Nursing care of patients with idiopathic parkinsonism

Through the discussion of the work of a movement disorder clinic in British Columbia, this paper focuses on the unique contribution of nursing care in the effective management of idiopathic parkinsonism. It follows on from a paper discussing the aetiology and treatment of the disease published on April 20 in Nursing Times.     

CT-verified intracranial calcifications and contrast enhancement in acute disseminated encephalomyelitis: a case report

Acute disseminated encephalomyelitis (ADEM) is a demyelinating disease which follows viral infection or vaccination. We report the CT findings in a 13-year-old boy with ADEM after infection with Epstein-Barr virus. After 11 days, the patient developed intracranial calcifications in addition to demyelinating lesions. This is a rare finding in ADEM.     

Symptoms and functional status of patients with disseminated cancer visiting outpatient departments

Considerable research has focused on pain and other symptoms in terminal cancer patients referred to hospices and palliative care services. These patients differ from Dutch cancer patients in the palliative stage of their disease because the latter are cared for by general practitioners at home and medical specialists in outpatient departments. To clarify the experience of these Dutch patients, a study was started to investigate the prevalence and severity of pain and other symptoms as well as the functional status of consecutive patients visiting oncology outpatient departments for follow-up. After randomization, one group (I) of patients was interviewed at home by a general practitioner using structured questionnaires. The other group (II) received the questionnaires by mail, and scored the symptoms independently. The results of the symptom assessment show that patients in groups I and II suffered 2.4 (SD = 1.7) and 2.8 (SD = 2.0) symptoms, respectively. Between 30% and 40% of all patients reported constipation, nausea, loss of appetite, coughing, and dyspnea. These percentages were 50% lower when only moderate, severe, or extremely distressing symptoms were included. Sixty percent of all patients had pain, and 20% indicated a daytime pain score of 5 or greater on a scale of 0 to 10. Functional status was measured by the COOPWONCA charts; the mean score for the charts "physical fitness" and "daily activities" was 1.5 points lower for cancer patients than a random sample from the community of the same age and gender. The findings of this study should motivate doctors to put more energy in symptom assessment and interventions in palliative care.     

Gangliosides and parkinsonism

The protective effect of GM1 ganglioside against nerve cell death induced by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) was analyzed in monkey mesencephalon. Administration of GM1 before and during MPTP treatment improved motor performances compared with MPTP-treated animals that received saline rather than GM1. Postmortem analysis revealed that GM1 did not protect dopaminergic cell bodies from MPTP intoxication but resulted in an increased immunoreactivity of tyrosine hydroxylase in the perikarya and processes of the surviving neurons. These data suggest that GM1 may be potentially used as a palliative rather than a curative therapy in Parkinson's disease.     

Progressive encephalomyelitis with rigidity

Two cases of encephalomyelitis are described in which the major clinical manifestation was muscular rigidity and stimulus-sensitive muscular spasms. It is suggested, from pathological evidence, that this rigidity was of spinal origin, and that this disorder is a rare but recognizable entity. Comparison is made with previously reported cases of rigidity of spinal origin, including encephalitis lethargica, and with 'subacute myoclonic spinal neuronitis' and the 'stiff man syndrome.'     

Early onset parkinsonism with diurnal fluctuation maps to a locus for juvenile parkinsonism

Early onset parkinsonism with diurnal fluctuation (EPDF) is a dopa-responsive parkinsonism characterized by early onset and improvement of parkinsonian symptoms after sleep. A pathologic study of one case of EPDF showed selective neuronal degeneration in the zona compacta of the substantia nigra without Lewy body formation. To determine the disease locus for EPDF, the authors examined 53 members of 17 EPDF families and analyzed 151 meioses. Multipoint linkage analysis gave a peak lod score of 14.2 at 1.0 cM telomeric to D6S305 and placed the disease locus in the 17-cM interval between D6S437 and D6S253, which is exactly the same position mapped for autosomal recessive juvenile parkinsonism (ARJP; Mendelian inheritance in man 600116). The highest two-point lod score was obtained at D6S305 (10.13 at theta = 0). Haplotype analysis agreed with the result of multipoint analysis. A high proportion of nonconsanguinity (8/17 families), the absence of commonly shared haplotypes, and the widespread geographic distribution of the families' origins suggest that EPDF has multiple founder mutations. The authors concluded that EPDF and ARJP are allelic disorders and should be included in the spectrum of a single-clinicogenetic entity of chromosome 6q-linked parkinsonism. This disease could be characterized by a benign clinical course and relatively selective degeneration of nigral dopaminergic neurons with no Lewy body formation. To determine whether ARJP/EPDF constitutes a significant proportion of familial or sporadic forms of benign early onset parkinsonism will require further study.     

Secondary immunodeficiency and its myelopid correction in patients with infiltrative and disseminated pulmonary tuberculosis

The combined treatment of 53 patients with colonic cancer, using neoadjuvant intraarterial polychemotherapy (IAPCT), was conducted. The trustworthy lowering of the catheterized artery thrombosis occurrence rate, the tumor recurrence-by 7%, distant metastases-by 13.9%, two-year survival increase-by 15.5% was promoted by verapamil application as a IAPCT modifier.     

Treatment of parkinsonism with dopaminergic system agonists

Solitary renal cysts associated with hypertension and documented hyperreninemia are relatively uncommon. A 13 year old boy with these findings is described (pressure 160/120 mm Hg). Contrast studies of the urinary tract and arteriography with selective renal vein sampling demonstrated a solitary cyst in the right kidney. The ratio of plasma renin activity in the right renal vein to left renal vein (RRV:LRV) was 2.35:1. After drainage of the cyst, there was a marked reduction in both systolic and diastolic pressures (126/84 mm Hg). Hypertension has not recurred during 14 months' follow-up.     

