Biomedical subjects
Publications and source records attributed to L M Shulman.
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The authors surveyed 101 patients with Parkinson disease (PD) about their experiences disclosing the diagnosis. Ninety percent disclosed early to family; more than 25% waited at least 1 year to disclose at work. The main concerns about disclosure were fear of reflecting negatively on themselves and fear of upsetting others. Patients who delayed disclosure were more likely male, younger, and employed. There is considerable variability among patients with PD in the time to disclose their diagnosis.
BACKGROUND: Electronic communication is important in healthcare, but the level of computer proficiency among patients with neurological disorders is unknown. OBJECTIVE: This study sought to determine the proportion of a movement disorder clinic population that was able to perform basic computer skills, and the effect of specific cognitive and motor features on computer proficiency. METHODS: One hundred and four movement disorder patients participated. Seventy-four completed both paper and computerized questionnaires to evaluate data entry skills and thirty subjects completed paper questionnaires only. Basic e-mail messaging and Internet skills were evaluated. Demographic information, Mini-Mental Status Examination (MMSE) score, and Hoehn and Yahr stage were assessed. RESULTS: Ninety-six percent of subjects successfully completed computerized data entry tasks, and over 70% completed e-mail and Internet tasks. Computer data entry had an average accuracy of nearly 95% when compared to paper data entry. Poorer performance on computer tasks was associated with older age, less education, and cognitive impairment. Computer performance was reduced in subjects with a history of parkinsonism and when both tremor and dyskinesia were present during task performance. Nearly three-quarters of subjects have access to a computer. Subjects who completed the paper questionnaire but refused to complete the computer questionnaire were older, less educated and more cognitively impaired. CONCLUSION: The majority of patients visiting a tertiary movement disorders center were able to perform computer data entry, e-mail messaging and Internet usage. These results reinforce the potential value of electronic communication and information systems in neurology practice.
BACKGROUND: Depression, anxiety, fatigue and sleep disorders occur commonly in patients with Parkinson's disease (PD). These non-motor symptoms often contribute to the reduction of functional abilities in PD patients. OBJECTIVE: This study was designed to evaluate the diagnostic accuracy of the treating neurologist for a variety of behavioral symptoms commonly associated with PD. METHODS: A prospective evaluation of 101 patients with PD selected in no particular order was conducted. All patients were evaluated with the Unified Parkinson's Disease Rating Scale (UPDRS), Hoehn & Yahr Stage (H/Y), and the Schwab & England Scale (S/E). The patients completed a brief screening questionnaire for depression and anxiety followed by the administration of a battery of standardized tests including the Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Fatigue Severity Scale (FSS), and the Pittsburgh Sleep Quality Inventory (PSQI). RESULTS: Standardized testing showed evidence of a problem with depression in 44% of patients, anxiety in 39%, fatigue in 42% and sleep disturbance in 43%. The prevalence of these conditions, identified by the treating neurologist was lower: 21% with depression, 19% with anxiety, 14% with fatigue and 39% with sleep disturbance. The diagnostic accuracy for the treating neurologists was 35% for depression, 42% for anxiety, 25% for fatigue, and 60% for sleep disturbance. CONCLUSION: This study demonstrates that during routine office visits, neurologists failed to identify the presence of depression, anxiety, and fatigue more than half of the time and failed to recognize sleep disturbance in 40% of patients. Awareness of the likelihood of underrecognition of behavioral symptoms in PD should generate approaches to improve diagnostic accuracy and facilitate timely therapeutic interventions.
The authors report a 34-year-old woman with a history of extensive cocaine abuse who developed choreodystonic dyskinesias and an obsessive-compulsive behavior disorder. The abnormal movements and behavior disorder persisted after a 20-month cocaine-free period.
Many patients with Parkinson's disease (PD) have clinically significant anxiety, depression, fatigue, sleep disturbance, or sensory symptoms. The comorbidity of these nonmotor symptoms and their relationship to PD severity has not been extensively evaluated. Ninety- nine nondemented PD patients were evaluated with the following battery of tests: Beck Anxiety Inventory (BAI), Beck Depression Inventory (BDI), Fatigue Severity Scale (FSS), Pittsburgh Sleep Quality Index (PSQI), a sensory symptom questionnaire, Unified Parkinson's Disease Rating Scale (UPDRS), Hoehn & Yahr (H/Y) Stage, and the Schwab & England ADL scale (S/E). The comorbidity of the nonmotor symptoms and their relationship to PD severity was analyzed. Thirty-six percent of the study population had depression (BDI > or =10), 33% had anxiety (BAI > or =10), 40% had fatigue (FSS > 4), 47% had sleep disturbance (PSQI > 5), and 63% reported sensory symptoms. Only 12% of the sample had no nonmotor symptoms. Fifty-nine percent of the patients had two or more nonmotor symptoms, and nearly 25% had four or more. Increased comorbidity was associated with greater PD severity (P < 001). This study reveals that the nonmotor symptoms of PD frequently occur together in the same patients. Increased comorbidity of the five nonmotor symptoms was associated with greater PD severity. These results suggest that recognition of these diverse nonmotor symptoms may be enhanced by looking for others when one nonmotor symptom has been identified.
