Prism adaptation effects in complex regional pain syndrome: A therapo-physiological single case experimental design exploratory report.

Complex Regional Pain Syndrome (CRPS) is an invalidating chronic condition that can occur after an acute peripheral lesion. Prism adaptation therapy is regarded as a promising tool to improve chronic pain in this syndrome but the mechanisms which lead to pain amelioration remain unknown. In this exploratory report we performed a retrospective analysis of longitudinal data collected from a single, atypical patient, who showed hyper-attention toward her affected (left) hand. Repeated assessments of pain and spatial neglect made during the course of the prism adaptation treatment revealed differential contributions of the two hands to adaptation-induced pain reduction. Treatment response appeared to be associated with a relative modification of the spatial behaviour of the two hands. This case study provides a new example of pain relief following prismatic deviation away from the pathological side.

Ehlers-Danlos arthrochalasia type (VIIA-B)--expanding the phenotype: from prenatal life through adulthood.

The Ehlers-Danlos syndromes (EDS) form a clinically and genetically heterogeneous group of inherited connective-tissue disorders characterized by joint hypermobility, tissue fragility and skin abnormalities. Six subtypes have been well characterized based on clinical features and molecular genetic abnormalities. The arthrochalasia type EDS (formerly types VIIA and B) is characterized by severe generalized joint hypermobility with multiple dislocations including congenital bilateral dislocation of the hips, muscular hypotonia and distinct dysmorphic features. The diagnosis of the arthrochalasia type EDS is of importance in the neonatal period because of consequences of physical disability in later life. However, the differential diagnosis may be difficult because of overlap with other hypermobility syndromes. In addition, the significant hypotonia may direct the physician toward various neuromuscular diagnoses. As patients become older, the hypotonia decreases and facial features become less distinct. In this report, we describe seven patients at different ages. Timing of diagnosis varied from prenatal life to adult age. The diagnosis of EDS type VII was confirmed by biochemical studies or mutation analysis showing characteristic mutations in COL1A1 and COL1A2. These mutations result in skipping of exon 6, which leads to defective collagen synthesis. For physicians treating patients with EDS type VII, achieving mobility for the patient is the greatest challenge and it may be impossible because of recurrent dislocations of nearly all joints in severe cases.

[Toxic leucoencephalopathy after use of sniffed heroin, an unrecognized form of beneficial evolution]. / Leucoencéphalopathie toxique après consommation d'héroïne prisée "sniff", une forme méconnue d'évolution favorable.

INTRODUCTION: Serious leukoencephalopathy can be related to heroin injection or inhalation. OBSERVATION: We report the first case of leukoencephalopathy observed three weeks after a 46-year-old man sniffed heroin. The clinical presentation included cognitive and behaviour disorders, pyramidal irritation and slight gait instability. Blood and cerebrospinal fluid analyse were normal. Brain magnetic resonance imaging showed diffuse, symmetrical supratentorial white matter lesions producing high intense signals on FLAIR and b1000-weighted sequences. Proton spectroscopy revealed an increased rate of cholin, in favour of active demyelinated lesions. Brain biopsy showed intramyelinic oedema with reactive gliosis. After two and a half years, moderate attentional fluctuations and difficulties in initiating activities persisted. Repeated MRI showed a reduction of the leukoencephalopathy. CONCLUSION: Heroin could be a cause more common than thought of leukoencephalopathy. The clinical and radiological expression and prognosis could be related to the mode of consummation (inhalation, intravenous injection, sniffing). This parameter may modulate severity and localization of brain lesions. More systematic use of MRI for patients with psychiatric symptoms after heroin intoxications could lead to a better evaluation of heroin-related neurotoxicity and potentially improve prevention.

[Transanal irrigation for bowel and anorectal management in spinal cord-injured patients]. / Intérêt des irrigations transanales dans la gestion des troubles intestinaux et ano-rectaux chez les blessés médullaires.

OBJECTIVE: Bowel dysfunction and disordered defecation are very common after spinal cord injury (SCI) and can have a major impact on patients' social life and quality of life. The aim of this study was to assess the safety and efficacy of transanal irrigations (TAI) in the management of Bowel dysfunction in SCI patients. METHODS: Forty-five consecutive SCI patients using TAI were retrospectively included. TAI efficacy was assessed through Neurogenic Bowel Dysfunction (NBD) score recorded before and after 8 weeks of regular use. Possible side effects were assessed with a semi-structured questionnaire. Patients who started TAI use at least 6 months previously were contacted to assess long-term compliance, efficacy and safety of TAI. RESULTS: After 8 weeks of regular use of TAI, the average NBD scores decreased by four points (P<0.0001) with a specific improvement in the items related to stool frequency (P: 0.036), occurrence of malaise, headache, or sweating during defecation (P: 0.043), use of drugs against constipation (P: 0.007) and frequency of fecal incontinence (P: 0.001). The main side effects were bleeding (10%) and abdominal pain (8%). At 6 months, 80% of the assessed patients had continued regular use of TAI with no particular problem. CONCLUSION: This study showed good medium and long-term efficacy and safety of TAI in the management of bowel dysfunction and defecation disorders in spinal cord-injured patients.

