Coping et qualité de vie dans la sclérose en plaques et la maladie de Parkinson : une réflexion clinique

Objective

In neurological disorders, such as multiple sclerosis (MS) and Parkinson’ s disease (PD), psychological and adaptive processes have not been extensively investigated, it has, however, been demonstrated that they have a great impact on “quality of life” (QoL). Interestingly, even though these two diseases affect people of different ages, both of them have a substantial impact on mood and QoL. In these two diseases, the authors objective was to analyse the style of coping in relation with the QoL taking depression and anxiety into account.

Methods

Two hundred and seventy MS and PD subjects were seen for a semistructured interview in order to collect sociodemographic and clinical information, after which there was an assessment of their mental and cognitive states using: the Mini International Neuropsychiatric Interview (MINI), the Montgomery and Asberg Depression Rating Scale (MADRS), the Depressive Mood Scale (EHD), the Hamilton Anxiety scale (HAMA) and the Frontal Assessment Battery (FAB). Then, all subjects completed three self-report questionnaires; two about coping strategies: the Ways of Coping Checklist (WCC), the Coping with Health, Injuries and Problems scale (CHIP), and one about QoL: the SEP 59 for MS and the French version of PDQ-39 for PD.

Results

The studies show that the psychosocial dimension of QoL is preserved in the two diseases studied. They also demonstrated that in MS and PD the two factors associated with a poor QoL are depression and emotion-focused coping strategies. Furthermore, these variables are highly dependent on the clinical courses in MS and motor signs in PD. However, the MS patients tended to be more irritable and to lose the control of their emotions more easily than PD patients.

Discussion

The report discusses the importance, first, of identifying these manifestations in patients with MS or PD and, second, of offering patients interventions tailored to the characteristics of each disorder. The authors propose some examples of psychotherapy, which could be used with neurological patients. The studies show that it is also important to work with MS and PD patients on both the expression and the management of their emotions in connection with their diseases.     

Maladie de Parkinson : de la physiopathologie des troubles psychiques à la maîtrise du traitement dopaminergique

Introduction

Behavioral changes in Parkinson's disease are complex and their pathophysiology is not yet fully understood. The dopaminergic system seems to play a major role and most of the behavioral disorders in Parkinson's disease can be classified into either hypodopaminergic if related to the disease itself or hyperdopaminergic if related to dopaminergic treatment.

State of the art

Subthalamic stimulation, which enables withdrawal of dopaminergic medication at an advanced stage in the disease, provides a model for the study of certain nonmotor, dopamine-sensitive symptoms. Such a study has shown that apathy, which is the most frequent behavioral problem in Parkinson's disease, is part of a much broader hypodopaminergic behavioral syndrome which also includes anxiety and depression. Nonmotor fluctuations - essentially fluctuations in the patient's psychological state - are an expression of mesolimbic denervation, as shown in positron emission tomography. Drug-induced sensitization of the denervated mesolimbic system accounts for hyperdopaminergic behavioral problems that encompass impulse control disorders that can be alternatively classified as behavioral addictions. The association of impulse control disorders and addiction to the dopaminergic medication has been called dopamine dysregulation syndrome. While L-dopa is the most effective treatment for motor symptoms, dopamine agonists are more effective in improving the nonmotor levodopa-sensitive symptoms. On the other hand, L-dopa induces more motor complications and dopamine agonist more behavioral side effects. There is increasing data and awareness that patients’ quality of life appears to be dictated by hypo- and hyperdopaminergic psychological symptoms stemming from mesolimbic denervation and dopaminergic treatment rather than by motor symptoms and motor complications related to nigrostriatal denervation and dopaminergic treatment.

Perspectives

Better management requires knowledge of the clinical syndromes of hyper- and hypodopaminergic behaviors and nonmotor fluctuations, a better understanding of their underlying mechanisms and the development of new evaluation tools for these nonmotor symptoms.

