Gait abnormalities induced by acquired bilateral pallidal lesions

Background Bilateral pallidal lesions induce a range of cognitive and motor disorders, principally a parkinsonian syndrome in which severe disturbances of gait and gait initiation are frequently reported. However, the precise clinical features of these disorders (and the role of the pallidum therein) remain to be established. Objectives The goal of this study was to characterise gait and gait initiation disorders within the context of a parkinsonian syndrome in patients with acquired, bilateral, pallidal lesions (PAL patients), to compare these disorders to those seen in Parkinson’s disease (PD), and to assess the corresponding physiopathological implications. Patients and methods By using a video motion analysis system (VICON), we studied gait kinematic parameters in two patients presenting with bilateral, pallidal lesions. Kinematic and kinetic parameters were also determined during gait initiation. The two patients were compared with a group of 17 PD patients and to 20 healthy controls. Results In both PAL and PD patients, kinematic parameters (gait and gait initiation) and kinetic parameters (gait initiation) were similarly impaired, evidenced by akinesia (difficulty in initiating gait characterized by impairment of anticipatory postural adjustments). Hypokinesia and bradykinesia (respectively reduced stride length and reduced speed during gait) were also noted. Conclusion The gait and gait initiation disorders seen in cases of bilateral pallidal lesions (namely akinesia, hypokinesia and bradykinesia) are similar to those observed in PD. Subject to confirmation in more extensive studies, we hypothesize that bipallidal patients may present higher level gait disorders,with potential mediation by cognitive impairment.     

The pattern of attentional deficits in Parkinson's disease

BackgroundCognitive impairment without dementia is frequent in Parkinson's disease. It often presents as a dysexecutive syndrome with deficient attentional resource allocation. The nature of attention deficits in Parkinson's disease has rarely been investigated with robust, theory-based tasks. The main objective of the present study was to investigate attention disorders in Parkinson's disease patients by applying a paradigm based on a model of attention. We also sought to identify the main demographic and clinical characteristics associated with attention deficits in Parkinson's disease.MethodsEighty non-demented Parkinson's disease patients and 60 healthy controls participated in the study. Attention was assessed in a computer-controlled reaction time paradigm. The test session comprised a simple reaction time task and four choice reaction time tasks: a go/no-go task, a one-dimension, focused-attention task, a two-dimension, divided-attention task and an alternating task. Performance was assessed by composite measures: (i) cognitive reaction time, corresponding to the difference between the simple reaction time and the choice reaction time in the given condition, and (ii) reaction time variability, corresponding to the sum of the coefficients of variance of the reaction times. Accuracy was also considered.ResultsApart from an overall slowing and greater reaction time variability, Parkinson's disease patients were only significantly impaired in the alternating condition. This set-shifting impairment was associated with their performance in the go/no-go and divided-attention conditions.ConclusionOur systematic assessment of the different attentional subcomponents revealed that mental flexibility is particularly impaired in non-demented Parkinson's disease patients.     

Excretory urography in the emergency department: utility of teleradiology

To test the feasibility of teleradiology for use with excretory urograms obtained in the emergency department in cases for suspected renal colic, radiographs from 43 patients were digitized, transmitted via standard phone lines, and viewed on a video monitor 2 miles distant. Diagnostic interpretations of the video image and the original radiograph were reviewed for degree of concordance. The video and film images of the 27 examinations showing obstruction were interpreted with 100% correlation. Agreement as to the site of obstruction was present in 93% of cases. Only two-thirds of renal and ureteral calculi seen on the radiographs were discerned on the video images, however. Teleradiology proved as useful for ongoing monitoring and modification of these examinations as for initial interpretation.     

