Kurt Segers

Degenerative Dementias and Their Medical Care in the Movies

Compared with other neurologic problems, few films have been dedicated to degenerative dementia. To our knowledge, this is the first systematic review about the way in which dementia patients and their medical care are described in films. Twenty-four of the 53 relevant films that were found in online movie databases could be viewed. The author describes the demographics of the characters suffering from dementia, the clinical picture including neuropsychiatric manifestations, diagnostic procedures, medical follow-up, pharmacologic and nonpharmacologic treatment and the attitude of the caregivers. Most characters are played by actors in their seventh or eighth decade. There is an overrepresentation of highly educated people. Although the clinical picture is often accurate, some films suggest that even in the late stages of the disease patients have sudden moments of full insight in their disease. Among the neuropsychiatric signs, activity disturbances and aggressiveness are most often described. Few patients seek medical help, only 2 patients take acetylcholinesterase inhibitors and follow-up is absent for 5 of the 11 relevant patients. Only in 10 of 23 films, the term “Alzheimer” is used. Although there is a growing cinematographic interest in Alzheimer patients, even recent films tend to reinforce therapeutic and even diagnostic nihilism.

What belgian neurologists and neuropsychiatrists tell their patients with Alzheimer disease and why: a national survey.

To check their opinions concerning the disclosure of the diagnosis of Alzheimer disease (AD), a questionnaire was sent to all neurologists and neuropsychiatrists currently active in Belgium, excluding neuropediatricians. Of 573 questionnaires, 44% were returned. Sixty-eight percent of the responders always announce the diagnosis to their patients, 24% prefer to reveal the diagnosis only to patients with mild dementia. Doctors who announce the diagnosis to all their patients and who believe that its a benefit for the patient (67%) were more likely to be younger, to be neurologists, and to speak Dutch. The most important arguments in favor of announcing the diagnosis were the patients right to know and the reinforcement of the doctor-patient relationship. The main arguments against revealing the diagnosis were the patients right not to know and fear of provoking a depression. Two-third of the participants informed the patients about the prognosis and natural evolution of AD. These doctors tended to be younger, to be neurologists, and to speak Dutch. Young doctors tend to be more “open” toward their patients concerning the diagnosis of AD, consistent with the current guidelines. The differences between Dutch and French speaking doctors might be partially due to the fact that in French, “démence” has a psychiatric connotation.

Adult polyglucosan body disease masquerading as "ALS with dementia of the Alzheimer type": an exceptional phenotype in a rare pathology.

We describe an exceptional clinical picture, namely, cognitive impairment of the Alzheimer disease type in a man who later developed manifestations typical of amyotrophic lateral sclerosis and who was subsequently found to have adult polyglucosan body disease (APGBD) upon postmortem neuropathologic explorations. The combined occurrence of amyotrophic lateral sclerosis and cognitive impairment of the Alzheimer disease type in APGBD has not been reported before. This case also underlines the diverse clinical presentation of this rare clinicopathologic entity (namely APGBD) and highlights the importance of recognizing the unusual association of clinical features in making the diagnosis.

Clinical utility and applicability of biomarker-based diagnostic criteria for Alzheimer’s disease: a BeDeCo survey

We conducted a survey regarding the medical care of patients with dementia in expert settings in Belgium. Open, unrestricted and motivated answers were centralized, blindly interpreted and structured into categories. The report of the results was then submitted to the participants in subsequent plenary meetings and through email. Fourteen experts responded to the questionnaire, confirming that recent propositions to modify Alzheimer’s disease (AD) diagnostic criteria and options have stirred up debate among well-informed and dedicated experts in the field. The opinions were not unanimous and illustrate how difficult it is to find a standardized method of diagnosing this disease. The responses to the survey suggest that application of a step-by-step pragmatic method is used in practice. Only when the combination of clinical findings and classical structural neuro-imaging is insufficient for a diagnosis or suggests an atypical presentation, additional biomarkers are considered. Interestingly, few differences, if any, were observed between the use of biomarkers in MCI and in AD. In conclusion, the Belgian experts consulted in this survey were generally in agreement with the new diagnostic criteria for AD, although some concern was expressed about them being too “amyloidocentric”. Although the clinical examination, including a full neuropsychological evaluation, is still considered as the basis for diagnosis, most experts also stated that they use biomarkers to help with diagnosis.

