ABSTRACT
BACKGROUND AND PURPOSE: To characterize patients with benign essential blepharospasm (BEB) by diagnosis, environmental risk factors, and family history. METHODS: Two hundred and forty patients with BEB were evaluated through a clinical examination and questionnaire. The questionnaire reviewed personal medical history, demographic factors, risk factors for the development of blepharospasm and family history of dystonia and other neurological conditions. RESULTS: Benign essential blepharospasm was more commonly found in women (2.8:1) and 93% of the patients were Caucasian. Fifty percent had pure BEB, 31% had BEB/Meige's syndrome, and 4% had BEB and eyelid opening apraxia (+/- Meige's syndrome). A minority of patients reported preceding photophobia (25%) or other eye conditions (22%). The majority were non-smokers, had no exposure to anti-emetic or antipsychotic agents, had a normal birth history, and had no history of head trauma. Seventy-two percent did report a stressful event immediately prior to the development of symptoms. Treatments reported included botulinum toxin (BoNT), oral medications, surgical procedures, and acupuncture. Thirty-two percent of patients reported a family history of focal dystonia, and BEB was the most commonly reported. CONCLUSION: This study confirms previous reports of usual age, sex, caffeine and tobacco use, and family history in patients with blepharospasm. New findings include a report on occupation, lower reports of preceding eye conditions and photophobia, and higher reported stressful events. Further, this study shows a change in treatment with an increase in BoNT use and decrease in surgical procedures.
Subject(s)
Blepharospasm , Adult , Age of Onset , Blepharospasm/complications , Dystonic Disorders/complications , Female , Humans , Male , Middle Aged , Pedigree , Risk FactorsABSTRACT
VATER association is an exceptionally rare condition; however it is associated with multiple rib anomalies, which is one of its unique presentation. We are reporting a case of VATER associated with rib anomalies in various forms like bifid rib, ribbon rib and rudimentary rib.
Subject(s)
Abnormalities, Multiple/diagnostic imaging , Ribs/abnormalities , Adolescent , Female , Humans , Radiography , Ribs/diagnostic imaging , UltrasonographyABSTRACT
BACKGROUND/AIMS: Previously we have shown that functional declines in Parkinson's disease (PD) and Alzheimer's disease (AD) correlate to global measures of cognitive decline. We now determine if the correlation between cognitive impairment and functional ability in PD is similar to that in AD using individual cognitive measures. METHODS: 93 PD subjects and 124 AD/MCI subjects underwent the Functional Assessment Staging (FAST), the Global Deterioration Scale (GDS), and a neuropsychological battery. RESULTS: In PD subjects, the FAST and GDS correlated significantly with Rey Auditory Verbal Learning Test (AVLT), Controlled Oral Word Association (COWA), Animal Fluency, and Stroop but not with Clock Draw or Judgment Line Orientation (JLO). In AD/MCI subjects, FAST and GDS correlated with all neuropsychological components except Stroop. In the AD/MCI group, the UPDRS significantly correlated with the FAST, GDS, MMSE, and all neuropsychological parameters except the Stroop. In the PD group, the motor UPDRS significantly correlated significantly with FAST, GDS, MMSE and all neuropsychological parameters except Digit Span, Stroop, Clock Draw and JLO. CONCLUSIONS: Similar to AD, functional decline in PD correlates with multiple measures of cognitive impairment. Some differences between PD and AD may be explained by the influence of motor disability and declines in visuospatial function in PD.
Subject(s)
Alzheimer Disease/complications , Cognition Disorders/complications , Motor Skills , Parkinson Disease/complications , Aged , Aged, 80 and over , Alzheimer Disease/physiopathology , Alzheimer Disease/psychology , Cognition Disorders/physiopathology , Cognition Disorders/psychology , Female , Humans , Male , Mental Status Schedule , Neuropsychological Tests , Parkinson Disease/physiopathology , Parkinson Disease/psychology , Statistics, Nonparametric , Verbal LearningABSTRACT
We sought to define quantitative electroencephalographic (EEG) measures as biomarkers of both early and late cognitive decline in Parkinson's disease (PD). PD subjects classified as cognitively normal (PD-CogNL), mild cognitive impairment (PD-MCI), and dementia (PD-D) were studied. Cognitive status and neuropsychological testing was correlated with background rhythm and frequency band EEG power across five frequency bands. We conclude that global EEG measures have potential use as biomarkers in the study of both early and late cognitive deterioration in PD, including for evaluating its treatment. PD-MCI has mean quantitative EEG characteristics that represent an intermediate electrophysiological state between PD-CogNL and PD-D.
Subject(s)
Cognition Disorders/etiology , Electroencephalography , Parkinson Disease/complications , Aged , Aged, 80 and over , Disease Progression , Female , Humans , Male , Neuropsychological Tests , Retrospective StudiesABSTRACT
OBJECTIVES: To systematically investigate the ability of Parkinson's disease patients to discretely and dynamically scale the size of continuous movements and to assess the impact of movement size on outcome variability. METHODS: Ten patients with Parkinson's disease (mean age 72 years) were compared with 12 healthy elderly controls (mean age 70 years). The subjects wrote with a stylus on a graphics tablet. In experiment 1 they drew circles, matching the size of five target circles ranging in magnitude from a radius of 0.5 cm up to 2.5 cm. In experiment 2 they drew spirals with a radius of at least 2 cm. In both experiments the drawings were initially performed as accurately as possible then as fast and accurately as possible. RESULTS: In both experiments the patients and controls drew at a similar speed. The within trial variability of the pen trajectory was greater for patients than controls, and increased disproportionately with the size of the movement. When the emphasis was on size rather than variability (circles), the patients' drawing movements were the same size as controls. When the emphasis was on accuracy of pen trajectory (that is, minimum variability) rather than size (spirals), the patients' drawing movements were smaller than controls. CONCLUSIONS: The movements made by Parkinson's disease patients are hypometric partly as an adaptive strategy used to reduce movement variability. This strategy is used primarily when the requirement to make accurate movements outweighs the need to make large movements.
Subject(s)
Attitude , Parkinson Disease , Psychomotor Disorders/diagnosis , Size Perception , Aged , Aged, 80 and over , Female , Humans , Male , Middle Aged , Severity of Illness Index , Time FactorsABSTRACT
As the population continues to age, it has become increasingly important for clinicians to recognize the clinical characteristics of normal aging. Impaired mobility is one of the most frequent effects of normal aging, and this necessitates a specific understanding of the effects of normal aging on the motor system. This article reviews the physiological basis and clinical manifestations of normal aging as related to movement disorders. The impact of normal aging on major hypokinetic and hyperkinetic movement disorders is also discussed.