Hemorrhagic cystitis (HC) is a known complication of stem cell transplantation. In contrast to early-onset HC that is usually attributed to cyclophosphamide and occurs within a few days of infusion, late onset HC is associated with viral infection. In recent years BK virus has emerged as an important causative agent. We describe two patients who developed late onset HC (38 and 92 days post transplant) associated with BK viruria concomitant with CMV reactivation and suggest a possible role of CMV in the process of BK virus DNA replication.
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Pregnancy in patients with Parkinson's disease (PD) is a rare occurrence. Previous reports based on retrospective analysis suggest that pregnancy may have a deleterious effect on PD. We describe the effects of pregnancy on the symptomatology of a 33-year-old woman with PD using quantitative neurologic and quality-of-life scales prepartum, intrapartum, and postpartum. During her pregnancy, she was only treated with carbidopa/levodopa. The pregnancy resulted in a normal full-term vaginal delivery of a healthy infant. Significant worsening of this patient's motor symptoms occurred during pregnancy without return to baseline at 15 months postpartum. Pregnancy may exacerbate PD and may have a long-term negative impact on the course of the illness. This report may assist physicians in the counseling of patients with young-onset PD who wish to consider pregnancy.
BACKGROUND: Controversy exists regarding the use of dopamine receptor agonists in elderly patients with Parkinson's disease because of concern about a high rate of intolerable side effects. METHODS: A retrospective chart review was used to examine our experience with dopamine agonist use in the very elderly by identifying patients in our Parkinson's disease database who were over the age of 80 years and who had received agonists. Sixty-nine patients were identified who had 120 separate trials of agonist therapy. Successful treatment with the agonist was defined as maintenance of the agonist for a minimum of 6 months. RESULTS: The overall success rate among the very elderly for an agonist trial was 46%. Success rates for individual agonists were 15 of 27 (56%) bromocriptine, 18 of 34 (53%) pergolide, 17 of 43 (40%) pramipexole, and 5 of 16 (31%) ropinirole. In successful trials with bromocriptine, the mean daily dose was 12.8 mg, mean duration of treatment was 40 months, and mean age at drug initiation was 82 years; for pergolide it was 1.8 mg, 32 months, and 83 years; for pramipexole 2.7 mg, 14 months, and 83 years, and for ropinirole 10.6 mg, 11 months, and 83 years. CONCLUSION: This study demonstrated that therapeutic dosages of dopamine agonists were well tolerated by 46% of very elderly patients who received a trial of an agonist. These results indicate that dopamine receptor agonist therapeutic trials are warranted in selected very elderly patients.
Dopamine receptor agonists are assuming increased importance in the treatment of both early and advanced symptoms of Parkinson's disease (PD). However, tolerability of these drugs can be a problem. Identifying patients who are at increased risk of adverse effects is central to using dopamine agonists in PD. The newer agonists, pramipexole and ropinirole, are generally adequate without levodopa for early symptoms and carry the hope for a more acceptable profile of long-term side-effects. In the patient with advanced disease, all four dopamine agonists significantly augment the response to levodopa, which reduces the problems of motor fluctuations and drug related dyskinesia. Understanding the common pitfalls when prescribing these drugs will facilitate their safety and efficacy.
