Remimazolam and Remifentanil Anesthetics for an Adolescent Patient with Stiff-Person Syndrome: A Case Report.

Stiff-person syndrome (SPS) is a rare autoimmune disease characterized by fluctuating rigidity and stiffness of the axial muscles. There are no reports on the use of remimazolam in a patient with SPS. A 16-year-old Japanese woman with SPS was scheduled to undergo intrathecal baclofen pump exchange. General anesthesia was induced and maintained using remimazolam, remifentanil, and intermittent rocuronium bromide. No intraoperative mobility or significant autonomic symptoms were observed. Additionally, electroencephalographic signature showed sufficient anesthetic depth. The patient's emergence from general anesthesia was uneventful. In conclusion, remimazolam could be considered an effective anesthetic drug for patients with SPS.

Surgical Treatment for Toe Deformities in Stiff-Person Syndrome: A Case Report.

CASE: Stiff-person syndrome is a rare neurological disorder characterized by rigidity and painful spasms of the trunk and limbs, and patients sometimes have difficulty in walking due to rigid toe deformities. This is a case report of a 76-year-old woman suffering from stiff-person syndrome with painful rigid toe deformities regained walking ability after metatarsal osteotomy and cutting of the toe extensors for all toes in the left foot. CONCLUSION: For patients with stiff-person syndrome, surgical intervention is a powerful treatment option when they have developed rigid and painful toe deformities despite adequate pharmacological treatment.

Successful Anesthetic Management of Patient with Stiff Person Syndrome.

We herein describe the successful anesthetic management of a patient with stiff person syndrome undergoing right total hip replacement under spinal anesthesia. We also describe the problems associated with general anesthesia. The advantage of using regional anesthesia in these patients is the avoidance of muscle relaxants. The use of general anesthesia carries the risk of hypotonia in stiff person syndrome postoperatively due to enhancement of γ aminobutyric acid (GABA) action on synaptic transmission by drugs that have a γ GABA agonistic action.

Multiple anesthetics for a patient with stiff-person syndrome.

Stiff-person syndrome is a progressive disease of muscle rigidity and spasticity due to a deficiency in the production of γ-aminobutyric acid. Because of the rarity of the condition, little is known about effects of anesthesia on patients with stiff-person syndrome. This report describes the clinical course for a single patient with stiff-person syndrome who received general anesthesia on 3 separate occasions. Her anesthetics included use of both neuromuscular blockade and volatile agents. Unlike several previous reports regarding anesthesia and stiff-person syndrome, the postoperative period for this patient did not require prolonged intubation or result in any residual weakness.

[Two Separate Instances of Anesthetic Management in a Patient Initially Diagnosed with Stiff-person Syndrome].

In this case report, we describe separate instances of general anesthetic management administered to a patient during treatment for two unrelated conditions. The patient, a 57-year-old woman who had been experiencing walking difficulties for about four years, fell down because of muscle rigidity and spasms and fractured her humerus. She was subsequently diagnosed with stiff-person syndrome. The fracture was treated conservatively, but three weeks later the alignment of the humerus became worse and the patient was scheduled to undergo an open reduction and internal fixation under general anesthesia (GA). Anesthesia was induced using propofol and fentanyl, and rocuronium was given for the tracheal intubation. The operation was completed successfully while anesthesia was maintained using sevoflurane and remifentanil without incident Four months later, the patient developed paraneoplastic syndrome stemming from breast cancer, and underwent a resection of the cancer under GA. The patient received propofol for the laryngeal mask insertion, and sevoflurane and pentazocine for maintenance of anesthesia. She was discharged from the hospital without any respiratory or airway problems. We used sevoflurane for maintenance of anesthesia on both occasions, and rocuronium for the first tracheal intubation. The patient's hemodynamics were stable during the operation. No prolonged effect of the muscle relaxant or any autonomic reactions were observed. The patient was fully conscious and in good respiratory condition after both surgeries, and was extubated in the operating room. There were no perioperative complications.

