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.     

Épilepsies généralisées idiopathiques chez le sujet âgé : le point de vue du gériatre

Introduction

Long-term follow-up studies indicate a low remission rate in idiopathic generalised epilepsies (IGE) (Martinez-Juarez et al., 2006), suggesting they may persist to an advanced age. However there are few estimates of IGE frequency in the elderly.

Methods

EEGs of 700 patients aged over 70 years, recorded between January 2006 and March 2007, were reviewed for anomalies consistent with IGE. We then examined the clinical history of patients with these anomalies.

Results

A persistent IGE was identified in four female patients (mean age: 79 years); in two cases it was a juvenile myoclonic epilepsy (JME) and in two an epilepsy with grand mal seizures. Seizures in three patients had begun in childhood or adolescence and in one at 40 years. Before hospitalization, few or no seizures were reported and IGE had not been diagnosed. IGE was revealed in each patient by a relatively severe event: an absence status (AS), subcontinuous myoclonic seizures or repeated convulsive generalised seizures (CGS). These events were not situation-related but in one patient the relapse of simple convulsive seizures, may have been related to the withdrawal of anti-epileptic drugs (AED) several months previously. EEG records showed generalised spikes or polyspikes and waves organised in a status epilepticus or in interictal rhythmic discharges. In one case they were evident only from a 24 hours recording. Clonazepam injection was used to suppress the AS episode and the subintrant myoclonia. After the AS, interictal generalised epileptic discharges persisted. Two of the four patients had familial history of epilepsy or febrile seizures but in no case was an epileptogenic lesion evident in brain CT scan or MRI. Clinical exams and biologic parameters were normal. All of the patients had worked and were married with children. Appropriate therapies were followed after the diagnosis of IGE. One patient with JME had been treated by Valproate which was discontinued by the general practitioner because of lethargy and replaced by Carbamazepine; seizures were aggravated under both Carbamazepine and then Lamotrigine and until the patient became seizure-free on Levetiracetam. The antiepiletic treatment was also modified in a second patient, while the two others responded well to Valproate.

Conclusions

IGE can exacerbate in the elderly, as different types of seizures including AS, subintrant myoclonia or repeated CGS. Our data suggest persistent IGE are quite frequent in an aged population and may be underestimated due to difficulties in diagnosis. Correctly diagnosed, adjustment of AED may offer substantial clinical improvements in IGE of the elderly.     

Atteinte multiple des nerfs crâniens : atteinte multiple des nerfs crâniens au cours du tétanos céphalique. À propos d’une observation et revue de la littérature

Introduction

Cephalic tetanus is the most serious form of localized tetanus. It associates trismus with impairment of one or more cranial nerves. It was a rare condition, whose diagnosis can raise several problems.

Case report

A 49-year-old-man presented multiple and unilateral cranial nerve involvement revealing cephalic tetanus.

Conclusion

This case illustrates the importance of considering cephalic tetanus when patients present cranial nerve palsy associated with injury.     

Mélanome malin leptoméningé primitif : une étiologie rare de pachy- et leptoméningite

Introduction

Leptomeningitis and pachymeningitis are known to occur consecutive to many causes.

Observation

We report the case of a 24-year-old woman with symptoms of raised intracranial pressure and repeated switching transient hemiparesis. The magnetic resonance imaging (MRI) showed a pachyleptomeningitis. Search for a cause was negative. The pathology examination of meningeal tissue revealed a malignant melanoma, without any sign of cutaneous melanoma, leading to the diagnosis of primary leptomeningeal malignant melanoma.

Conclusion

A meningeal biopsy can enable the rare diagnosis of primary leptomeningeal malignant melanoma in a patient with unexplained pachyleptomeningitis.     

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.     

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.     

Programme spécialisé pour les premiers épisodes psychotiques : analyse d’une cohorte de 100 patients

Background

The emergence of specialized programs for the treatment of first-episode psychoses in non-research settings calls for a better definition of this group of patients and of the psychological interventions offered.