Conclusions

The neurologist who strives to gain mastery of dopaminergic treatment needs to fine tune the dosage of levodopa and dopamine agonists on an individual basis, depending on the presence of motor and nonmotor signs respectively.     

Stimulation cérébrale profonde et troubles de la marche dans la maladie de Parkinson

Introduction

Gait disorders and freezing of gait (FOG) are seen in most patients with advanced Parkinson disease. Response to levodopa and deep brain stimulation is variable across patients.

State of art

Thalamic stimulation is ineffective on gait and can even worsen balance when bilaterally applied. Pallidal stimulation moderately improves gait disorders and FOG although this effect tends to wane after three to five years. Stimulation of the subthalamic nucleus (STN) improves levodopa-responsive gait disorders and FOG. However, some patients worsen after STN stimulation and others are better improved under levodopa than under STN stimulation. Synergistic effects of the two treatments have been reported. As for pallidal stimulation, there is a failure of long-term STN stimulation to improve gait, probably due to the involvement of non-dopaminergic pathways as the disease progresses. Levodopa-resistant gait disorders and FOG do not usually benefit from STN stimulation. In the rare cases of levodopa-induced FOG, STN stimulation may be indirectly effective, as it enables reduction or arrest of the levodopa treatment.

Perspectives

Pedunculopontine nucleus stimulation has recently been performed in small groups of patients with disabling gait disorders and FOG. Although encouraging, the first results need to be confirmed by controlled studies involving larger series of patients.

Conclusions

Overall, gait disorders remain a motor PD symptom that is little improved, or only temporarily, by current pharmacological and surgical treatments. Patient management is complex.     

Diagnostic performance of iodine-123-metaiodobenzylguanidine scintigraphy in differential diagnosis between Parkinson's disease and multiple-system atrophy: a systematic review and a meta-analysis

Background and purpose

This study was designed to review the diagnostic performance of iodine-123-metaiodobenzylguanidine (MIBG) scintigraphy in differential diagnosis between Parkinson's disease (PD) and multiple-system atrophy (MSA).

Methods

A comprehensive computer literature search of studies published through March 2011 regarding MIBG scintigraphy in patients with PD and MSA was performed in PubMed/MEDLINE and Embase databases. Only studies in which MIBG scintigraphy was performed for differential diagnosis between PD and MSA were selected. Pooled sensitivity and specificity of MIBG scintigraphy were presented with a 95% confidence interval (CI). The area under the ROC curve was calculated to measure the accuracy of MIBG scintigraphy in differential diagnosis between PD and MSA.

Results

Ultimately, we identified 12 studies comprising a total of 1226 patients (593 patients with PD, 117 patients with other Lewy body disease, 129 patients with MSA, and 387 patients with other diseases). The pooled sensitivity of MIBG scintigraphy to detect PD was 89% (95% CI: 86-91%); the pooled specificity of MIBG scintigraphy to discriminate between PD and MSA was 77% (95% CI: 68-84%). The area under the ROC curve was 0.93.

Conclusions

MIBG scintigraphy is an accurate test for PD detection and differential diagnosis between PD and MSA; this method shows high sensitivity and adequate specificity in this field. Nevertheless, possible causes of false negative and false positive findings should be considered when interpreting the scintigraphic results.     

Anévrismes intracrâniens et sclérose tubéreuse de Bourneville : une association rare

Introduction

Tuberous sclerosis is an autosomal dominant inherited phakomatosis. It is associated with a wide variety of central nervous system abnormalities, but intracranial aneurysms are rare.

Case report

We report a 34-year-old patient fulfilling the diagnostic criteria of tuberous sclerosis in association with intracranial aneurysm.

Discussion

This association has been reported in only 17 other cases of tuberous sclerosis. We discuss the etiopathogenic mechanisms, preferential localizations and the various therapeutic propositions.     

Intérêt de l’analyse factorielle discriminante pour classer les syndromes parkinsoniens à partir de la tomoscintigraphie d’émission monophotonique

Introduction

The objective was to assess the value of single photon emission computerized tomography (SPECT) and factorial discriminant analysis (FDA) in the differential diagnosis of Parkinson's disease (PD), progressive supranuclear palsy (PSP), and corticobasal degeneration (CBD).

