[A patient with Creutzfeldt-Jakob disease supported by home medical care from the stage of disease progression to death through hospital-clinic cooperation and medical-welfare cooperation].

The patient was a 63-year-old woman who presented with slowness of speech after cerebral infarction. Diffusion-weighted MR images and investigations of cerebrospinal fluid showed abnormal values, and the patient was diagnosed as having sporadic Creutzfeldt-Jakob disease(CJD). This is an intractable disease and affects one in one million people; it progresses relatively rapidly, eventually resulting in death. For procedures such as intravenous fluid replacement and the treatment of pressure sores, we require thorough hand washing, eye protection, and disposal of gloves and dressings by incineration. It is desirable for patients to spend the limited amount of time available to them peacefully at home with their family. Visiting physicians and nurses need to take the initiative in sharing information obtained from the CJD infection control guidelines and core hospitals with welfare personnel such as caregivers, in order to provide correct information on all aspects of patient care and the management of this disease in the home environment. Excellent supportive care was provided for the patient at home, and she passed away with her family by her side.

Lateralized cortical involvement and contralateral parkinsonism without basal ganglia involvement in two autopsy cases of corticobasal syndrome-Alzheimer's disease.

Corticobasal syndrome (CBS) is characterized by lateralized motor disturbance due to levodopa nonresponsive parkinsonism and progressive apraxia. Although CBS is neuropathologically heterogeneous, it remains unclear whether the clinical features of all CBS cases are the same. We report two autopsy cases diagnosed clinically as CBS and pathologically as Alzheimer's disease characterized by lateralized cerebral cortical degeneration and absence of significant nigrostriatial lesions. Cerebral cortical degeneration in both cases was contralateral to their motor disturbances. Thus, nigrostriatial lesions and contralateral cerebral cortical lesions can cause motor disturbances in CBS, necessitating the need for bedside examination in patients with CBS.

Malignant syndrome in Parkinson's disease: concept and review of the literature.

We reviewed literature on malignant syndrome occurring in patients with Parkinson's disease (PD) during the course of drug therapy. Clinical features were high fever, marked rigidity, consciousness disturbance, autonomic dysfunction, and elevation of serum creatine kinase. The clinical features were essentially similar to those of neuroleptic malignant syndrome. The immediate triggering event was, most often, discontinuation or reduction of anti-parkinsonian drugs, particularly of levodopa. But no anti-parkinsonian drug was the exception to the induction of malignant syndrome. Serious complications were severe pneumonia, disseminated intravascular coagulation, and acute renal failure. Early treatment with intravenous fluid infusion and external body cooling are essential for good recovery. Bromocriptine and dantrolene sodium were used frequently. It has been claimed that they are effective; however, randomized controlled studies are needed to explicitly prove the efficacy of these drugs in malignant syndrome associated with PD.

A collaborative study on the malignant syndrome in Parkinson's disease and related disorders.

We report the results of a collaborative study on malignant syndrome (MS) that developed in patients being treated with levodopa and other anti-parkinsonian drugs. We analyzed clinical features, laboratory findings, precipitating events, and risk factors for poor outcome. The study was conducted in five centers in Japan. Patients who developed MS between January 1991 and December 1997 were included. The enrollment criteria used were the same as those for neuroleptic MS proposed by Levenson et al. (1985).A total of 99 episodes were encountered in 93 patients (72 with Parkinson's disease and 21 with secondary parkinsonism); one patient had four recurrences of MS and three patients had two recurrences. High fever was the most frequent clinical manifestation of MS followed by worsening of parkinsonism, and then altered levels of consciousness. Serum creatine kinase was abnormally elevated in all the patients studied. Life-threatening complications were rhabdomyolysis, disseminated intravascular coagulation, and acute renal failure. The most frequent precipitating event was discontinuation or dose reduction of anti-parkinsonian drugs, particularly levodopa. No drug was the exception in the precipitation of MS. Intercurrent infection was the next most common precipitating event. MS developed without drug withdrawal or infection in some patients. In five patients, severe "wearing off" phenomenon was the only event preceding the onset of MS. Hot weather and dehydration appeared to be the cause in three patients. Among the total of 99 episodes, patients recovered to the pre-MS state following 68 episodes (68.7%); in the remaining 31.3%, patients failed to recover to their previous state. Older age, higher Hoehn and Yahr stage during the symptomatic phase of MS, higher akinesia score, and the absence of wearing off phenomenon prior to developing MS were associated with poor outcome. The most frequently used treatments of MS were intravenous fluid, levodopa, dantrolene sodium, and intragastric bromocriptine. Early introduction of treatment is important. Any elevation of body temperature during the course of anti-parkinsonian drug treatment should be considered as MS until proved otherwise.