Do local meteorological conditions influence skin irritation caused by transdermal rivastigmine? A retroprospective, pilot study.

Objective This retrospective study aimed to evaluate the incidence of transdermal rivastigmine treatment withdrawal secondary to adverse skin reactions among the patients from our Memory Clinic. In addition, we tested whether climatic conditions might have an influence on skin irritations leading to eventual treatment disruption. Methods We performed a retrospective review of patients from the Brugmann University Hospital Memory Clinic having started transdermal rivastigmine between June 2008 and December 2010. Local meteorological data were provided by the Royal Meteorological Institute of Belgium. Results A total of 26.9% of the patients experienced adverse skin reactions at the rivastigmine application site, leading to treatment discontinuation in 19.2% of the cases. Rivastigmine cutaneous tolerability was not found to be related to demographic parameters, Mini Mental Status Examination score, or type of dementia. High temperature and low air humidity during the first month of treatment were found to be associated with a higher incidence of skin reactions and secondary treatment disruption. Conclusions Transdermal rivastigmine induced a higher incidence of cutaneous adverse events than previously reported in a prospective clinical trial. Moreover, it seems that meteorological conditions favoring skin perspiration (high temperature and low air humidity) during the first month of treatment might have an influence on transdermal rivastigmine skin tolerability.

Involvement of Peripheral and Central Nervous Systems in a Valosin-Containing Protein Mutation

Background Inclusion-body myopathy with Pagets disease of the bone and frontotemporal dementia (IBMPFD) is a rare, late-onset autosomal disorder arising from missense mutations in a gene coding for valosin-containing protein. Case Report We report the case of a man carrying the previously described p.Arg159His mutation, who had an unusual axonal sensorimotor neuropathy as the first clinical manifestation of IBMPFD, and for whom diagnosis only became clear 8 years later when the patient developed frontotemporal dementia. Conclusions Peripheral neuropathy is a rare manifestation of IBMPFD. This underdiagnosed disorder should be considered when a patient develops dementia or has signs of Pagets disease.

Can mirtazapine counteract the weight loss associated with Alzheimer disease? A retrospective open-label study.

Weight loss is a frequent complication of Alzheimer disease (AD), associated with increased morbidity and mortality. Increased appetite and weight gain are known side effects of the antidepressant mirtazapine. This analysis was undertaken to assess the safety and potential utility of mirtazapine to counteract weight loss in patients with AD or mixed AD (AD with cerebrovascular lesions). We performed a retrospective analysis of the clinical records of all outpatients attending our memory clinic for AD or mixed AD, who had received mirtazapine (30 mg daily) with the specific purpose of inducing weight or appetite gain. Data were available for a total of 22 patients (mean age, 80.9 y, 86.4% female). The mean weight at baseline was 52.4 kg and the mean BMI was 20.5 kg/m2. 77.3% of the patients had gained weight after 3 months (mean gain, 1.93 kg or 3.9% of initial body weight) and 82.3% after 6 months (2.11 kg or 4.6%). One patient had to discontinue mirtazapine because of daytime sleepiness. Mirtazapine seems to be a safe and useful approach to counteract weight loss in AD, if possible in combination with nonpharmacological interventions. Body weight should be monitored during treatment to avoid excessive weight gain.

Performance of middle-aged and elderly European minority and majority populations on a Cross-Cultural Neuropsychological Test Battery (CNTB)

