Anesthesia in a patient with Stiff Person Syndrome / Anestesia em paciente com síndrome da pessoa rígida

Abstract Stiff Person Syndrome (SPS), typified by rigidity in muscles of the torso and extremities and painful episodic spasms, is a rare autoimmune-based neurological disease. Here we present the successful endotracheal intubation and application of TIVA without muscle relaxants on an SPS patient. A 46 years old male patient was operated with ASA-II physical status because of lumber vertebral compression fracture. After induction of anesthesia using lidocaine, propofol and remifentanil tracheal intubation was completed easily without neuromuscular blockage. Anesthesia was maintained with propofol, remifentanil and O2/air mixture. After a problem-free intraoperative period the patient was extubated and seven days later was discharged walking with aid. Though the mechanism is not clear neuromuscular blockers and volatile anesthetics may cause prolonged hypotonia in patients with SPS. We think the TIVA technique, a general anesthetic practice which does not require neuromuscular blockage, is suitable for these patients.

Resumo A síndrome da pessoa rígida (SPR), caracterizada pela rigidez dos músculos do tronco e das extremidades e por episódios de espasmos dolorosos, é uma doença neurológica autoimune rara. Apresentamos o ocaso de intubação endotraqueal bem-sucedida e aplicação de AVT sem relaxantes musculares em um paciente com SPR. Paciente do sexo masculino, 46 anos, estado físico ASA-II, submetido à cirurgia devido à fratura por compressão da coluna lombar. Após a indução da anestesia com lidocaína, propofol e remifentanil, a intubação traqueal foi concluída com facilidade, sem bloqueio neuromuscular. A anestesia foi mantida com propofol, remifentanil e mistura de ar/O2. Após o período intraoperatório, que transcorreu sem intercorrências, o paciente foi extubado e, sete dias depois, recebeu alta, deambulando com ajuda. Embora o mecanismo não esteja claro, bloqueadores neuromusculares e anestésicos voláteis podem causar hipotonia prolongada em pacientes com SPR. Acreditamos que a técnica de AVT, uma prática de anestesia geral que não requer bloqueio neuromuscular, é adequada para esses pacientes.

Anesthesia in a patient with Stiff Person Syndrome.

Stiff Person Syndrome (SPS), typified by rigidity in muscles of the torso and extremities and painful episodic spasms, is a rare autoimmune-based neurological disease. Here we present the successful endotracheal intubation and application of TIVA without muscle relaxants on an SPS patient. A 46 years old male patient was operated with ASA-II physical status because of lumber vertebral compression fracture. After induction of anesthesia using lidocaine, propofol and remifentanil tracheal intubation was completed easily without neuromuscular blockage. Anesthesia was maintained with propofol, remifentanil and O2/air mixture. After a problem-free intraoperative period the patient was extubated and seven days later was discharged walking with aid. Though the mechanism is not clear neuromuscular blockers and volatile anesthetics may cause prolonged hypotonia in patients with SPS. We think the TIVA technique, a general anesthetic practice which does not require neuromuscular blockage, is suitable for these patients.

Effects of clotrimazole on experimental spinal cord injury.

BACKGROUND: We examined a possible neuroprotective effect of clotrimazole on spinal cord clip compression injury. METHODS: Rivlin and Tator's acute extradural clip compression injury (CCI) model was used for producing SCI on 24 albino Wistar rats weighing 180-250 g. All rats were anesthetized with 30 mg/kg ketamine HCl intraperitoneally and were breathing spontaneously without tracheal intubation. Total laminectomy of T8-T12 was performed on all rats under operation microscope, and CCI was performed on all rats (expect those in group 1) with a 50-g closing force aneurysm clip for 1 min. Three hours later, all of the rats were killed with sodium pentobarbital. Spinal cords were excised for a length of 2 cm; 1 cm rostrally and caudally to the injury site and deep frozen at -76 degrees C for biochemical studies. RESULTS: Treatment with clotrimazole decreased MDA levels in rats with SCI with a statistically significant difference. CONCLUSIONS: To our knowledge, this the first study that shows the effects of clotrimazole on spinal cord clip compression injury. Clotrimazole was found to be effective on spinal cord clip compression injury, but further investigations are mandatory.

Ruptured chronic encapsulated intracerebral hematoma in infancy: review of the literature with a case report.