Patients and methods

Sixty-two patients with clinical diagnoses of either CBD, PSP or PD were studied using brain HmPaO-SPECT. Thirteen pairs of regions of interest (ROIs) were drawn on the slices located 50 mm and 90 mm above the canthomeatal plane. Twenty-six uptake indices and 13 asymmetry indices were determined. FDA was performed in order to determine whether or not the patients could be classified into the correct clinical group on the basis of SPECT data alone. The most discriminant parameters were used to generate two predictive scores, which were tested in a second group of 15 patients.

Results

FDA of all 39 variables correctly classified all the patients. A subset of 10 variables was used to build predictive scores, which correctly classified 90% of PD patients, 100% of PSP patients and 86% of CBD patients. When tested in the validation group of 15 patients, these predictive scores correctly classified 87% of the individuals. The frontal medial, temporoparietal and parietal regions were the most discriminant.

Conclusion

Using SPECT data alone, this study enabled us to distinguish between PD, PSP and CBD in patients with clear clinical presentations of the diseases in question. This novel, statistical approach provides reliable information. However, a prospective study dealing with de novo parkinsonian syndromes will be necessary.     

Le suicide dans la maladie de Huntington : état actuel des connaissances

Objectives

In 1872, George Huntington was the first to describe a genetic disease combining three types of disorder symptoms viz: motor, cognitive - evolving to cortical dementia and psychiatric. The purpose of this paper is to provide a selective review of the major issues and findings concerning suicide in Huntington's disease. The aim was to understand why patients with Huntington's disease have a higher suicide rate than those suffering from other neuro-developmental diseases.

Materials and methods

The present review is based on a MEDLINE survey of the relevant literature. The terms used in the search were: “Suicide”, “predictive genetic testing”, “suicidal risk factors” and “suicide attempt” all in combination with “Huntington's disease”. All abstracts were read and potentially relevant articles were examined in full. Various other important cross-references were included.

Results

Most of the authors found that suicidal occurrence in Huntington patients was four times higher than that in the general population. No specific individual risk factor was found except a lack of offspring and of psychological support. This review also considered the patient's attitude towards predictive genetic testing and its results. Reactions varied from psychological relief to paradoxical suicidal resurgence.

Conclusion

This paper emphasizes the need of appropriate psychiatric care for Huntington's patients in order to try to prevent suicidal behaviour.     

Quality of life in patients with idiopathic subclavian vein thrombosis

Introduction

Idiopathic subclavian vein thrombosis (SVT) is a rare disease but these otherwise healthy patients often suffer from prolonged clinical manifestations. The aim of this retrospective study was to assess the range and severity of thrombosis-related disability of the upper extremity in patients after an episode of documented idiopathic SVT in the long-term follow-up.

Materials and Methods

The quality of life (QoL) of 37 patients with documented idiopathic SVT was assessed by two standardized questionnaires (DASH and SF 36). The DASH and SF-36 questionnaire each use a 100 point scale. 0 stands for uncompromised functioning, 100 for maximum limitation in the DASH, while in the SF-36 0 marks the lowest rating of QoL and 100 indicates the best imaginable quality of life.

Results

Mean follow-up time was 120 ± 80.1 months (range: 14 to 286 months). The mean DASH score was 10.7 ± 12 and the mean scores for the SF-36 dimensions Physical Component Summary (PCS) and Mental Component System (MCS) were 52 ± 9.3 and 46.3 ± 9.5, respectively.

Conclusions

Patients suffering from idiopathic SVT report good overall QoL judged by the mean DASH and satisfactory QoL by the SF-36 score in the long-term follow-up. These patients deal well with their physical limitations.     

Polymyosite révélant un syndrome de Gougerot-Sjögren

Introduction

Near 10 to 20% of patients with myositis have another systemic, sometimes inaugural, disease.

