Síndrome de hombre en barril secundario a trauma raquimedular. Reporte de caso / Man-in-the-barrel syndrome secondary to spinal cord trauma. Case report

Resumen Presentamos caso de un varón de 77 años con antecedentes de alcoholismo, limitación crónica al flujo aéreo, y trauma encéfalo craneano (TEC) antiguo, que ingresa por cuadro de insuficiencia respiratoria global con descompensación aparentemente infecciosa que evoluciona tórpidamente con asistencia de ventilación mecánica no invasiva (VMNI) persistente. Se objetiva diparesia de predominio braquial proximal lo que hace sospechar síndrome de hombre en barril (SHB). Se completa estudio con resonancia nuclear magnética (RNM) de columna cervical que muestra severa atrofia medular desde bulbo distal hasta C4. El paciente se mantiene con VMNI c/ BiPAP y apoyo kinésico, con lo que logra progresiva mejoría ventilatoria.

We present a patient 77-year-old male with a history of alcoholism, chronic airflow limitation, and old brain trauma injury, who is admitted due to a global respiratory insufficiency with apparently infectious decompensation that evolves rapidly dependent on non-invasive mechanical ventilation and with a, Man-in-the-barrel syndrome. A magnetic resonance of cervical spine showed severe spinal atrophy from the distal medulla oblongata to C4. The patient remains with kinesic motor support, thereby achieving progressive ventilatory improvement.

Miastenia gravis refractaria con buena respuesta a rituximab: caso clínico / Refractory myasthenia gravis with good response to rituximab: report of one case

Myasthenia gravis (MG) is a heterogeneous disease, and there is no unique therapeutic approach for all patients. In 2013 the Myasthenia Gravis American Foundation (MGFA) panel of experts defined refractory MG as the lack of change or deterioration after the use of corticosteroids and two immunosuppressive agents, in adequate doses and time. We report a 51-years-old female with MG of bulbar predominance, who presented four myasthenic crises in 17 months despite the use of corticosteroids, azathioprine and mycophenolate. The high costs associated with her hospitalizations, as well as severe caloric - protein malnutrition, the need for tracheostomy and gastrostomy support, led us to use rituximab. The patient evolved with an excellent response, free of crises after 30 months. She gained 12 kg of weight, without tracheostomy and gastrostomy, only using pyridostigmine support 4 times a day.

Neuromielitis óptica y lupus eritematoso sistémico en un hombre: caso clínico / Neuromyelitis optica presenting concomitantly with systemic lupus erythematosus: report of one case

Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system, which preferentially attacks the optic nerve and spinal cord. It is associated with antibodies against aquaporin 4. Morbidity and mortality are higher than in multiple sclerosis and its treatment focuses on immunosuppressive drugs. Immunomodulators are contraindicated. We report a previously healthy 35-year-old man, presenting with NMO concomitantly with systemic lupus erythematosus. His evolution was torpid with three outbreaks in the 10 months after the diagnosis, requiring a first-line therapy with methylprednisolone and cyclophosphamide and then a second-line therapy with rituximab.

[Neuromyelitis optica presenting concomitantly with systemic lupus erythematosus: Report of one case]. / Neuromielitis óptica y lupus eritematoso sistémico en un hombre: Caso clínico.

Neuromyelitis optica (NMO) is a severe demyelinating disease of the central nervous system, which preferentially attacks the optic nerve and spinal cord. It is associated with antibodies against aquaporin 4. Morbidity and mortality are higher than in multiple sclerosis and its treatment focuses on immunosuppressive drugs. Immunomodulators are contraindicated. We report a previously healthy 35-year-old man, presenting with NMO concomitantly with systemic lupus erythematosus. His evolution was torpid with three outbreaks in the 10 months after the diagnosis, requiring a first-line therapy with methylprednisolone and cyclophosphamide and then a second-line therapy with rituximab.

Trombosis de seno venoso secundaria a traumatismo encéfalo craneano: caso clínico / Cerebral vein thrombosis secondary to closed head injury: Report of one case

Intracranial sinus thrombosis (1ST) after closed head injury is an uncommon but potentially serious complication. It has no correlation with the severity of the injury. The symptoms and clinical course are highly variable. The most frequent but least specific symptom is severe headache. Cerebral lesions and neurologic signs develop in half of patients with IST. We report a 29 year-old male who had an IST after a severe closed head injury. The patient initially developed headache and had later 2 secondarily generalized seizures. The magnetic resonance imaging showed a superior sagittal sinus thrombosis. Anticoagulation with unfractionated heparin and intravenous phenytoin was started. At the moment of this report he is asymptomatic and continues with oral anticoagulants and phenytoin.

Síndrome anti-GQ1b: Descripción de cuatro pacientes y revisión de la literatura / Anti-GQ1b syndrome: Report of four cases

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bickerstaff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.

[Cerebral vein thrombosis secondary to closed head injury. Report of one case]. / Trombosis de seno venoso secundaria a traumatismo encéfalo craneano: caso clínico.

Intracranial sinus thrombosis (1ST) after closed head injury is an uncommon but potentially serious complication. It has no correlation with the severity of the injury. The symptoms and clinical course are highly variable. The most frequent but least specific symptom is severe headache. Cerebral lesions and neurologic signs develop in half of patients with IST. We report a 29 year-old male who had an IST after a severe closed head injury. The patient initially developed headache and had later 2 secondarily generalized seizures. The magnetic resonance imaging showed a superior sagittal sinus thrombosis. Anticoagulation with unfractionated heparin and intravenous phenytoin was started. At the moment of this report he is asymptomatic and continues with oral anticoagulants and phenytoin.

[Anti-GQ1b syndrome: report of four cases]. / Síndrome anti-GQ1b: Descripción de cuatro pacientes y revisión de la literatura.

Anti-GQ1b syndrome includes Miller Fisher Syndrome (MFS), Guillain Barré Syndrome (GBS), Bicker staff`s brain stem encephalitis (BBE) and Acute Ophtamoplegia (AO). We report four patients aged 16 to 76 years, with anti-GQ1b syndrome. All presented with MFS, one of them evolved to GBS pharyngeal-cervical-brachial variant and other to GBS with BBE. All had a previous history of diarrhea or upper respiratory tract infection. All had positive anti-GQ1b serum antibodies. Both brain magnetic resonance imaging and cerebrospinal fluid analysis were normal. Electrophysiology studies were compatible with a demyelinating disease. Two patients needed airway protection with an orotracheal tube and developed dysautonomia. All four patients were treated with immunomodulation. On the sixth month follow-up, patients had only minimal alterations in the neurological examination.