Aims

The aim of this study is to describe a specialized program for first-episode psychotic patients and to define the patients referred to, their different distinguishing characteristics and their relative use of the different services offered them.

Method

From an initial population of 127 patients, 100 agreed to have their data used to determine their detailed socio-demographic and symptomatological characteristics, their treatment delays (duration of untreated psychosis, referral delay) and their use of specific treatment modalities offered.

Results

The sample is similar to others described in the current literature in terms of socio-demographics, diagnostic distribution, and duration of untreated psychosis. The referral delay is 1.66 year. The symptomatological and neuropsychological portraits observed are characterized by heterogeneity. Services offered appear clinically indicated for most patients referred to (81%), with different characteristics observed across the groups of patients referred to in the different modalities.

Conclusion

The heterogeneity of the clinical presentation and of needs observed implies that such a program has to include a detailed assessment of each patient and a basic range of interventions. The implementation of such interventions in a non-research setting, and eventually on a large scale, should be accompanied by an evaluation process that could help guide clinical work and the organization of psychiatric services for patients suffering from psychosis.     

Hémorragie méningée focale et angiopathie amyloïde cérébrale : une association non fortuite

Introduction

Cerebral amyloid angiopathy is a degenerative angiopathy due to amyloid deposits in the walls of the meningeal and cortical vessels. It is considered as a major cause of cerebral hemorrhage to the elderly. It was recently demonstrated that the association of focal meningeal bleedings and cerebral hemorrhage is very suggestive of cerebral amyloid angiopathy. In contrast, the links between subarachnoid hemorrhage and amyloid angiopathy are less well-known.

Cases reports

We report nine cases of cerebral amyloid angiopathy. The clinical presentation was variable, but all had at least one inaugural meningeal bleeding. As cortico-meningeal biopsies were not performed the Boston criteria were used to establish the diagnosis.

Conclusion

Cerebral amyloid angiopathy is an underestimated cause of subarachnoid hemorrhage. Our observations show that this diagnosis should be evoked when focal meningeal bleeding occurs without head trauma or when focal subarachnoid hemorrhage is followed by a subcortical hematoma in an elderly subject.     

Apport diagnostic des marqueurs biologiques de la maladie d’Alzheimer (MA) dans une observation de dégénérescence lobaire fronto-temporale (DLFT)

Introduction

Cerebrospinal fluid biomarkers are a fundamental contribution for the diagnosis of Alzheimer disease, in particular for young people.

Case report

We report a case of Alzheimer disease of a 51-years old man whose clinical presentation corresponded to frontotemporal behavioural criteria. The clinical evaluation noticed some troubles in verbal memory. Only the CSF biomarkers confirmed the diagnosis.

Conclusion

There are different phenotypes of presenile Alzheimer disease and among them we note frontotemporal dementia. It is very important to identify them to introduce a specific treatment.     

Déficit multiple en acyl-CoA déshydrogénases : une cause traitable de lipidose musculaire d’origine génétique

Introduction

Multiple acyl-CoA dehydrogenase deficiency (MADD) is a rare genetic disease involving fatty acid oxidation. It is due to the deficiency of one of the two electron transporters: electron transfer flavoprotein (ETF) or electron transfer flavoprotein ubiquinone oxydoreductase (ETF-QO). Symptoms begin more often in childhood or in young adulthood with a multisystemic disease with encephalopathy or muscular weakness.

Case reports

We report here two adult cases with ETF-QO deficiency, confirmed by mutation analysis (ETFDH gene), revealed by a muscular weakness associated with muscle lipidosis. One of our patients presented an acute encephalopathy with vomiting ten years before the onset of muscular symptoms. The second patient exhibited a slowly progressive pelvic girdle muscle weakness. Diagnosis was established by characteristic abnormalities of acylcarnitine profile by tandem mass spectrometry. For both patients, a dramatic clinical improvement was observed under treatment with riboflavine and L-carnitine.