Case report

A 48-year-old woman was admitted with progressive hypoesthesia in V2 and V3 areas on both sides, difficulties to chew and swallow and then, proximal and axial muscular deficiency, with weight loss. Brain MRI showed gadolinium-enhanced trigeminal nerves and biological tests revealed anti-SSA and anti-Pm/Scl antibodies and a grade IV in Chisholm scoring system on the labial salivary gland biopsy. Neurophysiological studies revealed a myogenic pattern on tibialis anterior muscles and a muscle biopsy confirmed the diagnosis of polymyositis.

Conclusion

The diagnosis of primitive Sjogren's syndrome was suspected because of the association of bilateral trigeminal neuropathy and anti-SSA and anti-Pm/Scl antibodies.     

La jeune fille aux piercings: Revue de la littérature à partir d’un cas de piercings multiples

Background

Body piercing is on the rise in Western societies. Its high prevalence among psychiatric patients draws attention to the relationship between body piercing and psychopathology.

Method

A case study concerning a young patient with multiple body piercing followed by a review of the existing literature concerning the psychopathology of body piercing.

Results

In the case study described, body piercing is symptomatic of the patient's borderline personality disorder (BPD). Current research emphasizes its association with high risk and suicidal behaviours, violence and addictions. However, little data is available on the relationship with specific personality disorders.

Conclusion

The high prevalence of BPD symptoms among pierced populations suggests an association with this disorder. Further research, however, is necessary to confirm this hypothesis. When confronted with multiple piercings, practitioners should investigate associated symptoms and disorders.     

Démence à corps de Lewy atypique révélée par une glossodynie et des troubles pseudo-psychiatriques

Introduction

Among the degenerative diseases of the nervous system, Lewy body disease has the most psychiatric symptoms especially hallucinations, delusion and identification disorders.

Case report

We report a case of Lewy body disease that started with a burning mouth syndrome for three year as the only symptom before the development of a pseudo-psychiatric syndrome (melancholy and Capgras). None of the usual cardinal criteria were present. MRI, cerebrospinal fluid, and DAT scan^® findings enabled the diagnosis.

Conclusion

The dopaminergic hypothesis put forward in some cases of burning mouth syndrome might explain this symptom in Lewy body disease.     

Leucoencéphalopathie multifocale progressive atypique chez un patient atteint de sarcoïdose médiastinopulmonaire

Introduction

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disease of central nervous system due to the JC virus. PML generally occurs in immunocompromised hosts and has a fatal outcome.

Observation

We report a case of an atypical PML in a patient with pulmonary sarcoidosis: MRI showed multifocal and punctate contrast enhancements. The diagnostic was made by brain biopsy.

Conclusion

The pathophysiology of this association is probably related to the immunodepression induced by sarcoidosis.     

Apport d’une démarche électroclinique dans le diagnostic de l’épilepsie chez une population de sujets âgés confus

Background

Delusion symptoms often occur in old people; epilepsy is one of the main reasons behind these acute episodes. Current guidelines and recommendations from the Academy of Medicine have proposed a double clinical and electroencephalographic approach. Recently, a working group of French experts has issued an electro-clinical scale. The aim of our study was to compare the usual approach with the new one based on the electro-clinical score.

Method

All EEG requests performed since December 2008 in Bretonneau Hospital for elderly people aged over 75 years for delusion syndromes were retained for this study.

Results

One hundred and fifteen old patients from a geriatric-hospital (age 83.5 ± 6.06 years) were included in this protocol. The classical diagnostic process yielded the diagnosis of epilepsy for 50 subjects. The electro-clinical scale confirmed the diagnosis of epilepsy in 30 patients and ruled it out in 29 patients.

Conclusion

This study underscores the importance of evidence-based medicine for the diagnosis of epilepsy in old people and points out the underuse of the new technical tool, EEG-monitoring, for the management of these patients.     

