[Seronegative myasthenia gravis associated with other autoimmune diseases]. / Miastenia gravis seronegativa asociada a otras enfermedades autoinmunes.

INTRODUCTION: The presence of muscle-specific receptor tyrosine kinase (MuSK) defines a subset of patients with generalized myasthenia gravis. The diagnosis of this disease, due to its clinical distribution and negativity of the acetylcholine receptor (AchR) antibodies, is mo re complicated, especially when linked to other autoimmune diseases. CASE REPORT: A 41 year woman with 3 month long symptoms consisting in diplopia, dysarthria and gait instability. On examination, only mild paresis of the right external rectus without fatigability. The complementary tests performed were evoked potentials were normal including multimodal except for the brain magnetic resonance imaging that detected six-eight hyperintense periventricular nodular lesions in both semioval centers, with impairment of corona radiata and corpus callosum without contrast uptake. Given these findings, she was diagnosed of demyelinating disease and treated with megadoses of methylprednisolone for 5 days with mild clinical improvement. At one month, her condition deteriorated, presenting dysphagia and respiratory failure. The neurophysiological study was extended, and very pathological Jitter was detected in the frontal muscle. Despite the negative AchR antibodies, treatment was initiated with pyridostigmine bromide with poor response, admission to the intensive care unit and plasmapheresis due to a new respiratory episode being required. The torpid course and positive outcome of the MuSK antibodies have confirmed the diagnosis. CONCLUSIONS: The detection of these antibodies and performance of neurophysiological tests in clinically deficient muscles are required for the diagnosis of these clinical forms, especially when the neuroimaging-based pathological findings do not justify the clinical course of the patient.

[Continuous infusion of baclofen and an antibiotic for treating meningitis related to refilling of an intrathecal infusion pump reservoir]. / Infusión continua de baclofeno con antibiótico para el tratamiento de meningitis asociada a relleno del reservorio de la bomba de perfusión intratecal.

Baclofen via intrathecal infusion pump is a widely used treatment severe spasticity. Complications are rare and usually mild, though they can also be serious. The sudden discontinuation of intrathecal baclofen may have significant adverse effects. We report the case of a 59-year-old man with chronic spasticity and torsion dystonia who developed meningitis due to Staphylococcus epidermidis due to contamination of the intrathecal, infusion pump reservoir during refilling. It was decided to treat the patient by administering vancomycin through the pump, together with the baclofen. We believe that changing the intrathecal perfusion pump is not necessary as the first measure to take in these cases. Combined infusion of baclofen and an antibiotic through the pump makes it possible to maintain antispastic treatment, sterilize the pump reservoir and tubes, and effectively treat infections that develop during use of these systems.

[Reference values for the central motor conduction time and silent period obtained by the transcranial magnetic stimulation]. / Valores de referencia para el tiempo de conducción motora central y período silente obtenidos por estimulación magnética transcraneal.

INTRODUCTION: Normal values of reference to transcranial magnetic stimulation for the motor central conduction time (CCT) and silent period (SP) is recorded in 30 healthy control subjects over abductor pollicis brevis. MATERIAL AND METHODS: We get for the CCT four measurements: two with low intensity of stimulus, 5% plus the motor threshold, with and without facilitation (CCT1 and CCT1 fac.); and two with high intensities of stimulus, elevating the magnetic stimulation intensity to 1.5 times the threshold (CCT2 and CCT2 fac.). RESULTS AND CONCLUSIONS: The mean and standard deviation of each measurement are: CCT1: 9.34 +/- 1.19, CCT1 fac.: 7.12 +/- 1.1. CCT2: 8.84 +/- 1.05 and CCT2 fac.: 6.57 +/- 1.05. Given that the CCT and SP doesn't follow a normal distribution, the medium and the 5-95% percentiles for the normal values of reference are calculated; there are: CCT1: 7.15-11.32, CCT1 fac: 5.27-9.42. CCT2: 7.05-10.73 and CCT2 fac: 4.91-9.14. For the silent period gets only one measurement employing high intensities. These last measurement were recorded in two localizations: on vertex and on motor area, selecting the greater duration. Given the great individual variability of this period in normal population absolute and ratio for the difference duration of SP between both sides are calculated. The latency of the SP is 50.2 +/- 5.99, 95 percentiles 39.1-64.63, the duration 151 +/- 32.51, 95 percentiles 102.63-239.55. The total SP measured from the discharge of the stimulus to the end of the silent period is 201.71 +/- 33.27; 95 percentiles: 151.39-296.4. The comparison of both hemispheres would give us pathological security for the 99.99% of the population for more than 14.94 ms of absolute difference, and for less of the 79.81% of ratio difference. A summary of the discoveries of the silent period in different pathologies is contributed in the discussion.

