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.     

Caractéristiques des enfants ayant une épilepsie suivis au CHU de Marrakech

Introduction

Epilepsy is one of the most frequent neurological diseases in the pediatric population. Many epidemiological studies have been published, but with rather discordant results, because of methodological differences. In our context, epilepsy constitutes a public health problem. National epidemiological data are scarce.

Objectives

To describe the characteristics of children with epilepsy, to analyze the risk factors and to assess the impact of the disease on schooling.

Methods

This was a retrospective study concerning 592 children attending the Mohammed VI university hospital center pediatric unit A outpatient clinic for epilepsy from August 2003 to December 2007.

Results

Epileptic syndromes were classified according to the criteria of the International League Against Epilepsy of 1989. Prevalence of epilepsy was 8.5%. Average age was 6 years 7 months. Age of seizure onset ranged from 2 months to 14 years. Male gender predominated. Antecedents were dominated by peri- and neonatal complications. Parental consanguinity and a family history of epilepsy were found in 19.2 and 11.6% of cases, respectively. Schooling was perturbed in more than one-third of the school-age children. Generalized seizures were most common (70.5%). Association with cerebral palsy was present in 18.6% of cases, with mental retardation in 4.7%. The epilepsy was idiopathic for 41% of the children, symptomatic for 39% and cryptogenic for 20%. Generalized epileptic syndromes were the most frequent, epilepsy absence (12%), Lennox-Gastaut syndrome (6%), West syndrome (5.5%) and myoclonic epilepsy (4%). The most common partial epileptic idiopathic syndrome was benign childhood epilepsy with centrotemporal spikes. Single-drug therapy was the rule for first intention treatment (96.8%). Sodium valproate was the antiepileptic drug most widely used (82%). Treatment led to resolution of the seizures in 76% of the children.

Conclusion

Preventive measures should be reinforced in our context with a considerable proportion of children presenting neonatal risk factors. Efforts should be made to improve schooling for children with epilepsy.     

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.     

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.     

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.     

Infarctus cérébral, infection VIH et syphilis méningovasculaire : étude de trois cas

Introduction

Various clinical manifestations can occur in syphilis, especially meningovascular syphilis. We report three cases of ischemic stroke related to meningovascular syphilis and HIV infection.

Case reports

Three men, aged 30, 31 and 41, presented typical neurologic deficit leading to the diagnosis of ischemic stroke. Radiographic investigations showed internal carotid thrombosis in two patients, and a basilar stenosis in the third. CSF analysis and blood samples revealed meningovascular syphilis associated with HIV infection. The other explorations were normal.

Conclusion

Due to the reemergence of syphilis, search for Treponema pallidum infection should be systematic in young stroke victims.     

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.     

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.     

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.     

Traitement de l’hypotension spontanée du liquide cérébrospinal par perfusion épidurale de sérum salé isotonique

Introduction

Spontaneous intracranial hypotension (SIH) is an uncommon cause of secondary headache due to a cerebrospinal fluid (CSF) hypotension. Lumbar epidural blood-patch (LEBP) is the most effective treatment and can be repeated in case of relapse. There is no standard therapeutic strategy for patients free of dural tears who fail to respond to several consecutive blood-patches. We report two cases of SIH successfully treated by an epidural saline infusion after two consecutive LEBP.

Case reports

A 35-year-old woman was admitted to hospital for severe orthostatic headache. The diagnosis of SIH was retained. Two LEBP were performed but with no clinical benefit. Headache disappeared totally after an epidural saline infusion. A second woman, aged 75 years, was admitted for chronic orthostatic headaches. The CSF pressure was low. Search for a dural tear was negative. After two unsuccessful LEBPs, the patient was treated with an epidural saline infusion. Her headache resolved completely and definitely.

Discussion

It is common procedure to search for a dural tear when patients fail to respond to several consecutive LEPB. Surgical repair is however exceptional. An epidural saline infusion might be an efficient therapeutic alternative despite the small number of cases reported in the literature.     

Premiers éléments pour une étude psychométrique de la fixation-régression avec la McGill Object Relations Scale

Introduction

Psychosexual development is generally assessed clinically, that is to say, in a qualitative manner (Piper and Duncan, 1999). Dymetryszyn, Bouchard, Bienvenu, De Carufel, and Gaston (1997) recently proposed a more quantitative approach based on the overall maturity of the subject's object relations.