Les dérivés conscients des mécanismes de défense au questionnaire de Bond (DSQ 40) : stabilité ou variabilité entre une situation non anxiogène et une situation anxiogène

Introduction

The aim of this research was to test the stability or variability of conscious by-products of defense mechanisms, evaluated with the Bond Defense Style Questionnaire (DSQ 40) so as to integrate them as transactional variables of stress in the model of Lazarus and Folkman.

Participants

One hundred and sixty-one college girls (first-year students in a French university) engaged in psychology studies participated in this research. They participated in two collective sessions: a first session took place in November in a non-stressful situation, and a second session during the January examination week.

Instruments

Two questionnaires were used twice in this experiment: (a) the Anxiety State Questionnaire of Spielberger (STAI-Y) which evaluates the anxiety-state and anxiety-traits; (b) the DSQ 40, which estimates 20 conscious by-products of defense mechanisms and distinguishes three factors corresponding to the mature, neurotic and immature styles according to the factorial analyses of Guelfi and al.

Hypotheses

Two hypotheses were formulated: (a) a first hypothesis postulates that an examination situation increases the State Anxiety score; (b) a second hypothesis postulates a variability of defense mechanisms assessed by Bond's questionnaire (DSQ 40). It verified that the scores of the Bond defensive style questionnaire are significantly different in the two sessions.

Results

The mean state-anxiety score is higher in the second session. The great majority of students are described as more anxious during the week of examinations than during the previous month. The first hypothesis is then validated. The situation of evaluation of the university performance appears as a situation more stressful than the courses period. The three defense mechanism factors vary in a significant way from the first to the second session. In the second session the scores in mature and neurotic defense mechanisms increased significantly whereas the average scores in immature defense mechanisms decreased significantly. The majority of the 20 defense mechanisms are not stable between both situations. Indeed, 17 conscious by-products of defense mechanisms differ significantly in both sessions and only three defense mechanisms show a certain stability. Eleven defensive styles (55 %) decreased, six increased (30 %) and three remained stable (15 %). The instability of conscious by-products of defense mechanisms between the two sessions was confirmed by correlations analyses.

Discussion

The results show that the instability between situations is not limited to three categories of defenses (mature, neurotic and immature): 17 out of 20 mean scores in defense styles differed significantly. Conscious by-products of defense mechanisms are, indeed, influenced by situational factors.     

Les maladies neurologiques repérées lors de la consultation multidisciplinaire de la chute du CHRU de Lille (France)

Background

People with neurological disorders including stroke, dementia, Parkinson's disease, and polyneuropathy are known to have an increased risk of falls.

Objective

To evaluate the prevalence and nature of neurological risk factors among the patients attending the Multidisciplinary Falls Consultation of the University Hospital of Lille (France), and to analyze the characteristic features of patients termed “neurological fallers” with neurological risk factors.

Methods

The study included 266 consecutive patients who were initially assessed by a geriatrician, a neurologist and a physiatrist, and again, six months later, by the same geriatrician.

Results

Two out of three patients had neurological signs that can be regarded as neurological risk factors of falling. These neurological signs had not been diagnosed before the consultation in 85% of cases. The most common conditions were deficit of lower extremity proprioception (59% of patients) and cognitive impairment (43%). The most frequently evoked neurological diseases were dementia (40% of patients), polyneuropathy (17%) and stroke (8%). Compared with other patients, “neurological fallers” were more frequently living in a nursing home, had lower ADL and MMSE scores at baseline, had experienced more falls in the six preceding months, had a lower probability of having a timed Up-and-Go test less than 20 seconds and a single limb stance equal to 5 seconds. In the follow-up, “neurological fallers” reported hospitalizations more often.

Conclusion

The findings show that a large proportion of old persons presenting at the Multidisciplinary Falls Consultation have unrecognized neurological disorders. Comprehensive neurological examination including an evaluation of cognition is required in every elderly faller.     

Diplégie faciale au cours d’une neuropathie démyélinisante inflammatoire aiguë secondaire à une varicelle

Introduction

Primo-infection by varicella-zoster virus (VZV) may be associated with several neurologic complications. Bilateral facial palsy is a rather uncommon manifestation.

