Scott R. Bundlie

Delayed emergence of a parkinsonian disorder in 38% of 29 older men initially diagnosed with idiopathic rapid eye movement sleep behavior disorder

We report longitudinal data on a group of 29 male patients 50 years of age or older who were initially diagnosed as having idiopathic REM sleep behavior disorder (RBD) after extensive polysomnographic and neurologic evaluations.Thirty-eight percent (11/29) were eventually diagnosed as having a parkinsonian disorder (presumably Parkinsons disease) at a mean interval of 3.7 plus minus 1.4 (SD) years after the diagnosis of RBD, and at a mean interval of 12.7 plus minus 7.3 years after the onset of RBD. To date, only 7% (2/29) of patients have developed any other neurologic disorder. At the time of RBD diagnosis, data from the RBD group with eventual Parkinsons disease (n equals 11) and the current idiopathic RBD group (n equals 16) were indistinguishable, with two exceptions: the RBD-Parkinsons disease group had a significantly elevated hourly index of periodic limb movements of non-REM sleep and an elevated REM sleep percentage. RBD was fully or substantially controlled with nightly clonazepam treatment in 89% (24/27) of patients in both groups. Thus, RBD can be the heralding manifestation of Parkinsons disease in a substantial subgroup of older male RBD patients. However, a number of presumed Parkinsons disease patients may eventually be diagnosed with multiple system atrophy (straitonigral degeneration subtype). Our findings indicate the importance of serial neurologic evaluations after RBD is diagnosed and implicate the pedunculopontine nucleus as a likely site of pathology in combined RBD-Parkinsons disease, based on experimental and theoretical considerations rather than on autopsy data. NEUROLOGY 1996;46: 388-393

Sleep violence--forensic science implications: polygraphic and video documentation

During the past century, infrequent, anecdotal reports of sleep-related violence with forensic science implications have appeared. Recent rapid developments in the field of sleep-disorders medicine have resulted in greater understanding of a variety of sleep-related behaviors, and formal sleep-behavior monitoring techniques have permitted their documentation and classification. Sleep-related violence can be associated with a number of diagnosable and treatable sleep disorders, including (1) night terrors/sleepwalking, (2) nocturnal seizures, (3) rapid eye movement (REM) sleep-behavior disorder, (4) sleep drunkenness, and (5) psychogenic dissociative states occurring during the sleep period. Potentially violent automatized behavior, without consciousness, can and does occur during sleep. The violence resulting from these disorders may be misinterpreted as purposeful suicide, assault, or even homicide. Sleep-related violence must be added to the list of automatisms. A classification system of both waking and sleep-related automatic behavior is proposed, with recommendations for assessment of such behavior.

A Retrospective Outcome Study and Review of Hypnosis as Treatment of Adults with Sleepwalking and Sleep Terror

Hypnosis has been described anecdotally to be effective in the treatment of sleepwalking and sleep terror, potentially dangerous parasomnias. The authors report the use of hypnosis in the treatment of 27 adult patients with these disorders. A total of 74% of these individuals reported much or very much improvement when followed over substantial periods after instruction in self-hypnotic exercises that were practiced in the home. Hypnosis, often preferred over pharmacotherapy by patients, required one to six office visits (mean=1.6). This represents a very cost-effective and noninvasive means of treatment, especially when constrasted with lengthy psychotherapy and pharmacotherapy.

Polysomnographic studies in amyotrophic lateral sclerosis

We retrospectively reviewed 17 polysomnograms (PSG) in symptomatic amyotrophic lateral sclerosis (ALS) patients to assess the type and frequency of sleep disordered events and correlated these findings with pulmonary function tests (PFTs), presenting complaints, presence of bulbar dysfunction, and response to bi-level positive airway pressure (PAP) treatment. PSG revealed abnormalities in 16 patients. Complaints of orthopnea, daytime sleepiness (but not morning headaches) and a low negative inspiratory force (NIF) correlated with sleep disruption. However, neither the forced vital capacity (FVC) nor the NIF reliably predicted any specific PSG finding. Twelve of 13 patients treated with bi-level PAP responded favorably. Since the response to bi-level PAP is frequently gratifying, PSG should strongly be considered in ALS patients with suspected sleep disturbances.

Interrater agreement in the assessment of motor manifestations of Huntington's disease

With prospects improving for experimental therapeutics aimed at postponing the onset of illness in preclinical carriers of the Huntingtons disease (HD) gene, we assessed agreement among experienced clinicians with respect to the motor manifestations of HD, a relevant outcome measure for preventive trials in this population. Seventy‐five clinicians experienced in the evaluation of patients with early HD and six non‐clinicians were shown a videotape compiled from the film archives of the United States–Venezuela Collaborative HD Research Project. Observers were asked to rate a 2–3‐minute segment of the motor examination for each of 17 at‐risk subjects. The rating scale ranged from 0 (normal) to 4 (unequivocal extrapyramidal movement disorder characteristic of HD). As measured by a weighted κ statistic, there was substantial agreement among the 75 clinicians in the judgment of unequivocal motor abnormalities comparing scale ratings of 4 with ratings that were not 4 (weighted κ = 0.67; standard error (SE) = 0.09). Agreement among the non‐clinicians was only fair (weighted κ = 0.28; SE = 0.10). Even under the artificial conditions of a videotape study, experienced clinicians show substantial agreement about the signs that constitute the motor manifestations of illness in subjects at risk for HD. We expect these findings to translate to a similar level of interobserver agreement in the clinical trial setting involving experienced investigators examining live patients.

Atrial fibrillation and stroke: Epidemiology, pathophysiology, and management

BACKGROUND- Atrial fibrillation is responsible for approximately one in seven strokes in patients of all ages and for one in four strokes in patients aged >80 years. Warfarin reduces stroke risk by approximately 70% and aspirin by 20%.REVIEW SUMMARY- Although the average annual risk of stroke is approximately 5%, there is substantial risk heterogeneity within the population of patients with atrial fibrillation. Risk stratification on the basis of demographic, clinical, and echocardiographic features identifies populations with annual risk of stroke ranging from >7%, clearly warranting warfarin anticoagulation, to <1%, at which the risks and disutility of antithrombotic therapy offset its benefits. Analyses of risk factors consistently show that a previous transient ischemic attack or stroke is a potent predictor of subsequent thromboembolism. The effects of advancing age, left ventricular dysfunction, diabetes, coronary disease, and hypertension are less uniformly established. Transthoracic echocardiography has an ancillary role in current stratification schemes, whereas those of transesophageal echocardiography and hemostatic studies have not yet been established. Predictors of thromboembolism, such as advanced age, unfortunately coincide with risk factors for bleeding on warfarin in atrial fibrillation patients. Audits of patient management have shown that many patients with atrial fibrillation are not receiving preventive treatments. This is particularly true among the elderly.CONCLUSION- Considerable progress has been made over the past decade in our ability to prevent strokes among patients with atrial fibrillation. Wider application of these advances could prevent many thousands of additional strokes each year. Better predictors, safer antithrombotic programs, and other approaches are still needed.

Teaching NeuroImage: Traumatic internuclear ophthalmoplegia

A 26-year-old right-handed man presented to the emergency department with a scalp laceration following a fall while walking on an icy sidewalk. He was intoxicated at the time and was witnessed by a friend to lose his footing, falling backward. There was brief loss of consciousness, lasting less than a minute. The patient himself had amnesia for the fall and for his transportation by ambulance. In the emergency department he had a headache, nausea, and “fuzzy” vision when looking to the left. The patient admitted to use of alcohol earlier that day but denied the use …