Thodoros Papapetropoulos

Genetic Analysis of Families with Parkinson Disease that Carry the Ala53Thr Mutation in the Gene Encoding α-Synuclein

We wish to thank Drs. C. Bissas, N. Georgopoulos, P. Ghikas, C. Kremmydas, P. Leonardos, S. Papapetropoulos, and, last but not least, A. Protonotariou, for their great help in sample collection. This work was supported by European Framework Program EPET II grant 236/234/603 to G.M.M.

The effect of vascular disease on late onset Parkinson's disease

The clinical severity of late onset Parkinsons disease (PD) varies from patient to patient and it is further complicated by the increasing prevalence of accompanying disorders in the elderly. We set out to study the impact of ischemic heart disease, minor stroke, hypertension and diabetes mellitus in a group of late onset PD patients (age ≥70 years). Consecutive late onset PD patients seen in the Department of Neurology, Medical School of Patras, Greece were included in this study. We used very strict criteria to eliminate the possibility of including patients with vascular parkinsonism. Comparisons were made between groups of patients suffering with idiopathic Parkinsons disease (IPD) and the above‐mentioned diseases. One hundred and sixty‐seven consecutive late onset PD patients were included in this study. The most common accompanying disorders in our group were hypertension in 31 (18%) of the patients and minor stroke in 20 (12%). The Hoen and Yahr score in late onset IPD patients who suffered from minor stroke, ischemic heart disease or diabetes mellitus was significantly higher when compared with patients without the above disorders. The results clearly suggest that the presence of vascular disease on an IPD patient may aggravate PD severity. In clinical grounds, these findings can be proved significant since early and aggressive prevention of vascular disease and treatment of vascular risk may contribute in controlling symptom severity in PD.

Clinical characteristics of the alpha-synuclein mutation (G209A)-associated Parkinson's disease in comparison with other forms of familial Parkinson's disease in Greece

An Ala53Thr mutation of the α‐synuclein has been recently identified as a rare cause of familial Parkinsons disease (fPD). In the present study, the clinical characteristics of Parkinsons disease (PD) patients with Ala53Thr α‐synuclein mutation (α‐synPD) were compared with fPD patients without any known mutation. An investigator blinded to the results of the genetic analysis examined 15 α‐synPD patients and 43 consecutive fPD patients. Demographic data, age at onset of the illness, duration of the disease and modality of presentation were collected. Segregation ratios for both sexes in individuals at risk of developing α‐synPD were estimated. The Unified Parkinsons disease rating scale (UPDRS) was also completed. The 15 α‐synPD patients were matched for duration of the disease and age at onset with 15 of the 43 fPD patients (MfPD). Comparisons were also made between 14 patients belonging to three multicase families with patterns of inheritance similar to α‐synPD. The α‐synPD patients were significantly younger (mean difference 11.8 years) and showed the first sign of the disease earlier in life (mean difference 12.7 years) as compared with the fPD patients. Tremor at onset was present in only one (6.7%) of the α ‐synPD patients compared with 18 (41.9%) of the fPD patients (P = 0.01). At the time of examination rigidity, postural instability, orthostatic hypotension and the overall clinical severity did not differ significantly either between α‐synPD and fPD or between α‐synPD and MfPD groups. Nevertheless, some clinically relevant trends concerning the psychiatric symptoms and complications of therapy were recognized. The overall clinical severity and the progression of the disease in patients with α‐synPD did not differ from that of the fPD patients. The α‐synPD patients presented the illness at a younger age and also had lower prevalence of tremor when compared with the fPD patients.

Carpal tunnel syndrome and hand configuration.

The likelihood that hand configuration is related to the development of carpal tunnel syndrome (CTS) was examined in this study. Based on neurophysiological criteria, 50 female subjects with CTS and 50 healthy female controls were selected and their external hand dimensions were measured. Our results showed that the palm length and third digit length were significantly shorter and the palm width larger in the subjects with CTS compared with controls. Regression analysis demonstrated that the hand ratio [(palm + third digit length)/palm width] was significantly correlated with median nerve conduction measurements. The hand ratio may be a simple and useful predictive measurement in determining the tendency for CTS.

Guillain–Barré syndrome in Greece: seasonality and other clinico-epidemiological features

The epidemiologic and clinical features of Guillain–Barré syndrome (GBS) during a 13‐year period in a region of south‐western Greece with an average population of 819 009 inhabitants are described. Clinical records of all patients between 1 January 1989 and 31 December 2001 who were admitted in the two referring hospitals of the above district and fulfilled the criteria for idiopathic GBS were reviewed. Overall 105 patients were identified. The age adjusted to European population incidence rate per 100 000 person‐years was 1.02 (95% CI: 0.84–1.24) and a male preponderance was found. The highest number of cases (35; 33.3%) occurred in spring and the lowest (17; 16.2%) in autumn, although this tendency did not reach a significant level. Cases with and without preceding illness were similarly distributed in the seasons. Electrophysiologic abnormalities of axonal type were found in 6.1% of patients. The mortality rate was 2.8% and the long‐term outcome 5%. The incidence of GBS was relatively low and the prognosis was close to the best reported. Spring clustering has also been observed in other countries with mild climate.

