Background: Evidence does not support the view that Parkinson disease (PD) rep resents an accelerated aging process; however, the additional contribution of ag ing to the severity of different motor signs in patients ...Background: Evidence does not support the view that Parkinson disease (PD) rep resents an accelerated aging process; however, the additional contribution of ag ing to the severity of different motor signs in patients with PD is not known. T his knowledge may have implications for clinical trials of neuroprotective agent s in PD. Objective: To investigate the contribution of aging to the severity of the different motor signs of idiopathic PD. Setting: Center for Parkinson Diseas e and Other Movement Disorders of the Columbia University Medical Center and a n eurology clinic that primarily served individuals from the Washington Heights-I nwood community in New York City. Patients: Sample of patients with a wide range of disease duration and age. Design: Cross-sectional clinic-based study. Pati ents with PD were evaluated using the Unified Parkinson Disease Rating Scale (UP DRS). The total UPDRS motor score was divided into 6 motor domains (tremor, rigi dity, bradykinesia, facial expression, speech, and axial impairment) and 2 subsc ores that represented predominantly dopaminergic (subscore A: tremor, rigidity, bradykinesia, and facial expression) and nondopaminergic (subscore B: speech and axial impairment) deficiency. Analyses were performed using linear regression models with the UPDRS motor domains and subscores as the outcomes. The variation (adjusted R2)of the outcome variables explained by the inclusion o f disease duration in the models, adjusting for sex, years of education, levodop a dosage, and use of other antiparkinsonian medications, was calculated. The add itional variation explained by adding age at examination to the models was used to gauge the contribution of aging to each motor domain and subscore of the UPDR S. Results: A total of 451 patients participated in the study. Mean age at exami nation was 62.0 years (SD, 12.6 years; median, 62.0 years; range, 18-93 years), and mean disease duration was 7.2 years (SD, 5.9 years; median, 5.6 years; rang e, 0.1-41.6 years). The additional variation of the展开更多
Background: Recently, six molecular subtypes of sporadic CJD (sCJD) have been identified showing differences regarding the disease course, neuropathologic lesion patterns, and sensitivity to diagnostic tools. Only iso...Background: Recently, six molecular subtypes of sporadic CJD (sCJD) have been identified showing differences regarding the disease course, neuropathologic lesion patterns, and sensitivity to diagnostic tools. Only isolated cases of the rare VV1 type have been reported so far. Objective: To describe the clinical characteristics and neuropathologic lesion profiles in nine cases. Methods: In the years 1993 until late 2003, 571 definite neuropathologically confirmed cases of sporadic CJD were identified in Germany. Of these, nine were homozygous for valine and displayed type 1 of the pathologic PrPSc in the brai n (VV1 type). Results: The authors describe eight men and one woman belonging to the VV1 type. All patients were relatively young at disease onset (median 44 years vs 65 years in all sCJD) with prolonged disease duration (median 21 months vs 6 months in all sCJD). During the initial stages, their main clinical signs were personality changes and slowly progressive dementia as well as focal neurologic deficits. None of the nine VV1 patients had periodic sharp-wave complexes (PSWCs) in the EEG. Only two out of seven displayed the typical signal increase of the basal ganglia on MRI, whereas signal increase of the cortex was seen in all patients. The 14-3-3 protein levels were elevated in CSF in all cases tested. Conclusions: The clinical diagnosis of the VV1 type of sCJD can be best supported by the 14-3-3 test and cortical signal increase on MRI. Because of the young age at onset vCJD is sometimes suspected as a differential diagnosis. MRI plays an important role in differentiating these two disease types and should be performed early during the disease course.展开更多
Purpose. To ascertain the therapeutic effect of periocular corticosteroids in diabetic papillopathy. Methods. Prospec-tively, five consecutive adult-onset diabetic patients with symptomatic diabetic papillopathy under...Purpose. To ascertain the therapeutic effect of periocular corticosteroids in diabetic papillopathy. Methods. Prospec-tively, five consecutive adult-onset diabetic patients with symptomatic diabetic papillopathy underwent visual fields and fluorescein angiography before and after superonasal subtenon injection of corticosteroids. Results. The median duration of papillopathy was 2.5 weeks by ophthalmoscopy and 3 weeks by fluorescein angiography. The median recovery time of best-spectacle-corrected visual acuity was 2 weeks. Two patients developed sequential diabetic papillopathy, and both reported faster visual recovery and better subjective vision in treated eyes. In these two patients, the final best-spectacle-corrected visual acuity and visual evoked responses were comparable between the two eyes, while automated visual fields were less constricted in treated eyes. Complications included ocular hypertension, mild progression of cataract, and mild ptosis in one patient each. Conclusions. Periocular corticosteroids shortened the duration of diabetic papillopathy from a reported median of 5 months to 3 weeks in the present uncontrolled observational study, partly by their angiostatic and antioedema effects at the level of the anterior optic nerve. Intraocular pressure needs to be monitored in eyes receiving periocular corticosteroids.展开更多
