Traitement des chondrosarcomes de la base du crâne

Background and purpose

Skull base chondrosarcomas are rare. Gross total removal is the treatment of choice, but can be difficult depending on the closeness of noble structures. Proton beam therapy can be associated in most cases.

Methods

Retrospective study of five cases treated in 13 years and study of the literature.

Results

Median age of patients was 34 years [28-46]. Cranial nerve palsy was the common clinical presentation. Tumor location was variable but always off midline. Treatment was surgical in all patients with a maximal resection and proton beam therapy associated for two cases. Surgical complications were rare with cranial nerve palsy as the main side effect. Outcomes were good with a median follow-up of 12.4 years [4.3-16.2].

Discussion

The review of the literature showed that chondrosarcomas of skull base are rare. The best outcome is achieved with total surgical resection. Medical imaging can only give clues to the diagnosis. Pathology is required to obtain a precise immunohistochemistry diagnosis. Multidisciplinary treatment using proton beam therapy and surgical removal enables a good local control (90-100%) at 5 years with good quality-of-life. It is difficult to determine how many cases have been published (around 220 cases in the literature) since many surgical or radiotherapy series included the same patients.     

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).     

Confirmation de l’intérêt de la biopsie cutanée dans la neuropathie des petites fibres. Résultats préliminaires

Introduction

In small-fiber neuropathy, skin biopsy reveals a reduction of intraepidermal nerve fiber density (IENFD), a feature often necessary for diagnosis. In France, this technique has not been widely used for this purpose.

Patient and method

To validate this method, we studied 13 patients with suspected small-fiber neuropathy, analyzed their nervous intra- and subepidermal network with a punch skin biopsy and compared our data with those of literature.

Results

Ten patients had pure small-fiber neuropathy and three an axonal polyneuropathy involving large-caliber nerve fibers. In the group of patients with pure small-fiber neuropathy, we found medium IENFD (11.6 ± 4.46 fibers per millimeter in the proximal thigh and 7.15 ± 3.59 fibers per millimeter in distal leg), well correlated with the electron microscopy quantitative and qualitative analysis of the unmyelinated subepidermal fibers.

Conclusion

This work demonstrated the good reproducibility of skin biopsy for analyzing the small-fibers in our cohort. These results require further confirmation in a larger cohort and validation in comparison with controls analyzed on a local level. Nevertheless, these techniques seem to be useful to assess the difficult diagnosis of small-fiber neuropathy.     

A painless complete abdominal wall palsy with groin and thigh paresthesiae related to traffic accident injury

Introduction

No case of complete unilateral abdominal wall palsy, occurring after a traffic accident, was found in the literature data. We report one case.

Case report

A 32-year-old woman was treated for a large abdominal hernia by MESH prosthesis nine years after a traffic accident injury. Surgery led to an iatrogenic lesion of the right lateral femoral cutaneous nerve (LFCN). During the electrodiagnostic examination for the LFCN lesion, abdominal wall palsy was diagnosed. Analysis of the patient's history and clinical, electrophysiological, and imaging data revealed that this abdominal wall palsy was related to a tear of the T10 to L2 anterior roots, plexus or nerves, a consequence of the violent deceleration during the car crash.

Conclusion

This report highlights the importance of clinical examination, electrodiagnosis and imaging in diagnosing abdominal wall palsy.     

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.     

Cognitive rehabilitation in multiple sclerosis: a review of the literature

Introduction

Cognitive impairment is now well-known in multiple sclerosis (MS). However, few rehabilitation interventions are proposed or really efficient.

Objectives

To present a review of cognitive rehabilitation intervention research conducted in people with multiple sclerosis (MS), regarding different findings about episodic memory, working memory, attention and executive function disorders in MS.

Data sources

A search of Medline (yield 20 papers) and of PsychInfo (yield 1 article), using combinations of the following terms: cognitive rehabilitation, multiple sclerosis, cognitive therapy, neuropsychological rehabilitation, in the title or in the abstract, from 1960 to March 2010, excluding animal studies.

Results

Episodic memory rehabilitation studies appear to be promising. Programs on working memory, attention and executive functions are in the very early phases.

Conclusions

Results are encouraging and allow specific recommendations for future research about: (1) inclusion criteria, often not defined, (2) a specific baseline adapted to the program of rehabilitation, (3) a control measure regarding program efficiency and (4) a role for the psychologist (presence and advice during the program).     

