Orthostatic Hemifacial Spasm in Idiopathic Intracranial Hypertension.

BACKGROUND: Among the symptoms seen in idiopathic intracranial hypertension (IIH), hemifacial spasm (HFS) is rare. Orthostatic-induced HFS preceding lumbar puncture (LP) is previously unreported. We treated two patients with unusual IIH presentations. This case series reviews the few reports of HFS in IIH and proposes a mechanism for spasm occurrence. METHODS: Case 1: A woman in her mid-40s with previously controlled IIH developed daily headache, pulsatile tinnitus, right-sided trigeminal paresthesia, and right-sided HFS. The latter 2 symptoms occurred exclusively when moving from a sitting to a standing position. Imaging was unremarkable; opening pressure (OP) on LP was 46 cmH2O with normal cerebrospinal fluid (CSF) components. Case 2: A woman in her late 40s presented with severe daily headache, pulsatile tinnitus, and left-sided HFS following weight gain. Imaging was unremarkable; OP on LP was 32 cmH2O with normal CSF components. RESULTS: HFS episodes persisted following LP in both patients. Increasing and initiating acetazolamide, respectively, resolved all symptoms. CONCLUSIONS: Earlier suggested mechanisms of HFS are based on elevated intracranial pressure (ICP) shifting the facial nerve into proximity of a vascular structure. HFS appearing upon standing and continuing after LP, and thus a lower ICP, contradicts this. We propose a mechanism based on the degree of ICP change. This theory is grounded in the lack of intracranial compliance in IIH, wherein substantial pressure changes occur following small volume changes.

Chronic Inflammatory Demyelinating Polyneuropathy With Reversible Severe Cognitive Impairment and Gastrointestinal Dysfunction.

We treated a patient with an unusual case of reversible rapidly progressive cognitive impairment, gastrointestinal dysfunction, and generalized neuromyopathy in chronic inflammatory demyelinating polyneuropathy (CIDP) with optic neuropathy. A man in his 50s presented with a four-month history of rapidly progressive cognitive decline in addition to a six-month history of proximal greater than distal painful muscle weakness, wasting in all extremities, almost complete loss of deep tendon reflexes in his lower extremities, and slow progressive vision loss. Additionally, he had a 90-pound weight loss over the past two years with loss of appetite and ongoing chronic diarrhea. The exam showed muscle weakness and wasting with absent deep tendon reflexes. Initial Saint Louis University Mental Status (SLUMS) exam score was 16/30. Visual acuity was 20/25 with full extraocular movements; optical coherence tomography revealed superior arcuate bundle thinning bilaterally. Gastrointestinal workup proved nonrevealing. Serologic studies for vitamin deficiencies, heavy metals, and autoantibodies were negative. Whipple, Giardia lamblia, and Campylobacter jejuni stool testing were negative. Imaging studies were unremarkable. Nerve conduction studies showed demyelinating sensorimotor peripheral neuropathy. Muscle biopsy was indicative of denervation with scattered myopathic changes; no evidence of inflammatory myopathy nor glycogen or mitochondrial abnormalities was seen. Intravenous immunoglobulin treatment was begun. The patient was started at a dose of 0.75g/kg every three weeks. Following good but incomplete clinical improvement after the first treatment, his dose was increased to 1g/kg every three weeks. He improved remarkably after four months of infusions, scoring 30/30 on SLUMS with a full return of muscle strength and reflexes. Diarrhea remitted. Visual acuity and conduction delay remained unchanged. Symptom timing and dramatic response to immunoglobulins suggest a common immunological mechanism. In light of extensive differential investigations, unremarkable imaging and serology, and no other systemic disease processes, this case plausibly represents a potential new CIDP phenotypic variant.

Contrast sensitivity visual acuity in REM sleep behavior disorder: a comparison with and without Parkinson disease.

