Current Concepts and Controversies in the Management of REM Sleep Behavior Disorder.

Rapid eye movement (REM) sleep behavior disorder (RBD) is characterized by dream enactment and the loss of muscle atonia during REM sleep, known as REM sleep without atonia (RSWA). RBD can result in significant injuries, prompting patients to seek medical attention. However, in others, it may present only as non-violent behaviors noted as an incidental finding during polysomnography (PSG). RBD typically occurs in the context of synuclein-based neurodegenerative disorders but can also be seen accompanying brain lesions and be exacerbated by medications, particularly antidepressants. There is also an increasing appreciation regarding isolated or idiopathic RBD (iRBD). Symptomatic treatment of RBD is a priority to prevent injurious complications, with usual choices being melatonin or clonazepam. The discovery that iRBD represents a prodromal stage of incurable synucleinopathies has galvanized the research community into delineating the pathophysiology of RBD and defining biomarkers of neurodegeneration that will facilitate future disease-modifying trials in iRBD. Despite many advances, there has been no progress in available symptomatic or neuroprotective therapies for RBD, with recent negative trials highlighting several challenges that need to be addressed to prepare for definitive therapeutic trials for patients with this disorder. These challenges relate to i) the diagnostic and screening strategies applied to RBD, ii) the limited evidence base for symptomatic therapies, (iii) the existence of possible subtypes of RBD, (iv) the relevance of triggering medications, (v) the absence of objective markers of severity, (vi) the optimal design of disease-modifying trials, and vii) the implications around disclosing the risk of future neurodegeneration in otherwise healthy individuals. Here, we review the current concepts in the therapeutics of RBD as it relates to the above challenges and identify pertinent research questions to be addressed by future work.

Predictors of poor outcome in patients with a spontaneous cerebellar hematoma.

BACKGROUND AND PURPOSE: The authors studied the clinical and neuroimaging features of cerebellar hematomas to predict poor outcome using comprehensive statistical models. METHODS: We retrospectively reviewed clinical and neuroimaging features in 94 patients with spontaneous cerebellar hematomas to identify predictive features for a poor neurologic outcome, defined as death or dismissal to long-term care facility. Data were analyzed using chi square and Fisher's exact test with calculation of odd's ratios together with 95% confidence intervals. RESULTS: Clinical and neuroradiologic predictors for a poor outcome at p < 0.05 were admission systolic blood pressure > 200 mm Hg, hematoma size > 3 cm, visible brain stem distortion, and acute hydrocephalus. Presenting findings predicting subsequent death at p < 0.05 were abnormal corneal and oculocephalic responses, Glasgow coma sum score less than 8, motor response less than localization to pain, acute hydrocephalus and intraventricular hemorrhage. CONCLUSION: A tree-based analysis model using binary recursive partitioning showed that cornea reflex, hydrocephalus, doll's eyes, age, and size were the most important discriminating factors. Absent corneal reflexes on admission highly predicts poor outcome (86 percent, confidence limits 67-96 percent). When a cornea reflex is present, acute hydrocephalus predicts poor outcome but only when doll's eyes are additionally absent.

Clinician's biases toward surgery in cerebellar hematomas: an analysis of decision-making in 94 patients.

BACKGROUND AND PURPOSE: No studies have examined clinical decision-making in cerebellar hemorrhages. Clinical and CT features may influence surgery in patients with a spontaneous cerebellar hematoma. One commonly accepted adage is to remove a clot when 3 cm or larger in axial diameter on presentation CT scan. It is possible that certain preferences impact on outcome. METHODS: We analyzed 94 patients with spontaneous cerebellar hematomas between the years of 1973-1993. Thirty-one patients underwent suboccipital craniectomy and clot removal with or without ventriculostomy. Deterioration denoted worsening of consciousness, new brainstem signs, or presentation in coma. Statistical analysis was performed utilizing a tree-based model fitted by binary recursive partitioning. Ninety-five percent confidence intervals (CI) were calculated. RESULTS: The overall probability of surgical intervention was 33% (CI 23-43%). The chance of surgery in stable patients was 7% (CI 2-20%). Neurologic deterioration was seen in 54 patients (57%) and increased the prospects of a surgical procedure (52%, CI 38-66%). Surgery was performed in all deteriorating patients with small hematomas (size <3 cm), but large clots (size >3 cm) did not substantially influence surgical decision-making (45%, CI 30-60%) except in patients younger than 70 years old (57%, CI 41-82%). CONCLUSIONS: Clinicians at our institution usually wait for clinical deterioration to unfold prior to operating on patients with cerebellar hematomas. After deterioration occurs, they prefer small hematomas but will operate on large hematomas when patients are younger than 70, generally withholding surgery from older patients. These attitudes may impact on outcome and should be considered in future treatment trials.