Conclusion

Since it is a treatable disorder, this diagnosis must be considered by performing an acylcarnitine profile in all patients presenting with an unexplained muscular weakness.     

Étude des liens entre sommeil et mémoire épisodique chez le sujet jeune et âgé, et dans la maladie d’Alzheimer

Introduction

A large body of evidence indicates that sleep favors memory consolidation.

State of the art

This process would occur, mainly during slow-wave sleep, by means of a dialogue between the hippocampus and neocortical areas. Low levels of acetylcholine and cortisol are also needed to favor the transfer of memory traces toward the neocortex, where they will be stored for the long-term.

Perspectives

The aim of this article is, first, to give an overview of studies conducted in young healthy subjects and underpinning the hypothesis that sleep is involved in memory consolidation. Then, we will investigate the potential links between changes in sleep architecture and episodic memory impairment in both aging and Alzheimer's disease. Finally, we will see how these results can affect clinical practice.

Conclusion

Sleep-dependent memory consolidation is impaired both in aging and Alzheimer's disease. These findings suggest the importance of taking into account sleep when assessing memory function in patients.     

Validation du CHIP (questionnaire de coping) dans une population française atteinte de maladies neurologiques

Introduction

While conceived in the 1960's by Lazarus, the concept of coping was a long time coming in the field of neurological diseases. So far, no valid instrument has been available in French for assessing the style of coping of patients with neurological diseases.

Methods

We used Coping with Health Injuries and Problems (CHIP), which was conceived for patients with chronic diseases. We proceeded to a factorial analysis in a population of 307 patients with multiple sclerosis (MS) or Parkinson's disease (PD) in order to identify the factors observed in this population.

Results

We finally retained a model with six factors, which saturated on 24 items and explained 48% of the total variance. Factors we found included palliative coping and distraction, both known for the original instrument, and four new factors: emotional regulation, seeking of well-being/self-preservation, seeking of information and cognitive avoidance.

Discussion

Tools for assessing coping in a setting of chronic diseases must be adapted to specific diseases. In this new validation, CHIP appeared to be well adapted to the specific features of neurological disorders. This instrument would be useful for the neurologist to better understand the specific ways by which patients cope with their disease. Such knowledge would certainly have a positive effect on the patient-neurologist relationship. Moreover, a better understanding of patients’ coping strategies in neurological diseases could pave the way for specific preventive interventions focusing on using coping strategies to promote better adaptation.     

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.     

Première évaluation d’un programme de remédiation cognitive chez des patients atteints de sclérose en plaques : PROCOG-SEP

Background

Although cognitive disorders are well-known in multiple sclerosis (MS), even in earlier stages of the disease, their management may be overlooked. Our objective was to elaborate and evaluate the efficiency of a remedial program (PROCOG-SEP) designed for MS patients. The evidence-based program proposes exercises to both stimulate preserved functions and develop new abilities compensating for cognitive disabilities.

Design/Methods

Twenty-four patients with MS participated in 10/2-hour PROCOG-SEP sessions over a 6-month period. A neuropsychologist recorded BCcog-SEP performances before and after the PROCOG-SEP program. In addition, the same neuropsychologist conducted psychoclinical interviews to complete the before and after cognitive evaluations. The statistical analysis used the t-test performed with Excel.

Results

Compared with the initial levels, subtests of BCcog-SEP showing improvement after PROCOG-SEP were: verbal memory (SRT), visuospatial memory (10/36), verbal fluency (animal categories) and response to conflicting orders. Also, individual psychological interviews tended to be in favor of a general improvement in quality of life (more social interactions for instance).

Conclusions/Relevance

To our knowledge, the management program we have elaborated is the first designed to improve cognitive deficits in MS. These encouraging results suggest possibilities for improving cognition and thus quality-of-life in MS patients.     