STUDY OBJECTIVES: REM sleep behavior disorder (RBD) is characterized by dream enactment behavior and is a premotoric sign associated with parkinsonism and dementia. We previously found contrast sensitivity visual acuity (CSVA) deficiencies in earliest stages of Parkinson disease (PD), plausibly associated with alpha-synuclein deposits in the inner retinal layers. We speculated that individuals with REM sleep behavior without clinical signs of parkinsonism might also show similar deficiencies. METHODS: Twenty-three patients with RBD and 28 healthy control patients. Eleven with PD and 12 with idiopathic RBD (iRBD). Twelve patients with RBD were re-evaluated after 1 year. Evaluations consisted of CSVA SLOAN low contrast acuity charts, optical coherence topography, Unified Parkinson's Disease Rating Scale (UPDRS), and general neurologic and ophthalmologic examinations. Data analyzed between groups using a one-way analysis of variance, and a paired samples t test for returning patients. RESULTS: Participants were classified into three groups: controls (n = 28), iRBD (n = 12), and RBD+PD (n = 11). Analysis of variance revealed CSVA scores were statistically significantly different between the three groups F2, 50 = 7.037, P = .002. Longitudinal analysis of RBD group showed CSVA decreased significantly at 1 year (P = .0141). To date, PD has developed in three individuals with iRBD based on progression of their UPDRS scores. CONCLUSIONS: CSVA is reduced in individuals with RBD and declines over time. It is plausible that patients with iRBD may show early loss in dopaminergic lateral inhibition in the retina, evidenced by their progressive loss of CSVA. This may represent a global loss of dopaminergic neurons similar to PD.

Shifting Spatial Neglect With Repeated Line Bisections: Possible Role of Lateralized Attentional Fatigue.

BACKGROUND AND OBJECTIVE: Many patients who have signs of neglect immediately after a right hemisphere stroke remain disabled even when they improve on tests of neglect. Few patients are tested for attentional persistence and fatigue despite their importance in many instrumental activities. To investigate whether stimulus repetition might alter the allocation of attention, we repeatedly tested a patient 16 weeks after she developed hemispatial neglect from a right hemisphere stroke. METHODS: During each of three testing sessions given in 1 day, we asked the patient to bisect 90 lines of two lengths, presented in 30-trial blocks in three locations: left, center, and right of her midsagittal plane, partially counterbalanced across sessions. We adjusted multiple regression analyses and analysis of variance as well as post hoc regression and t tests for persistence in the repeated measures using variance-sample size analysis to estimate self-similarity, a measure related to the fractal correlation dimension. RESULTS: In each session, the patient's line bisections revealed transient leftward bias in her left peripersonal space, and constant rightward bias in her right peripersonal space. Her leftward bias shifted to a rightward bias over repeated trials in her left space. CONCLUSIONS: The patient's left-to-right shift with repeated trials suggests either fatigue from an attempted compensation for leftward inattention or habituation of a contralesional spatial attentional attraction, revealing contralesional left-sided neglect in both the left and right spaces. Future studies are needed to learn if other patients show similar or different deficits with repetition, and how best to treat them.

Talking while walking: Cognitive loading and injurious falls in Parkinson's disease.

Multitasking has become a way of life, from operating multiple software packages simultaneously on a computer, to carrying on a conversation on a cell phone while driving. Perhaps one of the most common dual tasks performed is talking while walking. In isolation, neither task would be considered difficult to perform, yet when coupled, the relative ease of each task may change. This paper details significant problems that result from injurious falls, and points out the vulnerability of those who have been diagnosed with Parkinson's disease. In addition, it provides an illustrative study that demonstrates the potential danger of talking while walking, especially when the cognitive-linguistic complexity of verbal tasks is manipulated. In this investigation, 25 participants with Parkinson's disease and 13 participants without neurological compromise completed gait tasks while conducting tasks of low (counting by ones), middle (serial subtraction of threes), and high load (alpha-numeric sequencing). The results indicated that cognitive-linguistic demand had an impact on gait, the effects of which were demonstrated in individuals without neurological compromise as well as those with Parkinson's disease. One finding, altered double-support time, distinguished the Parkinson group from the control participants. These results suggest that it might be prudent for healthcare professionals and caregivers to alter expectations and monitor the cognitive-linguistic demands placed on elderly individuals, particularly those with neurological compromise who might be at greater risk for injurious falls.

The effects of auditory distraction on visual cognitive performance in multiple sclerosis.

BACKGROUND: A subset of individuals with multiple sclerosis (MS) endures degradation of cognitive function during disease progression. The purpose of this study was to compare visual cognitive reaction time performance during three conditions of auditory distraction (four-talker babble; word repetition; babble combined with word repetition) to a quiet, undistracted condition. METHODS: Twenty-two patients with mild relapsing-remitting MS (Expanded Disability Status Scale mean of 3.0) and 17 age-matched and education-matched control subjects free of neurologic disease were tested on four cognitive visual processing subtests of simple reaction time, choice reaction time, and visual working memory for same and sequential digits concurrently during three conditions of auditory distraction. RESULTS: When reaction times for MS and control participants were pooled across all four cognitive tests, the scores of the MS patients in quiet (528 ms) were significantly slower than those of the control subjects (459 ms). The auditory distraction condition of word repetition combined with four-talker babble degraded cognitive performance more than most of the other distraction conditions in both groups. CONCLUSIONS: Even in mild MS, subtle visual cognitive processing deficits may be elicited by auditory distraction.