Activation of coagulation and fibrinolysis in patients with abdominal true aortic aneurysm associated with disseminated intravascular coagulation

Two cases of abdominal true aortic aneurysm (AAA) associated with disseminated intravascular coagulation (DIC) were reported. Case 1 was an 81-year-old male who was admitted because of hematoma on the left leg and in whom was found by MRI an aortic aneurysm of 14 cm in diameter. Coagulation studies indicated DIC by revealing thrombocytopenia, hypofibrinogenemia and increased level of FDP. DIC was well controlled by surgical repair of the aneurysm after the administration of a small dose of heparin. Case 2 was a 60-year-old male who was admitted because of lumbago and hematoemesis and in whom was found by CT and echography an aortic aneurysm of 5.5 cm in diameter. Coagulation studies indicated DIC by revealing thrombocytopenia and an increased level of FDP. On the 2nd hospital day, he suddenly died due to the rupture of the aortic aneurysm. In most of 9 cases with AAA without DIC, plasma levels of thrombin-antithrombin III complex, plasmin-alpha 2 plasmin inhibitor complex and FDP-D dimer were also elevated. These findings indicate that the coagulation and fibrinolysis systems were generally activated in patients with AAA, and that DIC tends to occur in patients with a giant aortic aneurysm or an impending ruptured aneurysm.     

Imaging of the dopaminergic neurotransmission system using single-photon emission tomography and positron emission tomography in patients with parkinsonism

Parkinsonism is a feature of a number of neurodegenerative diseases, including Parkinson's disease, multiple system atrophy and progressive supranuclear palsy. The results of post-mortem studies point to dysfunction of the dopaminergic neurotransmitter system in patients with parkinsonism. Nowadays, by using single-photon emission tomography (SPET) and positron emission tomography (PET) it is possible to visualise both the nigrostriatal dopaminergic neurons and the striatal dopamine D2 receptors in vivo. Consequently, SPET and PET imaging of elements of the dopaminergic system can play an important role in the diagnosis of several parkinsonian syndromes. This review concentrates on findings of SPET and PET studies of the dopaminergic neurotransmitter system in various parkinsonian syndromes.     

Sexual function in patients with disseminated sclerosis. A 5-year follow-up study

Sexual dysfunction is known to occur in multiple sclerosis (MS). The purpose of the study is to describe the change in sexual function and symptoms in a five year follow-up study. Forty-nine patients (27 females, 22 males) with definite MS were interviewed and examined. The number of patients with sexual dysfunction increased significantly (p = 0.004) and involved females and males equally. Males usually had one or two symptoms, while females frequently had two or more symptoms. It is concluded that the risk of sexual dysfunction increases over time. Further studies concerning treatment possibilities are needed.     

Reactive hemophagocytic syndrome: a new presentation of disseminated histoplasmosis in patients with AIDS

We report the cases of six patients with AIDS in whom reactive hemophagocytic syndrome (RHPS) secondary to disseminated histoplasmosis was diagnosed. RHPS was diagnosed by established criteria, including fever (duration of > or = 7 days, with peak temperatures of > 38.5 degrees C), unexplained thrombocytopenia with anemia and/or neutropenia, and bone marrow biopsy findings of hemophagocytic histiocytosis. Disseminated Histoplasma capsulatum infection was diagnosed on the basis of the results of cultures of the bone marrow sample. The serum lactate dehydrogenase (LDH) level was elevated (> 1,000 IU/L) in all patients, and five of six patients had hyperferritinemia (range of ferritin level, 15,848-425,984 ng/mL). Five patients had features resembling severe sepsis with multiorgan dysfunction. Three patients recovered, and the findings of RHPS resolved following therapy with amphotericin B. In patients with AIDS, the combination of fever, cytopenia, elevated serum LDH level (> 1,000 IU/L), and/or hyperferritinemia (ferritin level of > 10,000 ng/mL) is a clue to the diagnosis of RHPS and disseminated histoplasmosis; bone marrow biopsy is valuable in establishing the diagnosis.     

PCR detection of DNA specific for Trichosporon species in serum of patients with disseminated trichosporonosis

Deep-seated trichosporonosis is a lethal opportunistic infection that disseminates rapidly and widely in immunocompromised patients, and early diagnosis is crucial for the treatment of this infection. We developed a novel nested-PCR assay that detects DNA specific for clinically important strains of Trichosporon in serum samples from patients with disseminated trichosporonosis. In this assay, two sets of oligonucleotide primers were derived from the sequence of 26S rRNA genes of Trichosporon asahii. The specific fragment was amplified from T. asahii and T. mucoides, but not from other microorganisms, including some other basidiomycetous fungi (Cryptococcus, Malassezia, Rhodotorula, and Sporobolomyces). Target DNA was detected by the nested PCR with as little as 5 fg of the extracted DNA of T. asahii. In a study using 11 clinical samples, the specific fragment was detected by the nested PCR in 64% (7 of 11) of sera from patients with histologically diagnosed disseminated trichosporonosis, while glucuronoxylomannan antigen was detected in only 54% (6 of 11) of the samples. Our new nested-PCR assay using serum samples can be performed repeatedly throughout the course of the disease. In addition, not only can it be used for early diagnosis of trichosporonosis, but it may also be beneficial for monitoring its progress or response to therapy.