Predicting deterioration in patients with lobar haemorrhages.

OBJECTIVE: To study the clinical course and determine predictors of deterioration in patients with lobar haemorrhages). METHODS: A comprehensive review of 61 consecutive patients with lobar haemorrhages was performed. Neurological deterioration was defined as (1) decrease in Glasgow coma sum score by 2 points, (2) new neurological deficit, or (3) clinical signs of brain herniation. A univariate logistic regression was performed and expressed in odds ratios. RESULTS: Sixteen of 61 (26%) patients with lobar haemorrhages deteriorated after admission. In a univariate analysis, only a Glasgow coma score <14 predicted deterioration (75% of deteriorators v 24% who did not deteriorate; p<0.0001). Initial CT characteristics predictive of deterioration included haemorrhage volume >60 ml (63% v 16%, p< 0.0001), shift of the septum pellucidum (75% v 36%, p<0.01), effacement of the contralateral ambient cistern (33% v 0%, p<0.0001), and widening of the contralateral temporal horn (50% v 0%, p<0.0001). Patients presenting and deteriorating within 12 hours of ictus declined due to enlargement of the haemorrhage. Those who deteriorated more than 12 hours after initial neurological symptoms, showed increased mass effect secondary to oedema. CONCLUSION-Patients with lobar haemorrhages presenting immediately after ictus are at risk for deterioration from enlargement of the haemorrhage and predictors of deterioration may be absent. Patients with large volume lobar haemorrhages presenting to the emergency department with decreased level of consciousness and shift on CT are at risk for further deterioration from worsening oedema. These patients require close observation and early aggressive management may be warranted.

Musical tradition, insurrection, and resurrection: the life and legacy of composer/bassist Charles Mingus.

Charles Mingus was perhaps the foremost straight-ahead jazz upright bassist and composer of his generation, blending the inspirational influences of gospel, jazz improvisation, and art music leanings into a unique style all his own. His demise from amyotrophic lateral sclerosis (ALS) in his fifth decade robbed the world of one of the great creative voices of American music. Aspects of Mingus' life, his career as a bassist, bandleader, and composer, and his neuromuscular illness are discussed, emphasizing his legacy for the disparate fields of jazz and neurology.

Predicting neurologic deterioration in patients with cerebellar hematomas.

BACKGROUND: Patients with cerebellar hematomas may appear stable but may worsen suddenly. Whether certain clinical or CT scan findings predict worsening is not known. METHODS: We reviewed clinical and neuroimaging data in 72 patients with cerebellar hematomas at the Mayo Clinic from 1973 through 1993 to identify predictive features for neurologic deterioration. Patients presenting in coma and patients with vascular malformations or malignancies were excluded. Data were analyzed using chi-square or Fisher's exact test, with calculation of odds ratios with 95% confidence intervals. Multivariate logistic regression analysis was performed on appropriate variables. RESULTS: Thirty-three patients (46%) deteriorated, with a decrease in level of consciousness, new brainstem signs, or worsened motor response on the Glasgow Coma Scale. Clinical and neuroradiologic predictors for neurologic deterioration at p < 0.05 were admission systolic blood pressure greater than 200 mm Hg, pinpoint pupils and abnormal corneal or oculocephalic reflexes, hemorrhage extending into the vermis, hematoma size more than 3 cm in diameter, brainstem distortion, intraventricular hemorrhage, upward herniation, and acute hydrocephalus. Multivariate analysis demonstrated that hemorrhage located in the vermis (p = 0.03) and acute hydrocephalus (p = 0.0006) on admission CT scanning independently predicted deterioration. CONCLUSION: Patients with a cerebellar vermian hematoma or acute hydrocephalus are at high risk for neurologic deterioration. These patients should be carefully monitored and are more likely to require consideration for neurosurgical intervention.