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This paper highlights the hemiparkinsonism-hemiatrophy (HPHA) syndrome as a unique presentation of the parkinsonian state. Clinically relevant diagnostic and treatment aspects are reviewed.
We report a case of HPHA, in a 21-year-old, otherwise healthy, woman. Clinical and radiographic features of our case are presented. We also review the current literature on the clinical, radiological and pathophysiological mechanisms of HPHA.
In our case, despite the lack of benefit from anticholinergics and dopamine agonists (the patient declined treatment with levodopa), the patient showed a dramatic improvement with subthalamic nucleus stimulator (STN) implantation. There are no reported cases of use of STN stimulator in HPHA.
Hemiparkinsonism-hemiatrophy is a distinct entity that may be clinically and pathogenetically different from idiopathic Parkinson’s disease; hence, HPHA needs to be considered as a possible syndrome in patients that have persistent unilateral parkinsonism. As medications are reported to be unhelpful in HPHA cases, early surgical intervention may be an option, such as in our case.
Objective: Multiple system atrophy (MSA) is an incurable neurodegenerative illness in which progressive symptoms, including stridor and acute laryngeal obstruction, occur. Advanced care planning and palliative care discussions in people living with MSA are not well defined. The aim of the present study is to evaluate advanced care planning and current practices in palliative care in MSA to identify opportunities for improving quality of care. Methods: The study is a retrospective chart review assessing the focus and timing of palliative care discussions in people living with MSA. Some 22 charts were reviewed. Results: A total of 22 patients were included. The most common symptoms were parkinsonism, orthostatic hypotension, GI/GU dysfunction, ataxia and gait impairment. Six patients had stridor. Of the palliative care discussions that took place, the most common topics were diagnosis, symptoms or symptom management, and prognosis. In the majority of patients who died and who had a do-not-attempt-resuscitation order, discussions surrounding resuscitation and goals of care took place only hours before death. Conclusions: There is no standard approach to advanced care planning and palliative care discussions in people living with MSA. We propose a framework to guide advanced care planning and palliative care discussions in MSA.
The stiff-person syndrome is a disorder of persistent, painful muscle contractions predominately affecting the axial musculature. We describe a patient with this disorder and review its pathophysiology. Molecular biologic and immunologic techniques have recently added to the understanding of the mechanism of this disorder. Association with diseases such as diabetes, vitiligo and hypothyroidism have strengthened the auto-immune nature of this syndrome. Auto-antibodies against glutamic acid decarboxylase (GAD), an intraneuronal enzyme, have been implicated in the etiology of this unique disease. Therapeutic intervention with agents such as benzodiazepines that modify central GABAergic activity have demonstrated significant benefit in patients with stiff-person syndrome.
Acute femoral neuropathy after renal transplantation is an uncommon and rarely recognized complication. Recovery of the nerve is usual. Although rare, five cases have come to our attention in the past twenty years. A detailed clinical and electrophysiological analysis with a six month follow-up is presented. A review of sixteen other reported cases is also provided. The possible pathophysiology including direct compression and nerve ischemia, is discussed. We believe that nerve ischemia, possibly caused by a steal phenomenon, occurs in all cases following the anastomosis of the graft renal artery to the internal iliac artery, with a superimposed component of compression in some cases. The severity of ischemia probably determines the degree of recovery.
The purpose of this study was to determine whether tasks involving effortful attention would cause augmentation of rigidity in patients with mild Parkinson disease.
In 17 subjects with mild Parkinson disease, rigidity in a single arm was assessed during various experimental conditions by a blinded movement disorders neurologist. Rigidity was scored separately at the wrist and the elbow using an ordinal scale. In three of the conditions, sustained attention was directed toward visual, auditory or movement-related stimuli. Two varieties of Froment maneuver served as positive controls: contralateral hand opening-closing or ipsilateral foot tapping. In addition, rigidity was assessed twice with subjects resting. The examiner was unaware of the sequence of experimental conditions and this was changed for each subject. Mean rigidity scores for the various experimental conditions were compared against the baseline state (an average of both trials with the patient resting) using a repeated measures ANOVA and post-hoc Tukey-Kramer multiple comparisons test.
Rigidity was significantly increased from baseline with each of the attentional tasks (p <0.01 to p <0.001) and also with the two Froment maneuvers (p <0.001). Rigidity augmentation with contralateral hand opening-closing was significantly greater than with any of the attentional tasks (p <0.05 to p <0.001).