文摘Background: Evidence does not support the view that Parkinson disease (PD) rep resents an accelerated aging process; however, the additional contribution of ag ing to the severity of different motor signs in patients with PD is not known. T his knowledge may have implications for clinical trials of neuroprotective agent s in PD. Objective: To investigate the contribution of aging to the severity of the different motor signs of idiopathic PD. Setting: Center for Parkinson Diseas e and Other Movement Disorders of the Columbia University Medical Center and a n eurology clinic that primarily served individuals from the Washington Heights-I nwood community in New York City. Patients: Sample of patients with a wide range of disease duration and age. Design: Cross-sectional clinic-based study. Pati ents with PD were evaluated using the Unified Parkinson Disease Rating Scale (UP DRS). The total UPDRS motor score was divided into 6 motor domains (tremor, rigi dity, bradykinesia, facial expression, speech, and axial impairment) and 2 subsc ores that represented predominantly dopaminergic (subscore A: tremor, rigidity, bradykinesia, and facial expression) and nondopaminergic (subscore B: speech and axial impairment) deficiency. Analyses were performed using linear regression models with the UPDRS motor domains and subscores as the outcomes. The variation (adjusted R2)of the outcome variables explained by the inclusion o f disease duration in the models, adjusting for sex, years of education, levodop a dosage, and use of other antiparkinsonian medications, was calculated. The add itional variation explained by adding age at examination to the models was used to gauge the contribution of aging to each motor domain and subscore of the UPDR S. Results: A total of 451 patients participated in the study. Mean age at exami nation was 62.0 years (SD, 12.6 years; median, 62.0 years; range, 18-93 years), and mean disease duration was 7.2 years (SD, 5.9 years; median, 5.6 years; rang e, 0.1-41.6 years). The additional variation of the
文摘Background: Recently, six molecular subtypes of sporadic CJD (sCJD) have been identified showing differences regarding the disease course, neuropathologic lesion patterns, and sensitivity to diagnostic tools. Only isolated cases of the rare VV1 type have been reported so far. Objective: To describe the clinical characteristics and neuropathologic lesion profiles in nine cases. Methods: In the years 1993 until late 2003, 571 definite neuropathologically confirmed cases of sporadic CJD were identified in Germany. Of these, nine were homozygous for valine and displayed type 1 of the pathologic PrPSc in the brai n (VV1 type). Results: The authors describe eight men and one woman belonging to the VV1 type. All patients were relatively young at disease onset (median 44 years vs 65 years in all sCJD) with prolonged disease duration (median 21 months vs 6 months in all sCJD). During the initial stages, their main clinical signs were personality changes and slowly progressive dementia as well as focal neurologic deficits. None of the nine VV1 patients had periodic sharp-wave complexes (PSWCs) in the EEG. Only two out of seven displayed the typical signal increase of the basal ganglia on MRI, whereas signal increase of the cortex was seen in all patients. The 14-3-3 protein levels were elevated in CSF in all cases tested. Conclusions: The clinical diagnosis of the VV1 type of sCJD can be best supported by the 14-3-3 test and cortical signal increase on MRI. Because of the young age at onset vCJD is sometimes suspected as a differential diagnosis. MRI plays an important role in differentiating these two disease types and should be performed early during the disease course.
文摘Purpose. To ascertain the therapeutic effect of periocular corticosteroids in diabetic papillopathy. Methods. Prospec-tively, five consecutive adult-onset diabetic patients with symptomatic diabetic papillopathy underwent visual fields and fluorescein angiography before and after superonasal subtenon injection of corticosteroids. Results. The median duration of papillopathy was 2.5 weeks by ophthalmoscopy and 3 weeks by fluorescein angiography. The median recovery time of best-spectacle-corrected visual acuity was 2 weeks. Two patients developed sequential diabetic papillopathy, and both reported faster visual recovery and better subjective vision in treated eyes. In these two patients, the final best-spectacle-corrected visual acuity and visual evoked responses were comparable between the two eyes, while automated visual fields were less constricted in treated eyes. Complications included ocular hypertension, mild progression of cataract, and mild ptosis in one patient each. Conclusions. Periocular corticosteroids shortened the duration of diabetic papillopathy from a reported median of 5 months to 3 weeks in the present uncontrolled observational study, partly by their angiostatic and antioedema effects at the level of the anterior optic nerve. Intraocular pressure needs to be monitored in eyes receiving periocular corticosteroids.