OBJECTIVES: We report a case of a 2-month-old boy with chronic encapsulated intracerebral hematoma (EICH) and discuss clinical, radiological and pathological features of the case. CONCLUSIONS: Chronic EICH in infants is extremely rare in the literature. Only two cases have been previously reported. One of the cases reported in the literature was supratentorial and the other was infratentorial.

Effects of citicoline on experimental spinal cord injury.

OBJECTIVE: To investigate the effects of citicoline on experimental spinal cord injury (SCI). BACKGROUND: Citicoline has been successfully used in clinical studies of head injury and cerebral infarction, but there is limited literature regarding its use in experimental SCI. STUDY DESIGN: Twenty adult Wistar rats were divided into four groups: sham, trauma, vehicle, and citicoline-treated. SCI was produced using a weight drop technique. Citicoline 300 mg/kg was given intraperitoneally, 5 minutes after the induction of trauma. The animals were sacrificed and 1 cm long samples of injured spinal cord were obtained at 48 hours post-SCI. Lipid peroxidation was estimated by the thiobarbituric acid test. Neurological examinations were performed using a previously described grading scale. RESULTS: Measures of lipid peroxidation and motor scores of the citicoline-treated group were significantly lower than those in the other injury groups. CONCLUSIONS: Citicoline attenuated lipid peroxidation after SCI and improved the motor scores. Further investigations will be required to determine the long-term effects of this drug on spinal cord injury.

Giant cranial extradural primary fibroxanthoma: a case report.

Primary fibroxanthoma of the cranium is an extremely rare condition. Xanthomatous tumors of the central nervous system are occasionally associated with diseases such as Hand-Schuler-Christian disease, malignant fibrous histiocytoma, hyperlipidemia, and a complication of metabolic or storage disorders. However, until 2003, only a few cases of primary fibroxanthoma have been reported. We report a giant fibroxanthoma destroying the lateral skull base unaccompanied by a systemic or metabolic disease.

Intracavernous carotid artery aneurysms.

Six patients with intracavernous carotid artery aneurysms (ICCAAns) were seen at our department from 1998 to 2002. All patients had only one intracranial aneurysm and their ages at diagnosis ranged from 36 to 72 years (median 56). Five were women and four had a history of hypertension. One patient was pregnant. All of the ICCAAns were symptomatic at diagnosis. Duration of symptoms was 2-30 days. On admission to our department, initial symptom was headache in four patients, visual loss in two, eye pain in one, third nerve paresis in two and subarachnoid hemorrhage (SAH) in one. Spontaneous thrombosis was present in two patients. All of the ICCAAns were saccular. Computed tomography (CT) was superior when compared with magnetic resonance imaging (MRI) for diagnosis of ICCAAns on admission. Angiography remains the gold standard for diagnosis and determination of specific anatomical details, which are necessary to plan treatment.

Midaortic syndrome and subarachnoid hemorrhage associated with ruptured middle cerebral artery aneurysm: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: We describe the presentation, screening, management, and clinical outcome of a 21-year-old man who sought care for a ruptured middle cerebral artery (MCA) aneurysm and midaortic syndrome (MAS). Only three cases of MAS and intracranial aneurysm rupture have previously been described in the literature. CLINICAL PRESENTATION: Cranial computed tomographic (CT) scanning, cerebral and abdominal angiography, and multislice three-dimensional CT angiography were used to evaluate intracerebral hemorrhage and to assess medically intractable hypertension in the patient. Digital subtraction angiography revealed a right MCA aneurysm, and multislice three-dimensional CT angiography revealed narrowing of the abdominal aorta. INTERVENTION: The patient's right MCA aneurysm was successfully clipped via a right pterional craniotomy. A narrowed abdominal aorta was confirmed by an abdominal aortic angiogram (performed at Day 5 after surgery) and then dilated by using percutaneous transcatheter angioplasty during the same session. The patient was normotensive even without antihypertensive medications. Neurological examination and postoperative cranial CT findings were within normal limits at the last follow-up examination, performed 4 months after the operation. CONCLUSION: Our patient is the first reported case of ruptured MCA aneurysm with MAS in an adult. The most important problem in the management of MAS associated with ruptured intracranial aneurysm is medically intractable hypertension, which may markedly increase the incidence of rebleeding. It is hard to achieve normotension unless the narrowed aorta and its branches are dilated. For these reasons, MAS should be considered in patients with medically intractable hypertension associated with ruptured intracranial aneurysm.