Background

Object-relation maturity is quantified using a profile that defines a score for each stage of an individual's psychosexual development : oral-narcissistic, oral-objectal, anal, phallic pre-oedipal, oedipal, and genital. The McGill Object Relations Scale (MORS) (Dymetryszyn et al., 1997) was adapted (Combalbert, Vautier, Bourdet-Loubère, Favard, & Bouchard, 2002) and then used to obtain the quantitative scores. The relationship between overall object-relation maturity and psychosexual development is complex. Psychosexual development is considered here to be a constant latent dimension. Accordingly, the higher the level of development, the greater the individual's possibility of establishing the object relations that correspond to more evolved stages. Inversely, the lower the level of development, the more the individual is forced to rely on object relations corresponding to earlier stages of development. However, the connection between the object-relation maturity profile and psychosexual development is made even more complex by the existence of the well-known phenomenon of fixation-regression.

Objectives

This article attempts to determine how fixation-regression is reflected in the structure of the profiles of a sample of individuals whose clinical diagnosis suggests probable regression to the anal stage.

Methods

The subjects chosen had borderline personality disorder coupled with either perversion or psychopathy. The data were modelled using an unfolding model. MORS was used on 60 criminal subjects who had been charged with, or convicted of, crimes against persons. The nosographic diagnosis was based on the psychiatric assessment of the prisoners. DSM IV diagnostic criteria were used as a reference to confirm or refute the pronounced diagnosis. Only subjects who met at least five criteria for borderline personality disorder were included in the sample. To assess the reliability of the individual scores, two expert judges blindly scored the 60 protocols.

Results

The results obtained with an unfolding model support the hypothesis that the observed ordinates for the oral-narcissistic, oral-objectal, phallic pre-oedipal, oedipal, and genital stages obey a law of synchrony that complies with the postulate of a continuum in psychosexual development. The second main result was the fact that the observed ordinates for the level corresponding to the anal stage could not be described by the model. Furthermore, this finding did not come from a possible scoring error, since inter-judge reliability was 0.93 for that level.

Discussion

These results are encouraging for the utilization of MORS as a technique for quantifying object relations. This study on a sample of individuals with a high probability of anal stage fixation-regression suggests that this type of phenomenon can be objectified by means of MORS profiles. However, the present study has some limitations of its own. The main methodological drawback pertains to the subjective aspect of the protocol scoring. The two judges had a great deal of joint experience in scoring other protocols on the MORS grid. This could explain the particularly high inter-judge correlations, compared to the within-class correlations published by Dymetryszyn et al. (1997).

Conclusion

One cannot rule out the possibility that our analyses would have had different outcomes had other independent judges scored the 60 protocols. Moreover, our results cannot be generalized without replication. A critical test would be to replicate our analyses on similar data, and to examine the unfolding model's behavior on data from a sample of ordinary adults diagnosed as not having strong fixation-regression tendencies. If the synchrony laws defined by the unfolding model are correct, the anal stage should be included in the arc formed by projecting the six stages on the factorial plane of the principal component analysis. Fundamental research based on the psychosexual theory of the libido and supported by statistical data can objectify the vicissitudes of psychosexual development (such as fixation-regression) or developmental differences across groups of individuals. This makes a strong case for pursuing MORS-based investigations.     

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.     

Analyse clinique au cours d’une marche simple et avec une double tâche chez une population de sujets âgés ambulatoires consultant en gériatrie

Introduction

Gait and balance often change with age. Few studies are available on gait analysis in the elderly. In our work, we have focused on slow walking and variation in pace. Since first introduced by Lundlin, the dual-task paradigm has been largely used to test for the risk of falls and to better understand the link between mild cognitive decline and variation in gait. To our knowledge, very few clinical data are available on the gait changes observed in the elderly in the dual-task situation. In this study, we compared changes in gait and balance between the simple task and the dual-task situation in a 10-m walk test conducted in community-dwelling old people.

Methods

Eighty-nine subjects attending a geriatric outpatient clinic (age 80.76 ± 2.82 years) were included in our protocol. Gait, balance and posture were systematically evaluated during a simple and dual-task 10-m walk using a specific 11-item clinical scale. Gait speed was noted.

Results

Few differences were found in the dual-task walk, only reduced speed, more stops during the walk and less compliance with instructions. These results were correlated with the Mini Mental Status (MMS) score. Systematic clinical analysis failed to identify any links with age, the MMS, the UPDRS, the Tinetti scale or gait speed except for the variables freezing, increased sustention polygon and ataxia.

Discussion

This kind of study appears to offer an attractive analytical approach but would require a more pertinent choice of variables for cognitive evaluation and a more exhaustive set of MRI criteria. Nonetheless, clinical gait analysis could improve our understanding of the consequences of mild cognitive impairment and aging.     

Juvenile myoclonic epilepsy in a patient with history of infantile hemiplegia

Introduction

The 1989 International Classification of Epilepsies and Related Syndromes considers normal cognitive, neurologic and anatomic findings to be prerequisites for the diagnosis of idiopathic forms of epilepsy.