Trouble psychotique révélant une épilepsie associée à une tumeur dysembryoplasique neuroépithétliale de l’hippocampe gauche

Introduction

Psychotic symptoms are a rare but well-known complication of epilepsy. The prevalence is estimated between 4 and 9%.

Patient

We report a case of a 40-year-old patient, unrecognized epileptic, who presented an acute psychotic syndrome which seemed to be of functional origin, the EEG performed during the episode, and the cerebral CT scan being normal. Nevertheless, the clinical presentation, especially the sudden ending of delusions, led to further investigations. Careful history taking and repeated EEG recordings allowed the diagnosis of partial epilepsy that had begun 17 years earlier and symptomatic of a dysembryoplastic tumour of the left hippocampus revealed by MRI.

Discussion and conclusion

Search for an epileptic origin of an acute psychotic syndrome must always be undertaken by systematic EEG. The possibility of a symptomatic temporal tumor must not be overlooked.     

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.     

Thrombose veineuse cérébrale et polyradiculonévrite aiguë révélant un lupus érythémateux disséminé

Introduction

Neurological manifestations of systemic lupus erythematosus are frequent and polymorphic. In 40% of cases, lupus can be revealed by neurological symptoms. Cerebral nervous system complications predominate and can be a negative factor for prognosis. Peripheral features are rare and various and can compromise functional prognosis, sometimes with fatal outcome.

Case report

We report the case of a 30-year-old woman who presented a cerebral venous thrombosis of the superior longitudinal sinus. Outcome was favorable with antibiotics and anticoagulants. Four months later, she developed an acute polyradiculoneuritis associated with an inflammatory syndrome and positive tests for antinuclear antibody and antinuclear anti-DNA. The diagnosis of neurolupus was retained on the basis of four criteria of the American college of Rheumatology. The patient was given steroid therapy associated with a course of intravenous immunoglobulin. She has fully recovered her deficit.

Conclusion

Cerebral venous thrombosis and acute polyradiculonévrites are rare events in systemic lupus erythematosus. Early diagnosis and management are crucial.     

Polynévrite, crise convulsive, syndrome cérébelleux, complication d’un traitement par métronidazole. Apport de l’IRM

Introduction

Neurological complications of metronidazole are rare, predominantly peripheral neuropathies, especially in patients on a long-term high-dose regimen. Cerebellar syndrome or seizures are less frequently reported. The concomitant occurrence of the three complications is exceptional.

Case report

We report herein a case with these three complications as side effects of metronidazole. For the cerebellar syndrome, the T2-weighted brain MRI showed a rounded and well-delimited zone of high signal intensity in the cerebellar dentate nuclei, extending up to the protuberance and the subthalamic nucleus, bilaterally and symmetrically.

Conclusion

Neurological complications are possible when a treatment with metronidazole is prescribed for a long duration or at high dose. In our patient, the clinical abnormalities and MRI signs regressed a few months after treatment withdrawal.     

Stimulation magnétique transcrânienne répétée (rTMS) et symptômes négatifs de la schizophrénie

Purpose

The author has endeavoured to present a qualitative review of current data on the interest of repetitive Transcranial Magnetic Stimulation (rTMS) in the treatment of negative symptoms of schizophrenia, its therapeutic impact and processes that underlie it.

Method

The method consisted in a review of the literature by an extensive consultation of the computerized Medline database.

Results

Despite the small number of controlled studies and the small sample sizes, rTMS appears to be an effective therapeutic method in the treatment of negative symptoms of schizophrenia and also helps understanding the patho-physiologic processes that underlie them (hypofrontality, dopamine hypothesis and responsiveness of the HPA axis).

Conclusion

Before any conclusion may be reached about the effectiveness of this new technology more studies are required using larger parameters for active treatment and optimal placebos (alpha-TMS at least 110 % of the motor threshold, focused on the hypo-active area).