION: Multiple sclerosis (MS) is the most common central nervous system disease in young adults, and one of the leading causes of disability in this age group. OBJECTIVES: To establish the prevalence and incidence of MS in Santiago de Compostela (Spain). Material and methods We performed a prospective, case-finding study on > 90,188 inhabitants, from 1 January 1998 to 15 September 2003, selected as day of prevalence. Sources of information were the University Clinical Hospital of Santiago, the hospitals and private clinics of the city, rehabilitators, pediatricians, ophthalmologists, psychiatrists, neurosurgeons, specialists in internal medicine, primary care physicians, association of patients of MS, social services and nongovernmental and religious associations. Informative days were programmed for the susceptible population, and campaigns were carried out in the media. All patients with Poser diagnostic criteria for MS, on the census of Santiago de Compostela as of 15 September 2003, were included in the study. RESULTS: On the day of prevalence, 71 patients with MS were registered on the census of Santiago de Compostela, therefore, the prevalence was 79 cases per 100,000 inhabitants. The incidence of the disease in the study period was 5.3 cases per 100,000 inhabitants and year. CONCLUSIONS: The prevalence and incidence rates of MS found in Santiago de Compostela are the highest reported, to date, in Spain.

[Brachial plexitis and myelitis and herpes-zoster lumbar plexus disorder in patient treated with infliximab]. / Plexitis lumbar por herpes zoster, mielitis y plexitis braquial en paciente tratada con infliximab.

Infliximab, a chimeric monoclonal antibody, is a TNF-a inhibitor approved for use in refractory rheumatoid arthritis and Crohn s disease. We present the case of a patient affected by severe rheumatoid arthritis who was successfully treated with infliximab. She suffered diverse neurological complications: brachial plexitis, asymptomatic thoracic myelitis with extensive lesions in MRI study, and herpes zoster lumbar plexitis. We review the neurological adverse effects of infliximab (aseptic meningitis, opportunistic germs infections, disseminated herpes zoster) and focus in their potential adverse effect to induce central and peripheral nervous system demyelination.

[Biologic markers in multiple sclerosis (I). Clinical correlations]. / Marcadores biológicos en la esclerosis múltiple (I). Correlaciones clínicas.

Seven biological markers were studied in a population of 236 patients (144 females, 92 males) with multiple sclerosis as was the relation with the different degrees of diagnostic certainty (Rose and Poser scales), the time of evolution and number of bouts of the disease. The IgG concentrations, IgG ratio and the quantification of the intrathecal IgG synthesis by the Tourtellotte formula isolatedly constituted the most sensitive biological markers for the diagnosis of multiple sclerosis. Altogether, the determination of the number of cells, IgG and the IgG ratio achieved diagnostic sensitivity of 94% for defined multiple sclerosis. The IgG ratio was the most closely related biological marker related with the number of bouts and the time of evolution of the disease.

[Biologic markers in multiple sclerosis (and II). Neurophysiologic and imaging correlations]. / Marcadores biológicos en la esclerosis múltiple (y II). Correlaciones neurofisiológicas y de imagen.

The relation between the results of 7 biological markers (cells, total protein, albumin, IgG, IgG ratio, Tibbling ratio, and Tourtellotte's formula) and 4 paraclinical tests (PEV, PEATC, CT and MR) in 236 patients with multiple sclerosis (MS) not selected by the localization of symptoms were studied. One hundred forty-one had clinically defined MS, 22 had defined MS supported by a laboratory and 68 had clinically probable MS. The existence of a relation between PEV and MRI abnormality and the increase in the concentration and the ratios of intrathecal IgG synthesis and the degree of certainty of disease diagnosis was demonstrated. The most sensitive test was MRI (93%) followed by VEP (83%) and BAEP (60%) and the sensitivity of the study with high resolution CT including 59 patients explored by double enhancement and delayed cut off was very low (33%). It was considered that for the lack of a specific diagnostic test the use of biological markers PEV and MR constituted a necessary aid in the diagnosis of MS.

Esclerosis múltiple de comienzo tardío / Late onset multiple sclerosis

Introducción: La esclerosis múltiple de comienzo tardío (EMCT) es una entidad infrecuente y no bien caracterizada, que suele plantear dificultades diagnósticas. Objetivos: Estudio retrospectivo de una serie hospitalaria de EMCT (primer síntoma a partir de los 50 a˜nos). Pacientes y métodos: Se estudiaron factores demográficos, síntomas iniciales, retraso diagnóstico, grado de discapacidad en el momento del diagnóstico, formas clínicas evolutivas y hallazgos en estudios de LCR, PEV y RM. Resultados: Se incluyeron 18 pacientes (12 mujeres y 6 varones) con EMCT (4,8% del total de la serie estudiada). Los síntomas iniciales mas frecuentes fueron déficits motores (33%), de afectación de múltiples sistemas (33%) y cerebelosos (16%). Las formas clínicas evolutivas (todos los casos con un seguimento mínimo de 5 a˜nos tras el diagnóstico) fueron: EM-PP (62%), EM-SP (22%) y EM-RR (16%). La EDSS en el momento del diagnóstico era superior a 4 en un tercio de los pacientes; el retraso diagnóstico superó los 5 a˜nos en dos tercios de los casos. El estudio de RM cerebral resultó anormal y compatible con EM en todos los pacientes y cumplía criterios de Barkhof en 12 (67%). Las BOC resultaron positivas en el 64% de los pacientes en los que fueron determinadas; los PEV estaban alterados en el 73% de los casos estudiados. Los diagnósticos erróneos previos mas frecuentes fueron patología cerebrovascular y mielopatía espondiloartrósica cervical. Conclusiones: La EMCT suele manifestarse con déficits motores o de múltiples sistemas, que progresan desde su inicio; se diagnostica con retraso, cuando hay ya un grado de discapacidad importante. El estudio de RM cerebral y medular, en conjunción con los PEV y BOC en el LCR facilita su diagnóstico. Patología cerebrovascular isquémica y mielopatía cervical son los diagnósticos erróneos más habituales (AU)

Introduction: Late onset multiple sclerosis (LOMS) is an unusual entity, poorly characterised and difficult to diagnose. Objective: To study a series of patients with LOMS (presentation of the first symptom of disease after the age of 50 years). Patients and methods: In this retrospective study we review demographic characteristics, first onset symptom, diagnostic delay, disability at the time of diagnosis (EDSS), disease course and findings in SCF, VEP and MRI studies. Results: We included 18 patients (12 F and 6M) with LOMS (4.8% of the total). The most frequent first symptoms were motors deficits (33%), multisystem deficits (33%) and cerebellum disorder (16%). Clinical course (all the cases with a minimal follow-up of 5 years after the diagnosis): primary progressive-MS (62%), secondary progressive-MS (22%), relapsing-remitting-MS (16%). The initial EDSS score was higher than 4 points in one third of patients and diagnosis delay was over 5 years in two thirds of cases. The cerebral MRI study was abnormal and compatible with MS in all patients and fulfilled the Barkhof criteria in 12 (67% of cases). Oligoclonal IgG bands were positive in the 64% of patients in the CSF study and VEP were abnormal in the 73%. The most frequent wrong diagnoses were cerebrovascular disorders and spondyloarthritic cervical myelopathy. Conclusions: LOMS course is often primary, progressive and motor and multisystem symptoms are the most frequent. The diagnosis is usually delayed and when it is made patients have a high disability score. The findings of cerebral and spinal MRI, CSF and VEP studies are of high diagnostic yield. Cerebrovascular disorders and spondyloarthritic cervical myelopathy are the most important entities in the differential diagnosis of LOMS (AU)