RESUMEN
STUDY DESIGN: Prospective cohort study. OBJECTIVE: Although Bracken et al have demonstrated a significant neuroprotective effect of high-dose intravenous (i.v.) methylprednisolone (MP) within 8 h post spinal cord injury (SCI), this practice has recently been challenged. We hypothesized it is possible that acute corticosteroid myopathy (ACM) may occur secondary to the MP. This pilot study was performed to test this hypothesis. SETTING: University of Miami School of Medicine/Jackson Memorial Hospital, Miami VA Medical Center, FL, USA. METHODS: Subjects included five nonpenetrating traumatic SCI patients, who received 24 h MP according to National Acute Spinal Cord Injury Studies (NASCIS) protocol, and three traumatic patients who suffered SCI and did not receive MP. Muscle biopsies and electromyography (EMG) were performed to determine if myopathic changes existed in these patients. RESULTS: Muscle biopsies from the SCI patients who received 24 h of MP showed muscle damage consistent with ACM in four out of five cases. EMG studies demonstrated myopathic changes in the MP-treated patients. In the three patients who had SCI but did not receive MP, muscle biopsies were normal and EMGs did not reveal evidence of myopathy. CONCLUSION: Our data suggest that MP in the dose recommended by the NASCIS may cause ACM. If this is true, part of the improvement of neurological recovery showed in NASCIS may be only a recording of the natural recovery of ACM, instead of any protection that MP offers to the injured spinal cord.
Asunto(s)
Metilprednisolona/efectos adversos , Músculo Esquelético/efectos de los fármacos , Enfermedades Musculares/etiología , Fármacos Neuroprotectores/efectos adversos , Adenosina Trifosfatasas/metabolismo , Adulto , Anciano , Biopsia/métodos , Electromiografía/métodos , Estudios de Seguimiento , Humanos , Masculino , Persona de Mediana Edad , Músculo Esquelético/patología , Músculo Esquelético/fisiopatología , Enfermedades Musculares/patología , Conducción Nerviosa/efectos de los fármacos , Estudios Prospectivos , Traumatismos de la Médula Espinal/tratamiento farmacológico , Traumatismos de la Médula Espinal/patología , Coloración y Etiquetado/métodos , Factores de TiempoRESUMEN
Research criteria for the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were proposed by an Ad Hoc Subcommittee of the American Academy of Neurology (AAN) in 1991, and since then these criteria have been widely used in clinical studies. We have been impressed by the frequent finding of electrophysiological changes of a demyelinating neuropathy in patients whose clinical presentation does not conform to the usually accepted clinical phenotype of CIDP. To determine the clinical spectrum of CIDP, we conducted a retrospective review of patients of the peripheral electrophysiology laboratory of the University of Miami-Jackson Memorial Medical Center. Diagnostic criteria for acquired demyelination of an individual nerve were adapted from the AAN research criteria for the diagnosis of CIDP (1991). Patients were accepted for inclusion when such evidence was demonstrated in at least one motor nerve or at least two sensory nerves. We then reviewed the clinical phenotype and the underlying etiology of the neuropathy in these cases. Eighty-seven patients, 63 male and 24 female, age of onset 4-84 (mean 49.3) years, met these inclusion criteria. Forty-seven patients (54%) had distinct features outside the usual clinical presentation of CIDP. Of these, 15 (17%) had predominantly distal features, 13 (15%) had exclusively sensory polyneuropathy; seven (8%) had markedly asymmetric disease, seven (8%) had associated CNS demyelination, four (5%) had predominant cranial nerve involvement, and one (1%) had only the restless legs syndrome. An associated medical condition that may have been responsible for the acquired demyelinating neuropathy was present in 60% of the patients. We conclude that spectrum of CIDP is broader than would be indicated by the strict application of the AAN research criteria, and that many of the cases meeting more liberal criteria frequently respond to immunosuppressive therapy.
Asunto(s)
Enfermedades Autoinmunes/fisiopatología , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/fisiopatología , Adolescente , Adulto , Edad de Inicio , Anciano , Anciano de 80 o más Años , Enfermedades Autoinmunes/diagnóstico , Enfermedades Autoinmunes/tratamiento farmacológico , Enfermedades Autoinmunes/epidemiología , Enfermedades Autoinmunes/metabolismo , Biopsia , Electroforesis de las Proteínas Sanguíneas , Proteínas del Líquido Cefalorraquídeo/análisis , Niño , Preescolar , Comorbilidad , Nervios Craneales/fisiopatología , Neuropatías Diabéticas/complicaciones , Electrofisiología , Femenino , Florida/epidemiología , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Paraproteinemias/complicaciones , Nervios Periféricos/patología , Nervios Periféricos/fisiopatología , Fenotipo , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/tratamiento farmacológico , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/epidemiología , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/metabolismo , Estudios Retrospectivos , Médula Espinal/fisiopatología , Insuficiencia del TratamientoRESUMEN
Sporadic inclusion body myositis (s-IBM) is characterized by late onset of slowly progressive weakness that involves the quadriceps and volar forearm muscles early in the course of the disease. There are hereditary forms of inclusion body myopathy (h-IBM) that histologically resemble s-IBM. The lack of inflammation on biopsy and the different ages at onset and patterns of muscle weakness distinguish s-IBM from h-IBM. We report twin brothers with the typical clinical and histologic features of s-IBM. The occurrence of s-IBM in these twins suggests the possibility of a genetic susceptibility to developing s-IBM.