The effects of loading and unloading treadmill walking on balance, gait, fall risk, and daily function in Parkinsonism.

Our study aims were: 1) to determine whether assisted weight bearing or additional weight bearing is more beneficial to the improvement of function and increased stability in gait and dynamic balance in patients with Parkinsonism, compared with matched controls (treadmill alone). Twenty-three men and women participants (M +/- SD = 74.5 +/- 9.7 yrs; Males = 19, Females = 4) with Parkinsonism were in the study. Participants staged at 1-7 (M +/- SD = 3.96 +/- 1.07) using the Hoehn & Yahr scale. All participants were tested before, after the intervention (within one week), and four weeks later on: 1) dynamic posturography, 2) Berg Balance scale, 3) United Parkinson's Disease Rating Scale (UPDRS), 4) biomechanical assessment of strength and range of motion, and 5) Gaitrite force sensitive gait mat. Group 1 (treadmill control group), received treadmill training with no loading or unloading. Group 2 (unweighted group), walked on the treadmill assisted by the Biodex Unweighing System at a 25% body weight reduction. Group 3 (weighted group), ambulated wearing a weighted scuba-diving belt, which increased their normal body weight by 5%. All subjects walked on the treadmill for 20 minutes per day for 3 days per week for 6 weeks. Improvements in dynamic posturography, falls during balance testing, Berg Balance, UPDRS (Motor Exam), and gait for all groups lead us to believe that neuromuscular regulation can be facilitated in all Parkinson's individuals no matter what treadmill intervention is employed.

The effects of balance training and high-intensity resistance training on persons with idiopathic Parkinson's disease.

OBJECTIVE: To assess immediate and near-term effects of 2 exercise training programs for persons with idiopathic Parkinson's disease (IPD). DESIGN: Randomized control trial. SETTING: Public health facility and medical center. PARTICIPANTS: Fifteen persons with IPD. INTERVENTION: Combined group (balance and resistance training) and balance group (balance training only) underwent 10 weeks of high-intensity resistance training (knee extensors and flexors, ankle plantarflexion) and/or balance training under altered visual and somatosensory sensory conditions, 3 times a week on nonconsecutive days. Groups were assessed before, immediately after training, and 4 weeks later. MAIN OUTCOME MEASURES: Balance was assessed by computerized dynamic posturography, which determined the subject's response to reduced or altered visual and somatosensory orientation cues (Sensory Orientation Test [SOT]). Muscle strength was assessed by measuring the amount of weight a participant could lift, by using a standardized weight-and-pulley system, during a 4-repetition-maximum test of knee extension, knee flexion, and ankle plantarflexion. RESULTS: Both types of training improved SOT performance. This effect was larger in the combined group. Both groups could balance longer before falling, and this effect persisted for at least 4 weeks. Muscle strength increased marginally in the balance group and substantially in the combined group, and this effect persisted for at least 4 weeks. CONCLUSION: Muscle strength and balance can be improved in persons with IPD by high-intensity resistance training and balance training.

Aerobic exercise intervention improves aerobic capacity and movement initiation in Parkinson's disease patients.

Parkinson's Disease (PD) is a progressive neurologic disorder, which includes an inability to activate appropriate muscle activity. Very little research has analyzed aerobic exercise for PD patients. The purpose of this study was to investigate the effect of a 16 week aerobic exercise intervention on aerobic capacity and movement initiation (MI) time for PD patients. With 8 PD subjects (Hoehn & Yahr stage 2), 4 completed the exercise intervention. Peak VO_2scores significantly improved (26%) Choice MI improved from 532 ms to 415 ms, while simple MI improved from 285 ms to 261 ms. The improvement in aerobic capacity suggests that PD patients may benefit from exercise just as much as a normal population. The change in MI indicates that aerobic exercise may reduce the detrimental effects of neuromuscular slowing within PD patients, by improving the subjects' ability to initiate and perform appropriate movement patterns.