[Tethered cord syndrome in the adult]. / Síndrome de médula anclada en el adulto.

INTRODUCTION: The tethered cord syndrome (TCS) is a congenital malformation with a pathologic fixation of the spinal cord in the spinal canal. It presents clinically as musculoskeletal, cutaneous, urological and neurological manifestations. The diagnosis is based on the clinical manifestations and on the MRI (Magnetic Resonance Imaging) of the lumbar spine. It is usually diagnosed in childhood, but the symptoms can appear in adult life. METHOD: We reviewed all the cases of TCS in the adult diagnosed in our hospital between 1998 and 2005. The following parameters were evaluated: mean age at onset, initial symptoms, signs, MRI findings and outcome. RESULTS: Four 22 to 72 year old patients were diagnosed. The age at onset varied from 16 to 52 years old and the diagnosis took between 2 and 20 years to be established. The most frequent initial symptoms were the muscular atrophy and the motor weakness in the lower extremities. Two patients exhibited cutaneous stigmata (one had hypertrichosis and the other one a lipoma in the sacrum area) and one a partial agenesis of the sacrum. The most frequent MRI finding was a low lying cord with a lipoma in the sacrum area. In three patients the cord was detethered surgically, but only two of them improved. CONCLUSIONS: The TCS is an uncommon disease in adult, which is usually diagnosed very late in the adult. Because of its insidious and non specific symptomatology, and of its potential surgical treatment, it should be considered in the differential diagnosis of medullar syndromes and polyneuropathies.

Síndrome de médula anclada en el adulto / Tethered cord syndrome in the adult

Introducción. El síndrome de médula anclada (SMA) es una malformación congénita en la que se produce una fijación de la médula dentro del canal espinal. Clínicamente se caracteriza por afectación musculoesquelética, cutánea, neurológica y urológica. El diagnóstico se basa en la clínica y en la resonancia magnética (RM) lumbar. Suele diagnosticarse en la infancia, aunque algunos pacientes inician los síntomas en la edad adulta. Método. Hemos revisado todos los casos de SMA en el adulto diagnosticados en nuestro hospital entre 1998 y 2005. Estudiamos la edad media de presentación, los síntomas iniciales, la exploración física, los hallazgos radiológicos y la evolución. Resultados. Cuatro pacientes, entre 22 y 72 años, fueron diagnosticados de SMA. La edad de inicio de los síntomas variaba entre 16 y 52 años y el diagnóstico se demoró entre 2 y 20 años. Las manifestaciones iniciales más frecuentes fueron la atrofia muscular y la pérdida de fuerza en miembros inferiores. Dos pacientes asociaban estigmas cutáneos (uno hipertricosis sacra y otro lipoma sacro) y uno agenesia atípica del sacro. En la RM el hallazgo más frecuente fue la presencia de un cono medular descendido y de un lipoma en el canal sacro. Tres pacientes fueron intervenidos, con mejoría posterior en dos de ellos. Conclusiones. El SMA es una enfermedad poco frecuente y de diagnóstico muy tardío en el adulto. Por su sintomatología insidiosa e inespecífica hay que considerarlo en el diagnóstico diferencial de los síndromes medulares y de las polineuropatías dada su potencial reparación quirúrgica

Introduction. The tethered cord syndrome (TCS) is a congenital malformation with a pathologic fixation of the spinal cord in the spinal canal. It presents clinically as musculoskeletal, cutaneous, urological and neurological manifestations. The diagnosis is based on the clinical manifestations and on the MRI (Magnetic Resonance Imaging) of the lumbar spine. It is usually diagnosed in childhood, but the symptoms can appear in adult life. Method. We reviewed all the cases of TCS in the adult diagnosed in our hospital between 1998 and 2005. The following parameters were evaluated: mean age at onset, initial symptoms, signs, MRI findings and outcome. Results. Four 22 to 72 year old patients were diagnosed. The age at onset varied from 16 to 52 years old and the diagnosis took between 2 and 20 years to be established. The most frequent initial symptoms were the muscular atrophy and the motor weakness in the lower extremities. Two patients exhibited cutaneous stigmata (one had hypertrichosis and the other one a lipoma in the sacrum area) and one a partial agenesis of the sacrum. The most frequent MRI finding was a low lying cord with a lipoma in the sacrum area. In three patients the cord was detethered surgically, but only two of them improved. Conclusions. The TCS is an uncommon disease in adult, which is usually diagnosed very late in the adult. Because of its insidious and non specific symptomatology, and of its potential surgical treatment, it should be considered in the differential diagnosis of medullar syndromes and polyneuropathies

Safety and efficacy of intrathecal baclofen infusion by implantable pump for the treatment of severe spinal spasticity: a spanish multicenter study.

