ABSTRACT
Multiple System Atrophy (MSA) and idiopathic Parkinson's disease (PD) can be difficult to distinguish. There is an ongoing debate about the diagnostic value of the growth-hormone response to clonidine (CGH-test) in PD and MSA. We investigated whether the CGH-test can identify individual patients in the early stages of PD (n = 21) and Striatonigral Degeneration (SND, n = 11), a particular variety of MSA. Patients were diagnosed on the basis of clinical criteria and IBZM-SPECT. Clonidine induced a greater total serum growth-hormone production in PD than in SND (p = 0.01). However, taking the difference in prevalence of PD and SND into account, and because of the low likelihood ratios of the test, an increase of GH after clonidine increases the pre-test probability for PD by about only 5 %, while an absent response of GH also increases the pre-test probability for SND by about 5 %. We conclude that the CGH-test discriminates between groups of patients with PD and SND, but has little practical diagnostic value for identifying individual patients.
Subject(s)
Clonidine , Growth Hormone/metabolism , Parkinson Disease/diagnosis , Striatonigral Degeneration/diagnosis , Aged , Analysis of Variance , Chi-Square Distribution , Diagnosis, Differential , Growth Hormone/blood , Humans , Middle Aged , Parkinson Disease/blood , Statistics, Nonparametric , Striatonigral Degeneration/bloodABSTRACT
There are conflicting reports about estrogen modulating the activity of nigrostriatal dopaminergic neurons. Furthermore, modulation may be influenced by progesterone levels. Therefore, the clinical effects of sex steroids on parkinsonian symptoms in postmenopausal women with Parkinson's disease (PD) were analyzed in the present study. Patients (n = 12) were under the age of 80, able to perform the motor function tests, and showed no contraindications for estrogen suppletion. Motor function was assessed using the Unified Parkinson's Disease Rating Scale (UPDRS) and a patient interview on subjective changes. In a placebo-controlled, randomized, double-blind trial lasting 8 weeks, no significant dopaminergic effect of estradiol (E2) could be demonstrated, whereas in an open trial phase lasting 2 weeks, progesterone seemed to have an antidopaminergic effect. Several mechanisms are discussed that can account for the fact that we found no effect of E2 on motor functioning in our patients with PD.
Subject(s)
Antiparkinson Agents/therapeutic use , Estradiol/therapeutic use , Parkinson Disease/drug therapy , Postmenopause/drug effects , Progesterone/therapeutic use , Aged , Double-Blind Method , Female , Humans , Middle Aged , PlacebosABSTRACT
Hereditary deficiency of prothrombin is a rare autosomal recessive bleeding disorder, with severe bleeding diathesis in homozygotes, but rarely resulting in intracranial haematoma. We describe two infants of consanguineous parents, presenting with acute subdural haematoma. Because such haematomas in infancy are highly indicative of trauma caused by child battering and because the socio-economic status of the family was unstable, there was a suspicion of child battering. However, further investigations revealed a bleeding diathesis due to a prothrombin deficiency. DNA analysis of the prothrombin gene showed homozygosity for a novel mutation, substituting Lys for Glu at codon 7 and resulting in decreased specific clotting activity. We discuss the probability of bleeding diathesis versus child battering in the aetiology of intracranial haematoma.
Subject(s)
Hematoma/etiology , Hypoprothrombinemias/diagnosis , Battered Child Syndrome/complications , Battered Child Syndrome/diagnosis , Consanguinity , Diagnosis, Differential , Fatal Outcome , Hematoma/diagnosis , Humans , Hypoprothrombinemias/complications , Hypoprothrombinemias/genetics , Infant , Male , PedigreeABSTRACT
A 3-month open-label trial was performed to evaluate the efficacy of 200 mg Q10 daily in 10 patients with Parkinson's disease. Motor performance was assessed with UPDRS and motor tests. There was no significant effect on the clinical ratings.
