Pregnancy-associated neuromyelitis optical spectrum disorder combined with primary Sjögren's syndrome: A critical illness case report / 北京大学学报(医学版)

Central nervous system involvement in primary Sjögren's syndrome (pSS) is less common and usually presents as white matter lesions, neuromyelitis optica spectrum disorder (NMOSD), or transverse myelitis. NMOSD is an immune-mediated inflammatory demyelinating disease of the central nervous system with a high rate of relapse and significant disability. Studies have shown that patients with pSS combined with NMOSD have more severe symptoms and poorer prognosis. Here, we present a case of critical illness in pregnancy-associated NMOSD combined with Sjögren's syndrome. The patient was a 30-year-old pregnant woman with a history of Sjögren's syndrome who was diagnosed with NMOSD. She received combination therapy with steroids, intravenous immunoglobulin (IVIG), and hydroxychloroquine during pregnancy, resulting in partial resolution of numbness below the waist. However, due to irregular medication adherence outside the hospital setting, she developed weakness in her right lower limb accompanied by inability to move it, while her left lower limb still had some mobility but occasional numbness along with urinary and fecal incontinence. Ten days later, she was admitted to the emergency department where an emergency cesarean section was performed to deliver a healthy baby boy. However, her condition worsened postpartum as she developed high fever accompanied by bilateral lower limb paralysis and weakness along with loss of voluntary control over urination and defecation. The patient underwent ano-ther course of treatment consisting of steroids and IVIG; however there was limited improvement in symptoms observed after this intervention. Following administration of rituximab for the first time, the patient developed urinary tract infection which was successfully managed before continuing regular infusions. In later stages the patient could walk slightly with a limp and regained control over urination and defecation, allowing her to resume normal activities. This case suggests that combination therapy with steroids, IVIG, and hydroxychloroquine should be considered for the patients with pregnancy-associated NMOSD combined with Sjögren's syndrome. Rituximab can significantly improve symptoms such as postpartum paralysis in patients with NMOSD, however, there may be a risk of infection associated with its use.

Síndrome de Tolosa Hunt / Tolosa-Hunt syndrome

RESUMEN El síndrome de Tolosa-Hunt es una entidad poco frecuente cuya etiopatogenia y mecanismos fisiopatológicos se mantienen controversiales. La cefalea asociada a parálisis de uno o más pares craneales, así como diplopía, estrabismo y ptosis palpebral causan un gran temor en el paciente que la padece. Su diagnóstico es por exclusión. Con el objetivo de describir y actualizar el conocimiento sobre esta enfermedad se presenta el cuadro de una paciente que acudió a consulta por cefalea marcada y dolor retroocular. Los hallazgos clínicos y la resonancia magnética confirman el diagnóstico del síndrome de Tolosa Hunt (AU).

ABSTRACT The Tolosa-Hunt syndrome is a few frequent entity whose etiopathogeny and physio-pathological mechanisms are still controversial. The headache associated to the paralysis of one or more cranial paired nerves, and also diplopia, strabismus, and palpebral ptosis cause a great fear in patients suffering it. Its diagnosis is by exclusion. With the objective of describing and up-dating the knowledge on this disease, it is presented the history of a patient who assisted the consultation because of remarked headache and retro-ocular pain. The clinical findings and magnetic resonance confirm the diagnosis of Tolosa-Hunt syndrome (AU).

case of multiple cranial nerve injuries

A female patient 30 years old, mother with one child, housewife with no special habits of medical importance complained of deviation of the angle of the mouth to the right side of two days' duration. Lacrimation of the left eye and difficulty in closing the left eye were present for two days, for which she had consulted aneurologist. She had a past history of chronic left ear discharge, tinnitus, and decrease in hearing acuity of left ear. General examination revealed no detectable abnormalities. Neurological examination revealed left trigeminal nerve palsy, sensory along three divisions. Corneal reflex was absent. There was decrease hearing acuity of left ear especially sensory neural affection. Left facial and hypoglossal nerves were affected. Long tract was not affected. High resolution power CT for left ear, and MRI brain with contrast were normal.ESR, CBC, C-RP were Normal. Sarcoidosis battery revealed elevated serum calcium, and Angiotensin converted enzyme [ACE] with elevated CSF ACE. The patient was treated with Methyl predinsolone l00mg/day in divided doses, Calcium supplement OD and Pantazole FBI 40 mg/day, and given physiotherapy. Recovery was seen within two weeks

Crisis neurológica en Tirosinemia hereditaria / Neurological crisis in hereditary Tyrosinaemia

La Tirosinemia tipo I es el resultado de un error innato en la etapa final del metabolismo de la Tirosina. Sus manifestaciones clínicas son variables, las cuales pueden verse agravadas con la aparición de crisis neurológica. El objetivo del presente trabajo es reportar el caso de una preescolar portadora de la enfermedad, que desarrolló parálisis fláccida asociada a insuficiencia respiratoria y que requirió conexión a ventilación mecánica.