Manejo anestésico del síndrome de Stiff Man / Anaesthetic management of Stiff Man syndrome

El síndrome de Stiff Man o síndrome de la persona rígida es una enfermedad rara de causa inmunológica. Se caracteriza por la rigidez en la musculatura axial y las extremidades inferiores, y espasmos dolorosos desencadenados por estímulos. Presentamos el caso de un paciente de 44 años con síndrome de la persona rígida al que se le realizó una infiltración del esfínter uretral con toxina botulínica bajo sedación. Antes de la inducción anestésica todo el equipo quirúrgico estuvo preparado con el objetivo de minimizar el tiempo anestésico. Se realizó una monitorización continua de ECG, SpO2 por pulsioximetría y presión no invasiva. Fue inducido con dosis fraccionadas de propofol hasta 150 mg, fentanilo 50 μg y midazolam 1 mg. A pesar de las dosis bajas y fraccionadas el paciente presentó una desaturación máxima del 90% que fue resuelta con ventilación manual. Durante la intervención no hubo episodios de espasmos ni de rigidez muscular. El paciente fue dado de alta 2 días después, sin incidencias. Aprovechamos el caso para revisar aquellos publicados hasta la fecha sobre este tema. El interés sobre el manejo anestésico de estos pacientes viene dado por las interacciones entre la medicación preoperatoria, los fármacos anestésicos y el sistema GABA. Recomendamos el uso de la anestesia total intravenosa frente a los agentes anestésicos inhalatorios, la vigilancia estrecha de la función respiratoria y el uso de la monitorización neuromuscular cuando sean utilizados relajantes musculares para un manejo anestésico más seguro (AU)

Stiff Man syndrome or stiff-person syndrome is a rare autoimmune disorder. It is characterized by increased axial muscular tone and limb musculature, and painful spasms triggered by stimulus. The case is presented of a 44-year-old man with stiff-person syndrome undergoing an injection of botulinum toxin in the urethral sphincter under sedation. Before induction, all the surgical team were ready in order to minimise the anaesthetic time. The patient was monitored by continuous ECG, SpO2 and non-invasive blood pressure. He was induced with fractional dose of propofol 150 mg, fentanyl 50 μg and midazolam 1 mg. Despite careful titration, the patient had an O2 saturation level of 90%, which was resolved by manual ventilation. There was no muscle rigidity or spasm during the operation. Post-operative recovery was uneventful and the patient was discharged 2 days later. A review of other cases is presented. The anaesthetic concern in patients with stiff-person syndrome is the interaction between the anaesthetic agents, the preoperative medication, and the GABA system. For a safe anaesthetic management, total intravenous anaesthesia is recommended instead of inhalation anaesthetics, as well as the close monitoring of the respiratory function and the application of the electrical nerve stimulator when neuromuscular blockers are used (AU)

Recurrent thymoma with stiff-person syndrome and pure red blood cell aplasia.

Stiff-person syndrome (formerly known as stiff-man syndrome) is a very rare autoimmune and neurogenic disorder, thought to present as a paraneoplastic variant in association with thymoma. Pure red blood cell aplasia is also a paraneoplastic disorder associated with thymoma. Although separate cases of stiff-person syndrome and pure red blood cell aplasia have been reported, we describe here what is to our knowledge the first case of recurrent thymoma with both stiff-person syndrome and pure red blood cell aplasia. We describe the successful treatment of the neurogenic symptoms of stiff-person syndrome and the progressive anemia associated with pure red blood cell aplasia by tumor excision.

Anesthesia considerations in stiff person syndrome.

A 34 year old morbidly obese stiffperson syndrome (SPS) patient was scheduled for a permanent catheter placement. SPS is a rare neurologic condition with a suspected autoimmune etiology. SPS most common manifestations are progressive, including severe muscle rigidity or stiffness affecting the spine and lower extremities more than other muscle groups. SPS have superimposed episodic muscle spasms that may resemble myotonic-like contractions and are precipitated by unexpected noises, tactile stimuli, or emotional stress. This case report describes a patient with SPS and morbid obesity, and his subsequent management perioperatively for a permanent catheter placement under monitored anesthesia care. Careful and methodical management of patients with SPS is strongly suggested given their sensitivity to inhalational anesthetics and neuromuscular blockers.