Asunto(s)
Miositis por Cuerpos de Inclusión/genética , Gemelos/genética , Edad de Inicio , Biopsia , Humanos , Masculino , Persona de Mediana Edad , Miositis por Cuerpos de Inclusión/patologíaRESUMEN
We describe a two-generation family with combined clinical features of myoclonic epilepsy, progressive external ophthalmoplegia (PEO), proximal myopathy, pigmentary retinopathy, progressive deafness, basal ganglia calcification, and ragged-red fibers in a muscle biopsy specimen. One family member died unexpectedly at age 22 years. The molecular tests revealed an A-to-G transition at nucleotide position 3243 of the mitochondrial tRNA(Leu(UUR)) gene. No one in this family had stroke-like episodes. Although the propositus (a 28-year-old woman) had a significant number of white hairs, the percentage of mutant mtDNA in white-hair roots was not different from that in the colored-hair roots. Our findings suggest that the 3243 mutation can be associated with mixed clinical features of myoclonic epilepsy with ragged-red fibers (MERRF) and PEO and that a preferential increase in the levels of the mutant mtDNA is not related to graying of hair, and hence to the hypothesized production of premature aging of cells.
Asunto(s)
ADN Mitocondrial/genética , Epilepsias Mioclónicas/genética , Síndrome MERRF/genética , Oftalmoplejía/genética , Mutación Puntual , ARN de Transferencia de Leucina/genética , Adulto , Sordera , Femenino , Color del Cabello , Humanos , Síndrome MELAS/genética , Masculino , Persona de Mediana Edad , Fibras Musculares de Contracción Rápida/metabolismo , Fibras Musculares de Contracción Rápida/patología , Músculo Esquelético/metabolismo , Músculo Esquelético/patología , Linaje , Reacción en Cadena de la Polimerasa , Polimorfismo de Longitud del Fragmento de Restricción , Valores de Referencia , SíndromeRESUMEN
OBJECTIVE: We describe the unusual syndrome of cytomegalovirus (CMV) polyradiculomyelitis and its MR findings in two patients with AIDS. MATERIALS AND METHODS: The clinical records and MRI studies of two patients with AIDS and CMV polyradiculomyelitis were reviewed. The MR images were performed on a Picker 1.0 or 1.5 T MR unit. Axial and sagittal T1-weighted images of the lumbar spine were obtained, pre- and post-Gd-DTPA (0.1 mmol/kg) administration. Gradient echo sagittal images were also obtained. RESULTS: Precontrast images demonstrated a thickened cauda equina in both patients. In one patient the conus was ill defined on precontrast images. Post-contrast images demonstrated diffuse enhancement of the cauda equina in both patients as well as enhancement along the surface of the conus. In one patient the nerve roots were clumped and adherent to the walls of the thecal sac as well as to other nerve roots. CONCLUSION: The clinical presentation of urinary retention, flaccid paraparesis, back and/or leg pain, and "saddle anesthesia" in a patient with AIDS should suggest the diagnosis of CMV polyradiculomyelitis. Although diffuse enhancement of the cauda equina on postcontrast MRI is a nonspecific finding, it would strongly support this diagnosis in the appropriate clinical setting. The diagnosis may be easily missed without the use of a contrast agent.
Asunto(s)
Infecciones Oportunistas Relacionadas con el SIDA/patología , Infecciones por Citomegalovirus/patología , Mielitis/patología , Polirradiculoneuropatía/patología , Adulto , Cauda Equina/patología , Citomegalovirus/aislamiento & purificación , Femenino , Humanos , Imagen por Resonancia Magnética , Masculino , Mielitis/microbiología , Polirradiculoneuropatía/microbiologíaAsunto(s)
Síndrome de Inmunodeficiencia Adquirida/complicaciones , Enfermedades Musculares/inducido químicamente , Zidovudina/efectos adversos , Síndrome de Inmunodeficiencia Adquirida/tratamiento farmacológico , Adulto , Humanos , Estudios Longitudinales , Masculino , Enfermedades Musculares/complicaciones , Zidovudina/uso terapéuticoRESUMEN
An examination of the medical and physiological effects of functional electrical stimulation (FES) applied to the quadriceps muscle of five paraplegic male volunteers with complete spinal cord injuries was performed. FES training was provided three days a week over a 12-week period using a progressive resistive exercise protocol. Stimulation was applied through use of a closed-loop microprocessor-based FES system. Prior to the start of and immediately following the 12-week training period, subjects were assessed on several measures, including quadriceps muscle bulk and histochemistry, laboratory studies, echocardiography, and arm ergometry exercise. Results of the study indicated substantial increases in muscle strength and muscle bulk. At the outset of the study one patient suffered a patellar fracture. No significant changes in pretraining and posttraining general examinations, laboratory studies, echocardiography, or arm ergometry exercise testing were noted.
RESUMEN
There is a spectrum of muscular abnormalities that occurs in patients with hypothyroidism. Alterations in deep tendon reflexes are commonly observed although more extensive muscle disease is less frequently seen. Two patients who demonstrated increased muscle mass, muscle stiffness with variable degrees of muscle weakness and low levels of serum thyroxine (Hoffmann's syndrome) are described. At the time of presentation, the serum creatinine phosphokinase level was more than 10 times greater than normal, and electromyography revealed repetitive positive waves. After therapy with thyroid hormone, there was complete resolution of the muscle abnormalities, and laboratory studies were performed. In this report, we review the clinical syndrome of muscle dysfunction that can be seen with the more severe forms of hypothyroid myopathy.