[Behçet's syndrome with neurological involvement: value of magnetic resonance]. / Síndrome de Behçet con afectación neurológica: importancia de la resonancia magnética.

Behçet's syndrome is a rare inflammatory, multisystemic disease of unknown etiology and chronic relapsing course, manifested chiefly by ocular and mucocutaneous lesions. Among its neurologic complications, a cerebral vasculitis can occasionally been observed, upon which there is little information at present. A 31-year-old man presented with fever, weight loss, oral and genital ulcers, blurred vision and memory loss during the last two months. The cerebrospinal fluid contained 170 lymphocytes/microliters. Computed Tomography and cerebral angiography were normal. Magnetic resonance imaging (MRI) showed small hyperintense lesions in the corticomedullary and periventricular regions. The patient's condition improved with corticosteroids. We stress upon the importance of MRI to detect small-vessel vasculitis in Behçet's disease.

[Meningitis caused by Candida albicans in a male patient infected by HIV and failure of treatment with amphotericin B]. / Meningitis por Candida albicans en paciente varón infectado por el VIH y fallo al tratamiento con anfotericina-B.

We report a case of Candida albicans meningitis in a male with human immunodeficiency virus (HIV) infection. This finding has seldom been reported, both in this group of patients and in those with other causes of immunosuppression or other underlying diseases. We discuss the clinical presentation and the features of cerebrospinal fluid, which showed only a mild inflammatory reaction as found in other fungal meningitis (basically cryptococcal) in AIDS patients. Finally, we emphasize the ineffectiveness of amphotericin therapy to achieve a complete microbiological cure and to prevent the relapse of meningitis in this patient. We also stress the need to make an early diagnosis in cases of fungal meningitis in patients with VIH infection, so that appropriate therapy is begun as soon as possible.

[Spinal cord involvement by the human immunodeficiency virus. Vacuolar myelopathy]. / Afección medular por el virus de la inmunodeficiencia humana. Mielopatía vacuolar.

Vacuolar myelopathy is the most frequent spinal syndrome in patients infected with the human immunodeficiency virus, presently considered to be related to a direct action of the virus. The authors review the historical, clinical, pathological and etiopathogenetic aspects of this new entity, stressing the difficulties posed by its differential diagnosis and its scarce therapeutic possibilities.

[Neurophysiologic study in patients with human immunodeficiency virus infection]. / Estudio neurofisiológico en pacientes con infección por el virus de la inmunodeficiencia humana.

Peripheral nervous system (PNS) involvement in the form of different neuropathies in human immunodeficiency virus (HIV) infection is increasingly more common. We present a series of 20 HIV + patients subjected to neurophysiological study. The most commonly observed neurological disorder was a slight axonal-type sensitivo-distal polyneuropathy that predominantly affected the lower limbs during the early stages of infection. As HIV infection evolves, the polyneuropathy worsens and becomes a sensitivo-motor polyneuropathy with a more important axonal component. Secondary demyelinization is observed, with additional involvement of the upper limbs in the final stages of infection. The most frequent neurophysiological finding was a decrease in somatosensory evoked potential (SEP) and conduction velocity (CV) of the sural nerve.