Neuraxial anesthesia for Kennedy’s motor neuron disease. A case report.

Introduction: Anesthetic management of patients with motor neuron disease represents a challenge. Kennedy’s disease (KD), also known as spinal and bulbar muscle atrophy (SBMA), is a rare, adult-onset, X-linked motor neuron disease characterized by progressive spinal and bulbar motor neuron disease and muscle atrophy. In this report, we describe a case of successful application of neuro-axial anesthetic (NA) during hip arthroplasty in KD through a combined spinal-epidural anesthetic. Materials and Methods: Informed consent was obtained from the patient for publication. As the case report is devoid of patient identifiable information, it is exempt from IRB review requirements as per Virginia Commonwealth University Healthcare policy. The authors were directly responsible for pre-operative evaluation, administration of neuro-axial anesthesia and monitored sedation during the surgical procedure. Daily post-operative follow-up and management of the epidural catheter were conducted by the anesthesia team per institutional protocols. The patient’s medical record was reviewed to depict outcome based on outpatient clinic evaluations. Case Report: We are reporting a 60-year-old gentleman with KD who presented with traumatic displaced intertrochanteric fracture of left femur. The patient’s neurological condition was advanced with pronounced dysarthria, dysphagia, and generalized weakness. The patient had a history of failure to extubate after general endotracheal anesthesia (GETA) requiring prolonged mechanical ventilation and tracheostomy that was later reversed. He was evaluated by anesthesiology and deemed a suitable candidate for NA. The patient tolerated the anesthetic and surgical procedures well and was discharged on postoperative day 3 after an uneventful recovery. Discussion: The disease phenotype is a function of mutated androgen receptor gene resulting in the accumulation of defective protein which leads to motor neuron loss and myopathy. Indeed, previous reports indicate a high risk of perioperative complications associated with GETA. Muscle relaxation poses the risk of hyperkalemic crises with succinylcholine or extended neuromuscular blockade with non-depolarizing agents leading to failure to extubate post-operatively and prolonged ventilation. Volatile anesthetic agents have also been implicated in severe rhabdomyolysis and anesthesia-related cardiac arrest in patients with Becker’s muscle dystrophy. The bulbar dysfunction characteristic of KD also inherently pose a heightened risk of aspiration and/or laryngospasm. Rapid sequence intubation (RSI), preferably without muscle relaxation and total intravenous anesthesia (TIVA) is, therefore, the preferred GETA technique. Neuro-axial anesthesia, when indicated, can spare the patient the risks associated with GETA, however, patients should be counseled on relatively higher morbidity of complications inherent to NA like high spinal and potential for transient neurological symptoms (TNS). Conclusion: Combined spinal-epidural anesthesia and monitored sedation can be safely and effectively deployed in selected cases of KD

Patient-reported outcome following nonsurgical management of type II odontoid process fractures in adults

Background: Transverse (type II) odontoid process fracture is among the most commonly encountered cervical spine fractures. Nonsurgical management through external immobilization is occasionally preferred to surgical management but is criticized for its higher rates of failure and lower patient satisfaction. Our aim is to analyze patient-reported outcomes in patients who underwent nonsurgical treatment for type II odontoid fractures. Methods: We identified patients >18-year-old who underwent external immobilization as a treatment for isolated type II odontoid fracture between 2007 and 2012. We collected demographic parameters, clinical presentation, mode of injury, imaging studies and modality and duration of treatment (soft collar, halo-vest, or both). Patients were contacted by telephone to participate in a 15-min survey addressing their recovery including their subjective rate of return to preinjury level of functioning. Results: Fifteen patients met the inclusion/exclusion criteria and participated in our survey. Patients were followed up for an average of 19 months after injury. Overall mean age was 61 years. Injury followed a mechanical fall or a road traffic accident in 11 and 4 cases, respectively. External immobilization was achieved by halo vest only in nine patients, soft collar only in two patients (13%), and through a sequential combination in the remaining 4 (27%). This was deployed for a mean of 7.8 months. Radiological studies at the last follow-up showed bony healing (27%), fibrous nonunion (60%), and persistent instability (13%). Patients reported gradual recovery of function throughout the 1st year after injury with levels above 70% of preinjury functioning achieved by 13% of patients at 6 months, 33% at 9 months, and 47% at 12 months. Overall satisfaction with nonsurgical management was 68%. Conclusion: In selected patients with type II odontoid fractures, external immobilization represents a good option with acceptable course of recovery

Phenotypic characterization of murine models of cerebral cavernous malformations.

Cerebral cavernous malformations (CCMs) are clusters of dilated capillaries that affect around 0.5% of the population. CCMs exist in two forms, sporadic and familial. Mutations in three documented genes, KRIT1(CCM1), CCM2, and PDCD10(CCM3), cause the autosomal dominant form of the disease, and somatic mutations in these same genes underlie lesion development in the brain. Murine models with constitutive or induced loss of respective genes have been applied to study disease pathobiology and therapeutic manipulations. We aimed to analyze the phenotypic characteristic of two main groups of models, the chronic heterozygous models with sensitizers promoting genetic instability, and the acute neonatal induced homozygous knockout model. Acute model mice harbored a higher lesion burden than chronic models, more localized in the hindbrain, and largely lacking iron deposition and inflammatory cell infiltrate. The chronic model mice showed a lower lesion burden localized throughout the brain, with significantly greater perilesional iron deposition, immune B- and T-cell infiltration, and less frequent junctional protein immunopositive endothelial cells. Lesional endothelial cells in both models expressed similar phosphorylated myosin light chain immunopositivity indicating Rho-associated protein kinase activity. These data suggest that acute models are better suited to study the initial formation of the lesion, while the chronic models better reflect lesion maturation, hemorrhage, and inflammatory response, relevant pathobiologic features of the human disease