Case report

We report the case of a woman with juvenile myoclonic epilepsy (JME) and a history of infantile hemiplegia. When she was a teenager, she had two generalized tonic-clonic seizures, later followed by a few seizures with loss of consciousness misinterpreted as complex partial seizures. Physical examination revealed right hemiparesis. A CT scan documented a left rolandic infarction and a wrong diagnosis of focal epilepsy was made. At 20 years, a nap video-EEG was performed. A burst of generalized spike-waves was recorded on awakening. Photic stimulation and watching a Japanese cartoon on television disclosed a marked photoparoxysmal response associated with myoclonic jerks. Myoclonic jerks were in fact known by the patients but unreported. She had jerks on roads with trees due to shade/sunlight alternance. A diagnosis of JME was made.

Conclusion

This observation illustrates that patients’ situation with a presumed genetic predisposition for JME are at equal risk for brain lesions as others subjects. Misdiagnosis of focal epilepsy may have dramatic consequences in patient with JME, as some patients will be aggravated by inadequate antiepileptic drugs.     

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.     

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.     

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.     

La thrombose veineuse cérébrale : étude étiologique prospective de 26 patients tunisiens

Purpose

The aim of the present study is to provide a clinical and etiological analysis of cerebral venous thrombosis (CVT) in the Tunisian population.

Methods

This is a prospective monocentric study including 26 patients referred to the Neurology Department of the Military Hospital of Tunis between January 2005 and January 2008. The diagnosis of CVT was confirmed in all patients by magnetic resonance imaging (MRI) and angiography. The clinical and biological risk factors of cerebral venous thrombosis were analyzed. The average follow-up was 18 months (range six to 30). The outcome was assessed clinically with the modified Rankin scale and with MRI.

Results

Mean age was 38.26 years, predominantly females (sex-ratio 4.2). The clinical onset was acute in 88.46% of the cases. Headache was the most common inaugural sign (84.6%). Lateral and superior longitudinal sinuses were the most commonly involved with equal frequency (61.53%). Parenchymal lesions were frequently noted (77%), especially hemorrhagic infarcts (46.15%). The causes of CVT were variable and usually combined (85%). Specifically, thrombophilia and obstetric-gynecological causes were predominant with a prevalence of 61.5 and 42.3%, respectively. Septic causes (38.46%) are also frequent, mainly oral infections (27%). Outcome was favorable in 77% of patients given heparin therapy, followed by oral anticoagulants and antibiotics as needed.

Conclusion

Our Tunisian population presented distinct clinical features compared with previous studies, including a high frequency of thrombophilia and gyneco-obstetrical disorders as well as infectious causes.     

Méthode et résultats du test de perfusion dans l’hydrocéphalie à pression normale : revue de la littérature

Introduction

The cerebrospinal (CSF) infusion test is used to analyze the dynamics of CSF circulation. We describe the technique and report test results obtained in subjects with normal pressure hydrocephalus.

State of the art

The CSF infusion test is based on pressure monitoring during a continuous infusion of saline solution into the CSF space via a lumbar puncture. The main parameters are: intracranial pressure, resistance to CSF outflow (Rout), and compliance of the cranial enclosure estimated by the pressure-volume index. Our review of the recent literature dealing with the results of infusion test in normal pressure hydrocephalus indicates that the positive predictive value is about 80 % for Rout greater than 12 mmHg/ml per minute, i.e. shunt response is observed in 80 % of patients. The information available in the literature for assessing negative predictive value of Rout and usefulness of compliance is limited.

Perspectives and conclusion

The CSF infusion test may be helpful for the diagnosis of normal pressure hydrocephalus, as supplemental testing when the diagnosis is not certain. We propose a procedure, which includes the infusion test, for identifying shunt-responsive patients.     

Évaluation de la représentation de la mère chez la femme alcoolique en soin : une étude exploratoire

Exploratory hypothesis

The representation of the mother among treated alcoholic women is specific and different from the mother's representation among non-alcoholic women.

Methodology

The present study involved 10 alcoholic women and 10 non-alcoholic women. The participants’ maternal representation, the level of dependency to alcohol and psychiatric comorbid disorders were assessed in both groups using the Thematic Apperception Test (TAT), the Alcohol Use Disorders Identification Test (AUDIT) and the Mini International Neuropsychiatric Interview (MINI).

Results

The mother's representation was found invading and intrusive among alcoholic women. A significant correlation between alcohol dependency and depression was evidenced.

Conclusion

The mother seems omnipresent in the alcoholic woman's psychological life. Alcoholic women seem to experience unsatisfying relationships with their mother and to perceive them as omnipotent. Dependency to alcohol could be an answer to this specific type of relationship. Before being generalised and included in the existing treatment programs, these findings should be confirmed by further studies using a wider sample size.