Serum creatine kinase is elevated in patients with Parkinson's disease: a case controlled study.

We report serum creatine kinase (CK) activity level in patients with Parkinson's disease and controls matched with gender and age. The clinical subjects consist of 84 patients with Parkinson's disease and 257 control subjects. Serum CK level was significantly elevated in parkinsonian patients (117.3+/-65.0 units/l) compared to that of the controls (85.1+/-33.2 units/l) (p<0.01). The elevation correlated with the use of levodopa and the duration of the disease. In our study, 56.0% of parkinsonian patients and 32.7 % of the control subjects showed higher than normal upper limits of serum CK activity (p<0.01). Whereas no correlation was noted between serum CK level and Hoehn-Yahr stage, age of onset, duration of the disease, predominant symptoms (rigidity vs. tremor), and average daily dose of levodopa. Patients with Parkinson's disease may be sensitive to physical activity resulting in leakage of CK from skeletal muscles. Neural mechanism mediated by hypothalamic dopamine and by autonomic nervous system may also be contributing to the elevation of serum CK.

Prevention and treatment of malignant syndrome in Parkinson's disease: a consensus statement of the malignant syndrome research group.

We report a consensus statement of the collaborative research group on the prevention and treatment of malignant syndrome (MS) in Parkinson's disease. The syndrome is quite similar to neuroleptic MS. Although sudden withdrawal of levodopa was the most frequent cause, many other precipitating events were found such as intercurrent infections, dehydration, hot weather, discontinuation of other anti-parkinsonian drugs, and "wearing off" phenomenon. Awareness of this syndrome is most important for its early detection and the prompt commencement of treatment. MS should be suspected whenever the body temperature rises above 38 degrees C without an apparent cause. Treatment consists of ample intravenous fluid, cooling the body, anti-parkinsonian drugs (particularly levodopa and bromocriptine), dantrolene sodium, and antibiotics if infection is present. Rhabdomyolysis, disseminated intravascular coagulation, and acute renal failure constitute serious complications.

[Long-term treatment of Parkinson's disease patients with selegiline hydrochloride (FPF 1100): outcome of 5-year treatment].

We herein report on the outcome of 5 year-treatment of Parkinson's disease patients with selegiline hydrochloride. The subjects participated in this study were 10 patients whose treatment had been maintained consecutively by administration of this agent even after completion of the Phase II trial (all cases under adjunct therapy with L-DOPA/DCI). The daily dose of selegiline hydrochloride was 6.6 +/- 2.5 mg in average at the end and/or termination of the study. As for L-DOPA, its daily dose decreased from 410 +/- 160 mg to 365 +/- 133 mg at the 6th month, but the dose reduction level after 9 months was not determinable due to an increase in dropouts. Regarding alteration in the scores for individual symptoms, improvement in wearing-off symptom was pronounced during the treatment period of 3 to 51 months. The Global Improvement Rate and Usefulness Rate remained stable during the period of 18 to 30 months treatment although these rates declined after 36 months probably because of exacerbation in disease conditions. This study may assure tolerability of selegiline hydrochloride in a long-term treatment of Parkinson's disease patients.