Abstract Objective: The aim of this study was to examine test performance on a cross-cultural neuropsychological test battery for assessment of middle-aged and elderly ethnic minority and majority populations in western Europe, and to present preliminary normative data. Method: The study was a cross-sectional multi-center study. Tests in the European Cross-Cultural Neuropsychological Test Battery (CNTB) cover several cognitive domains, including global cognitive function, memory, executive functions, and visuospatial functions. Results: A total of 330 participants were included: 14 Moroccan, 45 Pakistani/Indian Punjabi, 41 Polish, 66 Turkish, and 19 former Yugoslavian minority participants, and 145 western European majority participants. Significant differences between ethnic groups were found on most CNTB measures. However, ethnic groups differed greatly in demographic characteristics and differences in test scores were mainly related to educational differences, explaining an average of 15% of the variance. Preliminary multicultural CNTB normative data dichotomized by education and age were constructed using overlapping cells. Applying this normative data across the whole sample resulted in an acceptable number of participants scoring in the impaired range across all ethnic groups. Factor analyses found the CNTB to have a stable and clinically meaningful factor structure. Conclusions: The CNTB represents the first European joint effort to establish neuropsychological measures appropriate for ethnic minority populations in western Europe. The CNTB can be applied in approximately 60 min, covers several cognitive domains, and appears appropriate for assessment of the targeted populations. However, due to the small sample size in some ethnic groups further studies are needed replicate and support this.

Alzheimer's disease and driving: review of the literature and consensus guideline from Belgian dementia experts and the Belgian road safety institute endorsed by the Belgian Medical Association.

Alzheimer’s disease (AD) is a highly prevalent condition and its prevalence is expected to further increase due to the aging of the general population. It is obvious that the diagnosis of AD has implications for driving. Finally, driving discussions are also emotionally charged because driving is associated with independence and personal identity. However, it is not clear how to implement this in clinical practice and the Belgian law on driving is rather vague in its referral to neurodegenerative brain diseases in general nor does it provide clear-cut instructions for dementia or AD compared to for example driving for patients with epilepsy and as such does not prove to be very helpful. The present article reviews what is known from both literature and existing guidelines and proposes a consensus recommendation tailored to the Belgian situation agreed by both AD experts and the Belgian Road Safety Institute endorsed by the Belgian Medical Association. It is concluded that the decision about driving fitness should be considered as a dynamic process where the driving fitness is assessed and discussed early after diagnosis and closely monitored by the treating physician. The diagnosis of AD on itself definitely does not imply the immediate and full revocation of a driving license nor does it implicate a necessary referral for a formal on-road driving assessment. There is no evidence to recommend a reduced exposure or a mandatory co-pilot. A MMSE-based framework to trichotomise AD patients as safe, indeterminate or unsafe is presented. The final decision on driving fitness can only be made after careful history taking and clinical examination, neuropsychological, functional and behavioral evaluation and, only for selected cases, a formal assessment of driving performance.

A Retrospective Belgian Multi-Center MRI Biomarker Study in Alzheimer's Disease (REMEMBER).

Background: Magnetic resonance imaging (MRI) acquisition/processing techniques assess brain volumes to explore neurodegeneration in Alzheimer’s disease (AD). Objective: We examined the clinical utility of MSmetrix and investigated if automated MRI volumes could discriminate between groups covering the AD continuum and could be used as a predictor for clinical progression. Methods: The Belgian Dementia Council initiated a retrospective, multi-center study and analyzed whole brain (WB), grey matter (GM), white matter (WM), cerebrospinal fluid (CSF), cortical GM (CGM) volumes, and WM hyperintensities (WMH) using MSmetrix in the AD continuum. Baseline (n = 887) and follow-up (FU, n = 95) T1-weighted brain MRIs and time-linked neuropsychological data were available. Results: The cohort consisted of cognitively healthy controls (HC, n = 93), subjective cognitive decline (n = 102), mild cognitive impairment (MCI, n = 379), and AD dementia (n = 313). Baseline WB and GM volumes could accurately discriminate between clinical diagnostic groups and were significantly decreased with increasing cognitive impairment. MCI patients had a significantly larger change in WB, GM, and CGM volumes based on two MRIs (n = 95) compared to HC (FU>24months, p = 0.020). Linear regression models showed that baseline atrophy of WB, GM, CGM, and increased CSF volumes predicted cognitive impairment. Conclusion: WB and GM volumes extracted by MSmetrix could be used to define the clinical spectrum of AD accurately and along with CGM, they are able to predict cognitive impairment based on (decline in) MMSE scores. Therefore, MSmetrix can support clinicians in their diagnostic decisions, is able to detect clinical disease progression, and is of help to stratify populations for clinical trials.