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.     

L’enfance des schizophrènes : revue de la littérature

Introduction

Pre-morbid antecedents in schizophrenia have been studied for some time now more particularly as potential markers of vulnerability. What are the tell-tale signs in some of the patient's childhood? The authors suggest a non-exhaustive review of the literature on this subject.

Method

The authors reviewed the literature (English and French) of prospective and retrospective studies.

Results

Many fields appear to be impaired during the childhood of some schizophrenic patients, fields such as: developmental abnormalities, speech impairments, social interactions, behaviour, cognitive functioning. The authors also noticed the presence of neurological soft signs and para-clinical abnormalities.

Discussion

The authors suggest a critical and synthetic review of existing data: what can be retained of this data? The authors also discuss the evolution of these signs and their interaction with the evolution of the disease itself.

Conclusion

Many of these signs were noticed in several children who later developed schizophrenia. For many authors, the more important these signs are, the more severe the disease will be. These pre-morbid antecedents give rise to theoretical questions and open perspectives concerning an early diagnosis of schizophrenia.     

Reconnaissance visuelle des émotions faciales au cours d’un trouble dépressif majeur de type mélancolie délirante

Background

Abnormal recognition of facial expressions (ARFE) is associated with poor inter-relational difficulties. Among depressive patients, the difficulties in recognising facial expressions lead to errors in emotionally charged information.

Objectives

To evaluate ARFE following the Analysis method and the search for emotional integration (MARIE). This tool uses a continuation of fictive portraits created from two real portraits and in varying proportions (Professor Eckman's portraits with his agreement). This is an emotional series, which can be either unipolar or bipolar (neutral/emotion, emotion/emotion). The subject has to choose between two possibilities for each portrait. The programme comprises nine series of 19 pictures of three different faces (a fair-haired woman, a dark-haired woman and a man).

Statistical analysis

An average between the two evaluations (X² test) can be compared according to the patient's answers. Comparison of results between patient's answers before and after the treatment and results before and after of a standard reference, which is, by definition, 100%. Comparing the averages of time answering between the two evaluations (Student test) by logarithms.

Results

The joy would be the emotion most sensitive to improve the mood and thus therapy. Conversely, recognition of fear and surprise would be the less sensitive. There would be a gradient answer of qualitative (type of emotion) and quantitative (intensity score) over treatment. The patient hardly recognizes the sadness at the opposite of the anger. There would be a hypo-sensibility to sadness or hypersensitivity to anger in a major depression. However, the disorder varies according to the face presented.

Discussion

We report the results of a unique case and we must remain critical. However, the results are in the line with most writers. It credits the interest of the measurement tool. The graduation in the intensity of the scores does not suggest a single system but seven emotional subsystems separated which intensity of the response is proportional to the mood disorder. This work should be extended to a homogeneous population of patients with more tests faces to avoid the idiosyncrasie and to better study the interactions between emotions and mood.     

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.     

Apports des dispositifs robotisés à la rééducation du membre supérieur hémiplégique

Introduction

Poststroke rehabilitation care has evolved considerably over the last decade. The emergence of the concepts of brain plasticity and motor learning has led to the development of new therapeutic approaches. Most of the new strategies are based on movement therapy, which can have a real impact on neurological recovery, sometimes with significant functional benefit for the patient.

State of the Art

In this context of evolving practices, the hemiplegic arm is the subject of special attention. Considering the often unfavorable “natural” prognosis and the relatively limited impact of conventional therapies; researchers have focused work on new alternatives. Cooperation between developers of technological advances and the medical community has led to the development of innovative therapeutic options often based on the use of specific technological tools (functional electric stimulation, virtual reality, transcranial magnetic stimulation…) to solicit or facilitate movement.

Perspectives/conclusion

Robot-assisted rehabilitation lies within this scope. The positive results reported in the most recent publications and the growing interest for this type of therapy in the fields of medical and engineering research should open the way for extremely promising prospects. The technological performance of new robots has nevertheless raised a large number of unanswered questions, implying a significant amount of further research.     

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.     

Maladie cœliaque et accidents vasculaires cérébraux ischémiques

Introduction

Neurological manifestations of celiac disease are various. An association with ischemic stroke is not common and has not been well documented. We report two cases.