[Post-traumatic focal fixed dystonia of the shoulder: a distinctive syndrome with speculative mechanisms?]. / Dystonie focale fixée de l'épaule à distance d'un traumatisme scapulaire minime : une entité pour quels mécanismes ?

INTRODUCTION: Whether post-traumatic focal fixed dystonia has a physiological or psychologically-mediated mechanism is discussed. CASE REPORT: We report the case of an active 22-year-old soldier with shoulder-fixed dystonia, eight months after a fall with minor right-acromioclavicular sprain. CONCLUSION: Psychiatric examination and search of complex regional pain syndrome, radicular or accessory nerve damage, and genetic predisposition to dystonia are necessary for selecting a difficult treatment in these patients.

Can you guess the colour of this moving object? A dissociation between colour and motion in blindsight.

Blindsight has been primarily and extensively studied by Lawrence Weiskrantz. Residual visual abilities following a hemispheric lesion leading to homonymous hemianopia encompass a variety of visual-perceptual and visuo-motor functions. Attention blindsight produces the more salient subjective experiences, especially for motion (Riddoch phenomenon). Action blindsight illustrates visuo-motor abilities despite the patients' feeling that they produce random movements. Perception blindsight seems to be the weakest residual function observed in blindsight, e.g. for wavelength sensitivity. Discriminating motion produced by isoluminant colours does not give rise to blindsight for motion but the outcome of the reciprocal test is not known. Here we tested whether moving stimuli could give rise to colour discrimination in a patient with homonymous hemianopia. It was found that even though the patient exhibited nearly perfect performances for motion direction discrimination his colour discrimination for the same moving stimulus remained at chance level. It is concluded that easily discriminated moving stimuli do not give rise to colour discrimination and implications for the 3 levels of blindsight taxonomy are discussed.

Isolated exercise-induced rhabdomyolysis of brachialis and brachioradialis muscles: an atypical clinical case.

UNLABELLED: OBJECTIVE AND PATIENT: To report an atypical case of exercise-induced bilateral brachialis and brachioradialis rhabdomyolysis in a 25-year-old woman. DISCUSSION AND CONCLUSION: Persistent focal muscle pain, atypical by its duration and intensity, even after moderate exercise, should prompt the search for rhabdomyolysis and discuss the possibility of acute compartment syndrome. MRI images can validate the muscle edema. Progressive and adapted training as well as respecting individual limits are necessary measures to prevent rhabdomyolysis.

[Artery of Percheron occlusion: Value of MRI. A review of six cases]. / Occlusion de l'artère de Percheron : difficultés du diagnostic clinique et place de l'IRM. À propos de six cas.

Blood supply to the human thalami is complex and multiple variants exist. The artery of Percheron is one of those variants and is characterized by a solitary arterial trunk that branches from one of the proximal segments of either posterior cerebral artery and supplies blood to the paramedian thalami. Its occlusion results in bilateral paramedian thalamic infarction sometimes extending to the midbrain. We report six cases of bithalamic infarction secondary to occlusion of the artery of Percheron. We will illustrate the complex clinical symptomatology and underscore the role of imaging, especially MRI, for diagnosis.

Common peroneal nerve palsy following TNF-based isolated limb perfusion for irresectable extremity desmoid tumor.

Hyperthermic isolated limb perfusion (ILP) (2 mg, TNF-alpha and 100mg, melphalan) was performed for an irresectable right thigh desmoid tumor with calf extension in a 49-year-old man. The patient had a history of four resections since the age of 19 years. Local ILP toxicity appeared with extensive edema and common peroneal neurologic impairment including paresis that remained severe 10 months later. One of the most troublesome side effects of perfusion is peripheral nerve damage, which has been reported at a rate of between 1 and 48% of perfused patients. ILP is an effective treatment in recurrence situations or where resection threatens loss of function; it, however, requires administration in specialized centers, progress in standardization and close monitoring to avoid locoregional toxicity, the mechanisms of which merit further investigation. Emergency compartmental pressure measurement may indicate fasciotomy, can be of great interest.