Clinical features and genetic analysis of a new form of spinocerebellar ataxia

BACKGROUND: The autosomal dominant cerebellar ataxias (ADCA) are a clinically heterogeneous group of disorders. The mutations for SCA1, SCA2, SCA3, SCA6, SCA7, SCA8, and SCA-12 are identified and caused by an expansion of a CAG or a CTG repeat sequence of these genes. Six additional loci for SCA4, SCA5, SCA-10, SCA-11, SCA-13, and SCA-14 are mapped. The growing heterogeneity of the autosomal dominant forms of these diseases shows that the genetic etiologies of at least 20% of ADCA have yet to be elucidated. METHODS: The authors ascertained and clinically characterized a four-generation pedigree segregating an autosomal dominant phenotype for SCA. Direct mutation analysis, repeat expansion detection analysis, and linkage analysis for all known SCA loci were performed. RESULTS: Direct mutational analysis excluded SCA1, 2, 3, 6, 7, 8, and 12; genetic linkage analysis excluded SCA4, 5,10, 11, 13, and 14, giving significant negative lod scores. Examination of the family showed that all affected members had gait ataxia and akinesia with variable features of dysarthria, hyporeflexia, and mild intellectual impairment. Eye movements were normal. Head MRI showed atrophy of the cerebellum without involvement of the brainstem. In 10 parent-child pairs, median onset occurred 10.5 years earlier in offspring than in their parents, suggesting anticipation. CONCLUSION: This family is distinct from other families with SCA and is characterized by cerebellar ataxia and extrapyramidal signs.     

Parkinson's disease dementia can be easily detected in routine clinical practice

Parkinson's disease (PD) is mainly characterized by its motor manifestations, but it is also frequently associated with dementia. Early diagnosis of PD dementia (PDD) is particularly important because effective cholinesterase inhibitor treatments are available. This study aimed at validating a short procedure for screening for PDD in routine clinical practice and which adopts recently published diagnostic criteria. One hundred eighty‐eight patients with PD participated in the study. The examination procedure comprised three steps: standard clinical examination, a short cognitive function assessment fulfilling the requirements of the Movement Disorders Society (Mini Mental State Examination, five‐word test, word generation task, and impact on daily life, including a questionnaire on compliance with medication) and an extensive evaluation of cognitive functions and behavior. After each step, the suspected presence or absence of dementia was recorded. After the short cognitive function assessment, PDD was suspected in 18.62% of the patients [95% confidence interval (CI): 13.32‐24.93%]. After the extensive assessment, 21.81% (95% CI: 16.13‐28.40%) met the criteria for probable PDD. The short battery's sensitivity and specificity were 65.85% (95% CI = 49.41‐79.92%) and 94.56% (95% CI = 89.56‐97.62%), respectively. A stepwise logistic regression analysis showed that use of a specific cut‐off considerably enhanced the short battery's sensitivity (85.37%, 95% CI = 70.83‐94.43%) without decreasing its specificity (83.67%, 95% CI = 76.69‐89.25%). With an easy‐to‐use, short battery of tests that are commonly used in routine clinical practice, it is possible to diagnose PDD in accordance with reference criteria and with the same sensitivity and specificity as in a more extensive evaluation. © 2010 Movement Disorder Society     

Cognitive impact of mitoxantrone and methylprednisolone in multiple sclerosis: an open label study

Interferons beta have shown some positive effects on cognitive function in multiple sclerosis (MS). The potential immunosuppressive impact of mitoxantrone on cognitive dysfunction in MS has never been evaluated. We assessed changes in cognitive dysfunction in patients with very active MS treated with mitoxantrone combined with methylprednisolone. We assessed a non randomized controlled trial including successively 15 consecutive MS patients. Very active MS was defined by a progression of at least two EDSS points or more than two relapses during the previous year and at least one enhanced lesion after gadolinium infusion on MRI. All patients received a monthly intravenous pulse of mitoxantrone (20mg) for six months with methylprednisolone (1g). Global cognitive efficiency, memory and executive function were assessed before treatment (M0) and after six months (M6) and 12 months (M12) of treatment. To evaluate the learning effect, 15 healthy subjects also participated. A significant improvement in global cognitive efficiency was observed at M6 and was sustained at M12, as a few parameters on memory and executive functions. We suggest that mitoxantrone combined with methylprednisolone has a potential positive effect on cognitive functions.