Polymyositis and pregnancy: report of a case with three pregnancies

Reports of polymyositis and dermatomyositis during pregnacy are uncommon and the available data referring to the possibility of recurrence as well as to the risks for the pregnant woman and the fetus are limited. The association of polymyositis-dermatomyositis and pregnancy is very rare, and this has been attributed to the low percentage (14%) of cases in which the disease begins during the reproductive period of a woman’s life1; it is also probable that family planning policy after the clinical manifestation of the disease lowers the association of polymyositis-dermatomyositis and pregnancy. A 35 year old woman was admitted to our neurological department (May 1992) after 16 weeks of pregnancy, with a six week history of fatigue, myalgia, and proximal muscle weakness of all four limbs which was progressively worsening. At the age of 17 years she had been investigated (blood biochemistry, EMG, and muscle biopsy) at the University Department of Neurology in Athens where the diagnosis of polymyositis (first episode) was established. …

Pattern reversal visual evoked potentials in minor head injury

Pattern reversal visual evoked potentials (PR-VEPs) have been recorded in 50 patients with minor head injury (MHI) on days 1 and 30 after trauma and the data compared to 20 normals. None of the patients had visual complaints. The aim was to investigate a possible visual pathway affection in MHI and test the usefulness of PR-VEPs as an objective noninvasive tool in the detection of a possible subclinical affection of the visual system in MHI. P100 latency and amplitude had no significant difference compared to normals. Comparison of patient data on days 1 and 30 after trauma showed a significant latency decrease and amplitude increase on day 30, compared to day 1. These alterations were not age dependent. Our data suggest affection of the human visual pathway in MHI. PR-VEP recording seems to be a useful, objective, noninvasive tool, helping to identify possible subclinical affections of the visual pathway in MHI.

A case of fatal progressive neuropathy Delayed consequence of multiple bites of a non-venomous snake?

Neurotoxicity is a common feature of the acute syndrome of systemic snake envenomation. In this report a patient with delayed onset of neurological manifestations after multiple bites of a non-venomous snake is described. The neurological cascade consisted of segmental painful muscle spasms presented several days after the snake bites, which gradually subsided and within a month was followed by muscle weakness in the legs. The latter progressed to flaccid quadriplegia with additional respiratory and autonomic failure leading to death after total disease duration of 5.5 months. The possibility that this unique neurological syndrome of fatal progressive neuropathy could be caused by either tetanus or an immune reaction initiated by snakebites is discussed.

Is the common carotid artery intima–media thickness associated with functional outcome after acute ischaemic stroke?

Background: Common carotid artery intima–media thickness (CCA-IMT) is an independent and early marker of generalised atherosclerosis. Brain affected by atherosclerosis may be more vulnerable to an ischaemic insult. Objective: To investigate the association between CCA-IMT and functional outcome after an acute ischaemic stroke. Design: Prospective cohort analysis. Methods: 284 consecutive patients (mean (SD) age, 68.7 (12.7) years, 126 (44%) female) with an acute ischaemic stroke had carotid ultrasonography, carried out by a single operator. Demographic data, vascular risk factors, initial stroke severity, and brain imaging findings were recorded. Outcome was assessed at seven days from stroke onset, at discharge from hospital, and at one year post-stroke. Results: CCA-IMT was not significantly associated with adverse short or long term functional outcome in univariate analysis, or after adjustment in a multivariate logistic regression analysis for demographic data, initial stroke severity, conventional vascular risk factors, and the characteristics of the ischaemic lesion. Age and initial stroke severity were the only independent predictors of outcome. Conclusions: CCA-IMT was not associated with adverse functional outcome after an ischaemic stroke. Adding CCA-IMT in a prediction model for stroke outcome would probably not improve the power of the model.

In Vivo Platelet Activation in Ischemic Stroke Patients

To the Editor: Recently, Marquardt et al1 addressed the important issue of evaluating markers for monitoring patients after ischemic stroke. They evaluated the expression of the platelet activation markers CD62P and CD63 by flow cytometry for 90 days after ischemic stroke. They showed that although both markers are initially increased, the expression of CD62P decreases rapidly and by day 14 is no longer significantly increased, while that of CD63 remains elevated for the study period. The expression of both antigens was independent of the inflammation markers studied, indicating that expression of platelet neoantigens might be used as an additional marker for monitoring these patients. Expression was not influenced by the different medications for secondary stroke prevention, suggesting that they might reflect mechanisms that could predict future vascular events. These results are very interesting because definition of reliable laboratory markers for diagnosing ischemic stroke, monitoring treatment, and predicting recurrence is a very important issue, particularly in the view of new and effective treatments that, however, are associated with significant complications.2,3 We performed a study investigating the expression of CD62P and CD63 antigens on circulating platelets in patients with acute ischemic disease on admission and after initiation of …