Observations

The first patient had experienced several transient ischemic strokes in the past 2 years and then had an acute ischemic stroke involving the territory of the right posterior cerebral artery. Investigations revealed celiac disease with no other recognizable etiology. The clinical course was marked by persistent visual aftereffects, but no new vascular event. The second patient had been followed since 1998 for celiac disease confirmed by pathology and serology tests. She was on a gluten-free diet. The patient had an ischemic stroke involving the territory of the left middle cerebral artery. Apart from a positive serology for celiac disease and iron deficiency anemia, the etiological work-up was negative.

Discussion

The mechanisms of vascular involvement in celiac disease are controversial. The most widely incriminated factor is autoimmune central nervous system vasculitis, in which tissue transglutaminase, the main auto-antigen contributing to maintaining the integrity of endothelium tissue, plays a major role. Other mechanisms are still debated, mainly vitamin deficiency.

Conclusion

Being a potentially treatable cause of ischemic stroke, celiac disease must be considered as a potential etiology of stroke of unknown cause, particularly in young patients, and even without gastrointestinal manifestations.     

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.     

Coma profond aréactif réversible après intoxication par des abats d’un poisson méditerranéen

Introduction

Neurotoxic fish poisoning appears to be a recent phenomenon in the Mediterranean Sea. We report a case of deep non-reactive reversible coma after ingestion of Mediterranean fish innards.

Case report

An 80 year-old man, heavy smoker who had a previous cerebral infarct in the posterior territory, was admitted for rapid deterioration of his neurological condition. He started having perioral tingling, then dysarthria, then became quadriparetic, then developed respiratory and hemodynamic failure and within 3-4 h, entered a state of deep non-reactive coma with absence of all brainstem reflexes. He started to improve after 20 h and recovered his neurological baseline within 36 h. Later on, he stated that all his symptoms started after he ingested the gonads of a toxic fish, Lagocephalus scleratus.

Discussion

Tetrodotoxin blocks voltage-gated sodium channels and inhibits the production and propagation of action potentials. This toxin is highly concentrated in the liver, gonads, intestines and skin of this fish that is well-known in Japan (where it is considered as a delicacy) and South-East Asia and seems to have migrated recently to the Mediterranean Sea. There is no known antidote to tetrodotoxin but intensive supportive treatment can be life-saving.     

Chorée paranéoplasique et troubles du comportement chez un patient présentant des anticorps anti-CV2/CRMP5 et deux cancers différents

Introducion

Paraneoplastic movement disorders are rare. Reported cases frequently describe association with anti-CV2/CRMP5 antibodies.

Case report

We report a case of an 80-year-old man who developed sensorial neuronopathy, following by movement disorders mimicking chorea and obsessive-compulsive and behavioral disorders. These manifestations were first considered to be associated with a prostatic adenocarcinoma but PET and surgical biopsy revealed a mediastinal small cell lung carcinoma classically associated with anti-CV2/CRMP5 antibodies.

Conclusion

This case demonstrates that in a context of paraneoplastic neurological syndrome, search for a classically associated cancer is necessary in order to institute adapted treatment early, even if another tumor is obvious.     

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.     

Dissémination sous-arachnoïdienne de gouttelettes lipidiques par rupture d’un kyste dermoïde intracérébral

Introduction

Dermoid cysts are rare slow-growing benign tumors of the central nervous system generally diagnosed in the third to fifth decade. They are formed from inclusion of ectodermal elements during neural tube closure, and are mostly located along the cranial or spinal midline axis. They cause many non specific symptoms such as headache and seizures, and may spontaneously rupture spreading fatty droplets into the ventricles and subarachnoid spaces. Rupture of dermoid cysts causes sequelae which may vary from no symptoms to death. In general, subtotal surgical removal is required for ruptured dermoid cysts.

Case reports

We report two cases of ruptured intracranial dermoid tumor with non-specific clinical presentations. The first rupture was asymptomatic and discovered on brain magnetic resonance imaging (MRI) performed for other purposes. The second case was identified on brain imaging performed because of daily headache. These dermoid cysts were not surgically treated. Surveillance was advised because of the spontaneously favourable outcome observed in both cases.

Conclusion

Surgical removal is not the only treatment of ruptured dermoid cyst. Monitoring with brain MRI can be sufficient if the rupture has no severe clinical impact.     

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.