Tríade incomum: rotura do manguito rotator e paralisia do plexo braquial associadas a luxaçäo escapulumeral anterior: relato de caso / Uncommon triad: rotator cuff tear and brachial plexus palsy associated to anteriopr dislocation of the shoulder

A ocorrência da lesao aguda do manguito rotador associada a luxaçao traumática anterior do ombro em pacientes de meia-idade está bem documentada na literatura, porém, muitas vezes, ela pode passar despercebida ou nao ser diagnosticada inicialmente. Quando simultaneamente ocorre a paralisia do plexo braquial, o diagnóstico da rotura do manguito rotador torna-se ainda mais difícil, propiciando, assim, inadequado tratamento. A publicaçao dos casos tem por finalidade mostrar a raridade da lesao, a dificuldade do diagnóstico e o tratamento instituído.

Pure motor hemiplegia with conjugate lateral gaze palsy in pontine lacunar infarction

The combination of pure motor hemiplegia and horizontal gaze palsy is a rare but identifiable lacunar syndrome. Among horizontal gaze palsies, one-and-a-half syndrome and abducens nerve palsy are reported to be associated with pure motor hemiplegia in pontine lacunar infarction. Although conjugate lateral gaze palsy is also hypothesized, pure motor hemiplegia with conjugate lateral gaze palsy has never been reported. We present a 75-year-old man who showed right hemiparesis and impaired left horizontal conjugate eyeball movement. Both the findings of the brain CT scan and those of the MRI study were consistent with a small infarction in the left midpontine tegmentum. Magnetic resonance angiography revealed no stenotic narrowing of the vertebrobasilar artery. Radiological findings suggested that pure motor hemiplegia with conjugate lateral gaze palsy, in our patient, might have been produced by the occlusion of a single penetrating branch of the basilar artery.

Paralisia do nervo femoral consequente à cirurgia ginecológica / Paralysis of femoral nerve following surgery gynecologic

Os autores apresentam um caso de paralisia do nervo femoral, em paciente submetida à histerectomia total abdominal, devido à miomatose uterina. Trata-se de lesäo traumática do nervo femoral por afastador cirúrgico. Os aspectos anatômicos, funcionais e terapêuticos säo descritos a seguir.

Parálisis postoperatoria residual en la sala de recuperación

Parálisis residual, definida como un radio de tren de cuatro 0.7, ha sido descrita frecuentemente después de la Anestesia, ésta ha sido detectada más a menudo (21-42 por ciento) después del uso de drogas bloqueadoras neuromusculares de larga acción (tubocurarina- Pancuronio- Gallamina- Alcuronium) que después del uso de agentes intermedios Atracurium - Vecuronio (0.9 por ciento) la severidad de bloqueo es compatible con una función respiratoria deteriorada. La parálisis persiste representa una falla en la reversión de los relajantes musculares, las Anticolinesterasas, Edrofonio y Neostygmine, actúan por diferentes mecanismos en la unión neuromuscular, la actividad de ellos depende de los relajantes usados y del nivel de bloqueo cuando son adminitrados. Los bloqueos intensos se recuperan lentamente y son más efectivamente antagonizados con Neostigmine que con Edrofonio. El uso seguro de relajantes, depende de una titulación ciudadosa de ambos, las drogas bloqueadoras neuromusculares y sus agentes de reversión. Un Bloqueo persistente sólo puede ser prevenido o reconocido y tratado por el uso de monitoreo neuromuscular durante y al final de la anestesia.

Tetanus with local palsy.

Five cases of tetanus with local palsy are reported. They presented with palatal palsy, pseudohernia, paralysis of both lower limbs, 3rd and 7th cranial nerve palsies and only 7th nerve palsy. Except the last tetanus became generalised in all.

Superior oblique muscle paralysis in the Crest syndrome

Un paciente varón de 54 de edad con la variante de CREST de esclerosis sistémica progresiva (escleroderma) se presentó con diplopia vertical diagnosticada como parálisis del músculo superior oblicuo izquierdo. Aunque en escleroderma se han reportado anormalidades de los nervios craneales, creemos que este es el primer informe de la variante de CREST en asociación a una parálisis del IV nervio la cual no se pudo explicar por otra etiología