Muscle & NerveVolume 20, Issue 5 p. 625-627 Short Report Characteristic morphologic manifestation of cadasil, cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy, in skeletal muscle and skin Hans H. Goebel MD, Corresponding Author Hans H. Goebel MD Department of Neuropathology, Mainz University Medical Center, Langenbeckstrasse 1, D-55131 Mainz, GermanyDepartment of Neuropathology, Mainz University Medical Center, Langenbeckstrasse 1, D-55131 Mainz, GermanySearch for more papers by this authorRichard Meyermann MD, Richard Meyermann MD Department of Neuropathology, Eberhard-Karls University, Tübingen, GermanySearch for more papers by this authorRegina Rosin MD, Regina Rosin MD Department of Neurology, Eberhard-Karls University, Tübingen, GermanySearch for more papers by this authorWolfgang Schlote MD, Wolfgang Schlote MD Department of Neuropathology, Edinger Institute, Johann Wolfgang-Goethe University, Frankfurt, GermanySearch for more papers by this author Hans H. Goebel MD, Corresponding Author Hans H. Goebel MD Department of Neuropathology, Mainz University Medical Center, Langenbeckstrasse 1, D-55131 Mainz, GermanyDepartment of Neuropathology, Mainz University Medical Center, Langenbeckstrasse 1, D-55131 Mainz, GermanySearch for more papers by this authorRichard Meyermann MD, Richard Meyermann MD Department of Neuropathology, Eberhard-Karls University, Tübingen, GermanySearch for more papers by this authorRegina Rosin MD, Regina Rosin MD Department of Neurology, Eberhard-Karls University, Tübingen, GermanySearch for more papers by this authorWolfgang Schlote MD, Wolfgang Schlote MD Department of Neuropathology, Edinger Institute, Johann Wolfgang-Goethe University, Frankfurt, GermanySearch for more papers by this author First published: 07 December 1998 https://doi.org/10.1002/(SICI)1097-4598(199705)20:5<625::AID-MUS17>3.0.CO;2-VCitations: 19AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. References 1 Lechner-Scott J, Engelter S, Steck AJ, Dellas S, Tolnay M, Probst A: A patient with cerebral autosomal dominant arteriopathy with subcortical infarcts and leucoencephalopathy (CADASIL) confirmed by sural nerve biopsy. Stroke 1995; 26: 235–236. 2 Ragno M, Tournier-Lasserve E, Fiori MG, Manca A, Patrosso MC, Ferlini A, Sirocchi G, Trojano L, Chabriat H, Salvi F: An Italian kindred with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Ann Neurol 1995; 38: 231–236. 3 Ruchoux M-M, Chabriat H, Bousser M-G, Baudrimont M, Tournier-Lasserve E: Presence of ultrastructural arterial lesions in muscle and skin vessels of patients with CADASIL. Stroke 1994; 25: 2291–2292. 4 Ruchoux M-M, Guerouaou D, Vandenhaute B, Pruvo J-P, Vermersch P, Leys D: Systemic vascular smooth muscle cell impairment in cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy. Acta Neuropathol (Berl) 1995; 89: 500–512. 5 Schröder JM, Sellhaus B, Jörg J: Identification of the characteristic vascular changes in a sural nerve biopsy of a case with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). Acta Neuropathol (Berl) 1995; 89: 116–121. 6 Tournier-Lasserve E, Joutel A, Melki J, Weissenbach J, Mark Lathrop G, Chabriat H, Mas L, Cabanis EA, Baudrimont M, Maciazek J, Bach MA, Bousser MG: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy maps to chromosome 19q12. Nat Genet 1993; 3: 256–259. Citing Literature Volume20, Issue5May 1997Pages 625-627 ReferencesRelatedInformation

We studied the kinematic patterns of gait initiation in 31 patients with Parkinson's disease and in 20 age- and sex-matched normals by using an optoelectronic tracking system (ELITE). Position markers were attached to the skin overlying the ankle, knee, hip, elbow, shoulder, and zygomatic bone. Subjects were instructed to start walking immediately after an acoustic go signal. Gait initiation was defined as the phase between standing motionless and steady-state locomotion. This phase was subdivided into a movement preparation period (the time between go signal and movement onset) and a movement execution period (the time between movement onset and the end of the first stride). Onset and duration of ankle, knee, hip, trunk, and arm motion within the first stride were analyzed. Movement preparation time was significantly increased in Parkinson's disease (p = 0.01), whereas movement execution times were similar in both groups (p = 0.23). Initiation of ankle, knee, hip, arm, and trunk movements was delayed in patients as compared with healthy subjects, but the relative timing and the sequence of submovements was comparable in both groups, indicating that the overall pattern of submovements was preserved in the patients. Our data suggest that gait initiation deficits in Parkinson's disease cannot be explained by a disordered sequence of limb and trunk submovements. More likely, gait initiation problems originate from the basal ganglia's internal cueing deficit for movement sequences, delaying onset and slowing the execution of all subcomponents.

In a controlled clinical study, we investigated the effects of behavioral treatment on postural and gait initiation problems idiopathic Parkinson's disease (PD). Comparable groups of patients received therapy (experimental group, n = 15) and nonspecific psychological treatment (control group, n = 14) for 10 weeks. We monitored various variables reflecting properties of posture and gait initiation by using an optoelectronic motion analyzer (electronic movement analysis system, ELITE). A clinician blind to group membership of the patients assessed PD severity with the United Parkinson's Disease Rating Scale (UPDRS) before and after the treatment period. ELITE measures of postural stability and movement initiation revealed treatment-specific effects. In addition, UPDRS motor scores showed significant improvement only after behavioral treatment. We conclude that behavioral treatment in Parkinson's disease may improve motor disabilities in moderately advanced PD patients.

In order to study the involvement of NMDA-receptor activation in brain development, rat pups were chronically treated with the non-competitive NMDA antagonist MK-801 during the neonatal period. We recorded the cortical EEG at various vigilance states throughout the treatment period. Spectral analysis of the EEG showed reduced power in the delta (δ) frequency range (1.5–4 Hz) during quiet sleep and less power in the theta (Θ) range (4–7 Hz) during REM-sleep in MK-801 animals than in controls. No significant differences were found for the total time spent in each of the different vigilance states. We conclude that chronic MK-801 treatment probably causes a developmental retardation in state-related brain activities.

The effects of psychological treatment of idiopathic Parkinson's Disease (PD) were investigated. Behavioral treatment focusing on control of motor activity was compared to a nonspecific psychological treatment. Patients were randomly assigned to 2 treatment groups with 20 patients in the behavioral group and 21 patients in the control group. The 2 groups were equivalent for age, demographic variables, and duration and severity of the illness. Twenty treatment sessions were held over a period of 10 weeks. Behavioral change was assessed by the Motor Performance Test Series (MPS), the Unified Parkinson's Disease Rating Scale (UPDRS) and by a psychologist's ratings and the patients' self-reports. Unspecific treatment effects were controlled by using several questionnaire measures. Results indicated that only behavioral treatment was effective in reducing tremor and in improving manual dexterity. We conclude that behavioral treatment is an effective supplement to traditional medical treatment with L-Dopa for improving motor performance and reducing tremor in Parkinson's disease.