Objective. To assess long-term efficacy, safety and functional benefit of intrathecal baclofen for severe spinal spasticity. Materials and Methods. This prospective multicenter study was performed in two stages: the first one consisted of an intrathecal bolus injection of baclofen, and the second of a continuous intrathecal baclofen infusion by means of an implantable pump. The sample consisted of 72 adult patients with severe spinal spasticity. Sixty-four were implanted and followed for 36 months. Muscular tone, spasms, and functional scales were evaluated before and periodically after administration of the drug, with a follow-up period of 36 months. Results. A very significant decrease in tone and spasms was observed in all cases (p < 0.001). Tolerance appeared during the first 12 months, increasing doses from a mean initial dose of 83.2 µg (range 25-200 µg) to a mean final dose of 270 µg (range 25-800 µg). Later on, efficacy remained stable, except in cases of mechanical problems of the infusion system.

[Spinal cord compression secondary to epidural extramedullary hematopoiesis in thalassemia: a clinical case and review of literature]. / Compresión medular secundaria a hematopoyesis extramedular epidural en talasemia: caso clínico y revisión de la literatura.

INTRODUCTION: Extramedullary hematopoiesis generally occurs in a variety of hematological disorders where the normal functioning of the blood forming organs is disturbed. It is a common manifestation in thalassemia where it occurs as a compensatory phenomenon in order to combat long standing anemia. Spinal cord compression as a consequence of extramedullary hematopoiesis in the intraspinal epidural space is an extremely rare complication, though this complication has been reported more commonly in thalassemia. CLINICAL CASE: A case of spinal cord compression due to extramedullary hematopoiesis in a patient with thalassemia is reported. The patient was successfully treated with radiation therapy and blood transfusions and he made a complete clinical recovery. Development. The literature is reviewed and the efficacy of several treatments such as surgery, radiotherapy and blood transfusion therapy is discussed. Until recently surgical decompression followed by radiation therapy remained the recommended treatment. Hematopoietic tissue is particularly sensitive to the ionizing radiation and low-dose radiotherapy is enough to relieve the spinal cord compression. Blood transfusion therapy may be diagnostically and therapeutically useful in the management of this entity. CONCLUSIONS: A prompt recognition of the syndrome and early treatment with radiotherapy and blood transfusion therapy is recommended to prevent irreversible damage to the spinal cord. Surgery may be only considered in the event of progressive neurological deficit despite of radiotherapy or blood transfusion therapy.

[Bovine cerebral cortex gangliosides in the treatment of radicular lesions (author's transl)]. / Los gangliósidos de corteza cerebral bovina en el tratamiento de las lesiones radiculares.

Bovine cerebral cortex gangliosides were given to 71 patients following surgical intervention for radicular compression of different etiologies. The extract was given for 20-25 days at daily doses of 20 mg for the first 15 days and 10 mg thereafter until completion of treatment. H reflexology was shown to be a good method for evaluating the response to therapy. Most information was gained by the use of the following parameters: AM/AH, AH/IE, and AH/number of stimulation pulses. The results have been compared with those obtained prior to surgical therapy, those collected in a series of 68 normal individuals and, finally, with those found in another series of 71 patients that were treated postoperatively with conventional measures (B vitamins, analgesics, antiinflammatory drugs, corticoids, etc.). The statistical analysis of the final data shows that the reflexologic parameters tested revert to normal quicker in the patients treated with gangliosides than in those not receiving such therapy. The authors consider the use of gangliosides of great interest in the postoperative period after surgical relief of radicular compression.