[Anesthetic management of a patient with stiff-person syndrome undergoing thymectomy].

Stiff-person syndrome is an uncommon disease characterized by muscular rigidity and painful spasms in the axial and limb muscles. We report a 58-year-old woman with stiff-person syndrome undergoing thymectomy under general anesthesia. Before surgery, her medications were 25 mg of diazepam, 2 mg of clonazepam, and 15 mg of gabapentin per day. After epidural catheterization for the postoperative analgesia, general anesthesia was induced and maintained with continuous remifentanil infusion and propofol with target controlled infusion. With train-of-four ratio (TOFR) monitoring by stimulating the ulnar nerve, her trachea was intubated after 0.6mg x kg(-1) of rocuronium intravenous administration. Since then, additional rocuronium was not given for 4 hours. After surgery, she was fully awake and TOFR recovered to 100%, but tidal volume was too low to remove the tracheal tube, and mechanical ventilation was continued in ICU. On the next day, the tracheal tube was removed, and she was discharged from ICU. Because anesthetics may delay the recovery of respiratory function in a patient with stiff-person syndrome, careful assessment of respiratory function is needed at the emergence from general anesthesia.

Stiff-person syndrome first manifesting in pregnancy.

Stiff-person syndrome (SPS) is a rare neurological disorder characterised by progressive stiffness and painful muscle spasms. We present a case of the autoimmune form of glutamate decarboxylase-positive SPS that initially manifested in pregnancy. The diagnosis was made based on clinical, laboratory and electromyoneurographic criteria. The patient was administered low doses of diazepam and baclofen. Considering the clinical picture of SPS patients, caesarean section is the method of choice for pregnancy termination.

[Anesthetic management for thymectomy in a patient with stiff-person syndrome].

Stiff-person syndrome is an autoimmune disease characterized by muscle rigidity accompanied by decreased respiratory function. We report a patient with this syndrome who underwent thymectomy under general anesthesia. A 79-year-old woman complaining of increasing muscle rigidity over the past four months was transferred to this hospital. Marked speech disturbance and dysphagia were observed on admission. The diagnosis of stiff-person syndrome was made based on an increase in serum anti-glutamic acid decarboxylase antibody level and thymoma in the anterior mediastinum. Following alleviation of muscle rigidity by high-dose gamma-globulin, thymectomy was scheduled. General anesthesia was given with propofol, fentanyl and epidural ropivacaine. Propofol was continuously infused to maintain BIS below 60 and vecuronium was intermittently administered when muscle contraction was observed in response to electrical stimulation of the ulnar nerve. Despite full recovery of muscle contractility following surgery, tidal volume was too low to remove the tracheal tube, and mechanical ventilation was continued in ICU. One hour after admission to ICU, the tracheal tube was removed, with no marked changes in respiratory condition thereafter. Since many anesthetics are respiratory suppressants that can delay the recovery of respiratory function, careful monitoring of respiratory condition is required postoperatively.

[Artificial hip joint implantation in stiff-man syndrome]. / Künstlicher Hüftgelenksersatz bei Stiff-Man Syndrom.

UNLABELLED: AIM AND METHOD OF THE STUDY: The Stiff-Man syndrome is characterized by progressive, usually symmetric rigidity of the axial muscles of the trunk with superimposed painful spasms precipitated by various stimuli. The neurologic disorder of SMS is discussed with respect to its symptoms of orthopedic interest including treatment modalities for total hip replacement. RESULTS: An increased incidence of hip disease in patients with SMS seems unlikely according to our analysis. CONCLUSION: SMS-patients should, however, be defined at risk for heterotopic bone formation following hip arthroplasty requiring a perioperative prophylaxis.