Tasks of effortful attention did appear to augment rigidity in patients with mild Parkinson disease. We speculate that the greater augmentation seen with the Froment maneuver could have an anatomic basis.
This review assesses the current opinion towards early palliative care in neurology and discusses the existing evidence base. A comprehensive literature search resulted in 714 publications with 53 being directly relevant to the scope of this review. The current literature reflects primarily expert opinion and describes a growing interest in the early introduction of palliative principles into neurological care. Early initiation of palliative interventions has the potential to improve quality of life, enhance symptom management and assist in advance care planning. Further data is required to determine whether this shift in philosophy has a positive impact on patient care.
Management of chronic diseases such as movement disorders can be challenging. Nurse-administered telephone follow-up programs have demonstrated clinical and cost efficacy in a variety of health care models. However, their efficacy in movement disorders has not been sufficiently addressed. This observational study fills a knowledge gap by reporting the nature of individuals utilizing a nurse-administered telephone service and the reasons for and the outcomes of calls.
Consecutive calls received by the clinic for a 12-month duration were recorded. A sample of 312 calls from 132 patient charts was analyzed. Variables for analysis and coding schema were determined a-priori and included demographic information as well as information around the reasons for and outcomes of calls. The narratives of documented calls were reviewed retrospectively and responses coded for analysis by a separate researcher. Data was analyzed using descriptive statistical methods.
Patients made the majority of calls (49%). 27% of calls related to worsening symptoms and another 35% of calls related to medication issues or renewals. The mean call duration was 15.93 minutes. The majority of calls were received mid-way between clinic visits (M = 89.24 days). The nurse resolved 84% of calls independently. The mean number of calls per patient was 2.93. Issues reported by patients were resolved (approximately 90%) without need for follow-up emergency, family, or subspecialty clinic visits.
The results underscore the complexity of medical issues in a movement disorders population. The current study provides support for a nurse-administered telephone follow-up program in movement disorders.
Individuals with Parkinson's disease (PD) show poorer balance and greater incidence of falls while turning. We investigated whether a disturbance in timing and sequence of reorientation of body segments is a potential cause of turning difficulty in PD and is altered by levodopa.
The sequence and timing of segmental reorientation during 45° and 90° walking turns were recorded in nineteen healthy controls and fourteen individuals with PD “off” and “on” medication.
Both healthy elderly and PD patients “off” medication displayed a top-down sequence of segment reorientation, but differed with respect to the delay time between segments: PD “off” medication displayed a shorter delay between the onset of head and shoulder reorientation and longer delays for pelvis and foot reorientation. Furthermore, for all segments the peak angular velocities were lower for PD patients than healthy controls, with greater difference between the two groups during larger turns. While for both groups the velocity and magnitude of rotation of all segments were greater during larger turns, the relative timing of reorientation of segments remained the same during small and large turns. Medication had no significant effect on the timing and sequence of reorientation of segments and caused only a small and non-significant increase to segment velocities.
This study further characterized the turning performance of individuals with PD. Our findings have clinical applications and therapeutic value for PD patients with difficulty turning. Understanding the specific deficiencies of turning performance of PD patients allows the therapists to opt for the most effective rehabilitation techniques.
Many of the falls among people with Parkinson's disease (PD) occur during sudden, on-the-spot turning which requires systematic reorientation of axial segments towards the new direction. We examined whether a disturbance in the coordination of segmental reorientation is an important cause of turning difficulty in individuals with PD and is altered by dopaminergic medication.
The sequence and timing of segmental reorientation during 45° and 90° on-the-spot turns was examined in fourteen individuals with PD while “off” and “on” medication and nineteen healthy controls (HC).
Regardless of the magnitude of the turn, HC reoriented their head, shoulder, and pelvis simultaneously followed by mediolateral foot displacement. PD patients displayed temporal coordination patterns similar to the HC. PD however, reduced the velocity and early magnitude of reorientation of each body segment which were both slightly improved by dopaminergic medication.
Our finding that the HC and PD patients turn en bloc when the turn is predictable and there are no time constraints shows that the strategy of en bloc turning is not wrong if the movement parameters are unconstrained. However, in real life situations, which usually require quick and unpredictable turns, the en bloc strategy may be unsafe and more likely to result in falls. While in such situations HC are able to change the strategy from en bloc to sequential segmental turning, PD patients may not be able to do so and continue to turn en bloc.
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