Case report

We report the case of a 38-year-old woman who developed bilateral facial diplegia and paresthesia affecting all four limbs with subacute onset several days after varicella virus primoinfection. Ancillary tests showed hyperproteinorachia and signs of demyelinating polyneuropathy in nerve conduction tests. The diagnosis of Guillain-Barré syndrome was retained and a treatment with intravenous immunoglobulines was started, leading to progressive improvement.

Conclusion

Immunotherapy is a possible therapeutic approach in the context of neurologic postinfectious complications after VZV infection where an underlying mechanism is probable.     

Neuropathie motrice multifocale : existe-t-il une altération de la conduction sensitive au long cours ? Une étude rétrospective chez 21 patients

Introduction

Multifocal motor neuropathy is a well described condition characterized by slowly progressive, predominantly distal, asymmetric limb weakness and wasting, predominantly in the arms within an anatomical distribution of individual motor nerves, with minimal or no sensory involvement.

Method

The aim of this retrospective study was to look for a significant reduction of the amplitude of sensory potentials in a cohort of 21 patients with defined multifocal motor neuropathy according to the Workshop Report criteria [Workshop Report, 2001. 79th ENMC International Workshop. Multifocal motor neuropathy 14-15 April 2000, Hilversum. The Netherlands. Muscle Nerve 11, 309-314], within a follow-up of at least 3 years.

Result

Thirteen patients (62%) (Group 1) had a reduction of the amplitude of at least one sensory potential, of whom four patients had abnormalities of two or more sensory potentials, while eight patients (Group 2) had no abnormality. No significant differences were found for gender, age at onset, number of involved motor nerves, CSF protein count, presence/absence of anti-GM1 serum antibodies and response to IgIV between the two groups.

Conclusion

This study underlines the difficulty in defining criteria for multifocal motor neuropathies capable of distinguishing them from other chronic acquired demyelinating polyneuropathies, and mainly from multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, also called Lewis-Sumner's syndrome.     

Aspergillose cérébrale chez le sujet immunocompétent. À propos de trois cas

Introduction

Cerebral aspergillosis is a severe disease most commonly suspected in immunocompromised patients.

Case report

We report herein three cases of cerebral aspergillosis in immunocompetent adults. Sinus involvement was noted in two cases, but there was no extracerebral involvement in the third case. Mycology samples provided the diagnosis in two cases. In the third case, cerebral imaging visualized a tumor; the patient underwent surgery and the pathology exam of the surgical specimen established the diagnosis. All patients were given antifungal treatment and achieved a good outcome.

Conclusion

Cerebral aspergillosis is highly uncommon in immunocompetent patients. In addition to immunodepression, the notion of pulmonary or ENT involvement may be suggestive. In the brain, aspergillosis mainly involves the basal nuclei and the thalamus.     

Un tremblement d’attitude révélant une maladie de Huntington

Introduction

Initial manifestations of Huntington's disease (HD) are varied and chorea is not always the first motor symptom.

Case report

We report the case of a 44-year-old woman, with a family history of HD, who presented isolated head and upper limbs tremor for 4 years. Genetic testing confirmed the diagnosis of HD and no cause of secondary postural tremor was found. Propanolol was introduced with success.

Conclusion

This kind of presentation is unusual and has mostly been reported in the juvenile form of HD.     

Pachyméningite associée à une probable maladie de Horton : étude d’une observation

Introduction

Horton's disease is the most common vasculitis of elder people. Several neurological complications are reported, but pachymeningitis is exceptional.

Observation

A 71-year-old patient who presented headache, hyperesthesia of the scalp, weight loss with a biological inflammatory syndrome and meningeal thickening on MRI. The diagnosis of pachymeningitis related to Horton's disease was retained. The patient was treated by corticosteroids with a good clinical, biological and radiological course after 22 months.

Conclusion

Horton's disease is a potential diagnosis in elderly persons with pachymeningitis and inflammatory syndrome.