An unusual, highly diverged derivative of the Sabin type 2 oral poliovaccine (OPV) strain was recovered from environmental samples during routine screening for wild polioviruses. Virus was cultivated in L20B cells and then passaged on BGM cells at 40 degrees C (RCT [reproductive capacity at supraoptimal temperature]-positive marker) to select against most OPV strains. All but 1 of 25 RCT-positive OPV-derived environmental isolates were antigenically and genetically (>99.5% VP1 sequence match) similar to the respective Sabin strains. However, isolate PV2/4568-1/ISR98 (referred to below as 4568-1) escaped neutralization with Sabin 2-specific monoclonal antibodies and cross-adsorbed sera, and had multiple nucleotide substitutions (220 of 2,646; 8.3%) in the P1 capsid region. Fourteen of the 44 associated amino acid substitutions in the capsid mapped to neutralizing antigenic sites. Neutralizing titers in the sera of 50 Israeli children 15 years old were significantly lower to 4568-1 (geometric mean titer [GMT], 47) than to Sabin 2 (GMT, 162) or to the prototype wild strain, PV2/MEF-1/EGY42 (GMT, 108). Two key attenuating sites had also reverted in 4568-1 (A(481) to G in the 5' untranslated region and the VP1 amino acid I(143) to T), and the isolate was highly neurovirulent for transgenic mice expressing the poliovirus receptor (PVR-Tg21 mice). The extensive genetic divergence of 4568-1 from the parental Sabin 2 strain suggested that the virus had replicated in one or more people for approximately 6 years. The presence in the environment of a highly evolved, neurovirulent OPV-derived poliovirus in the absence of polio cases has important implications for strategies for the cessation of immunization with OPV following global polio eradication.
An outbreak of poliomyelitis with 20 cases occurred in Israel, Gaza, and the West Bank from October 1987 to October 1988. The wild type 1 poliovirus associated with the outbreak was most closely related to viruses found in the Nile Delta. The epidemiologic links among patients involved in the outbreak and patients with community-acquired infections during the outbreak were inferred from the evolutionary relationships among isolates of the outbreak virus. Complete VP1 sequences (906 nucleotides) were determined for 12 clinical and 4 sewage isolates. A total of 58 nucleotide differences were found among the 16 isolates; 74% of all substitutions were synonymous third-position transitions. An evolutionary tree, representing both the pathways of VP1 sequence evolution and the inferred chains of virus transmission during the outbreak, was constructed under the assumption that each substitution had occurred only once. The combined epidemiologic and molecular data suggest that a single founder strain was introduced into Israel from the vicinity of Gaza in the fall of 1987. Poliovirus circulation was apparently localized to southern communities during the winter and spread north by the following summer into the Hadera subdistrict of Israel, where it radiated via multiple chains of transmission into other communities in northern Israel and the West Bank. The close sequence matches (>99%) between clinical and sewage isolates from the same communities confirm the utility of environmental sampling as a tool for monitoring wild poliovirus circulation.
In stereotactic pallidotomy for Parkinson's disease, care must be taken to avoid internal capsule injury while maximizing improvement of rigidity and tremor. In 21 patients, intraoperative electromyography (EMG) was used to assess stimulation thresholds required for capsular responses and to monitor muscle tone and tremor. Surface EMG electrodes were placed on the face and multiple muscle groups of the extremities. The stimulation and lesion electrode was introduced via MRI-guided stereotaxis toward a point 2-3 mm anterior to the midcommissural point, 5-6 mm inferior to the AC-PC plane, and 21-22 mm lateral to the midline. Exact targets were modified according to MRI-visualized anatomy. With stimulation at 5 and 50 Hz, thresholds for detection of EMG responses were usually seen at 4-5 mA. EMG responses were consistently seen prior to visual observation of muscle activity. Timing of EMG response relative to stimulus aided in differentiating stimulus-related movement from spontaneous tremor. Resting spontaneous EMG activity was seen to decrease as rigidity was improved by incremental lesion production. EMG activity related to tremor was recorded; tremor decrease by lesion production was documented by EMG recording. Patient cooperation with physiologic testing during stimulation and lesion production may become limited. Intraoperative EMG monitoring provides an adjunct to improve reliability of assessment of capsular stimulation and rigidity while providing documentation of lesion impact on rigidity and tremor.
We report a 48-year-old woman with a 17-year history of PD who developed a peripheral sensory-motor neuropathy secondary to chronic administration (8 years) of amantadine. Discontinuation of amantadine resulted in resolution of trophic skin ulcers, paresthesias, and distal weakness. Amantadine may be hazardous to patients with severe and chronic livedo reticularis.
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Progressive supranuclear palsy (PSP) is a progressive neurodegenerative disorder with no effective treatment. Dopaminergic agents occasionally produce transient symptomatic improvement. The authors report the results of pramipexole treatment (4.5 mg daily) in six patients with PSP (average disease duration, 4.4 years). Patients were treated for 2 months. Patients were evaluated with the Unified Parkinson's Disease Rating Scale, Hoehn and Yahr stage, and Schwab and England Activities of Daily Living Scale at baseline and 2 months. Pramipexole was not efficacious for the symptoms of PSP.