Neonatal pancytopenia in a child, born after maternal exposure to natalizumab throughout pregnancy.

Natalizumab is a monoclonal antibody indicated for the treatment of patients with relapsing-remitting multiple sclerosis. Its use is prohibited during pregnancy. However, natalizumab exposures throughout the gestation period or during the third trimester, because of intense disease activity, are possible and begin to be reported. There are enough reassuring arguments against a teratogenicity, through pregnancy registries; but deleterious effects in the monitoring of newborn, are not well known. A disorder of hematopoiesis is possible with anemia, thrombocytopenia or pancytopenia, as discussed by the author through an observation. These hematological disorders seem to be asymptomatic; they resolve spontaneously and require a simple biological and clinical monitoring of the newborn.

[Impact of headache among studied military population in Afghanistan deployed in the Kabul military field hospital]. / Impact des céphalées parmi le personnel militaire déployé au sein de l'hôpital médico-chirurgical de Kaboul.

INTRODUCTION: Headaches are a common reason for consultation with a prevalence of 30%. Few data exist for military personnel, including in situations of war operations. The main objective of this work was to measure the evolution of the impact of headache in such a context. MATERIALS AND METHODS: Two hundred and one personnel deployed in the Kaïa military field hospital in Afghanistan were recruited. A questionnaire designed to recognize headaches, supported by two quality of life scales (MIDAS and HIT-6) and a stress questionnaire were filled out before departure and upon return from missions. DISCUSSION: Sixty-three patients with headache were initially identified, of whom 52 remained symptomatic during the mission. The average total score of MIDAS before departure was 4 days and fell to 1.4 days upon return, with a mean measured change of 3.3 days. For HIT-6, the mean total score was 51.2 points initially and 51.9 points at the end of the mission with a mean change of-0.3 points. Nine patients without headache initially became symptomatic: MIDAS and HIT-6 were not affected. CONCLUSION: Thus, the impact of headache in the particular context of presence in a theater of operations was low: improved MIDAS score and the lack of influence on the HIT-6 score are underlined.

[Type 2 neurofibromatosis: intergenerational differences in genetic and clinical expression]. / Neurofibromatose de type 2 : différence inter-générationnelle d'expression génétique et clinique.

INTRODUCTION: Neurofibromatosis type 2 (NF2) is a rare dominantly inherited disease. Its clinical presentation can be completely different in children and adults and early diagnosis is often difficult. The NF2 gene molecular analysis can help for diagnosis, but its result can be negative in case of NF2 mosaicism. OBSERVATIONS: We report the case of a 43-year-old man who had developed a severe phenotype with bilateral vestibular schwannomas at 19 years of age. His son presented a retinal hamartoma with loss of vision in his right eye at 2 months of age. At 9 years of age, asymptomatic schwannomas of the cranial nerves were discovered: cranial nerves X (left), XI (left), and VIII (bilateral). Partial constitutional NF2 deletion (from exons 2-7) was detected in his son. The deletion was not detectable in the DNA blood of his father and we strongly suspect a mosaic form of NF2. CONCLUSION: Ophthalmological manifestations can be the initial sign of NF2 in childhood. These features must be actively sought during the first year of life in individuals at risk of NF2. NF2 mosaicism is often described as a mild form of NF2 with a very low risk of transmission to the carrier's children. We show that NF2 mosaicism can sometimes develop severe NF2 symptoms and we confirm that the transmission risk to the offspring depends on the proportion of zygotes carrying the mutation. NF2 remains a life-limiting and life-spoiling condition. Early diagnosis is necessary to prevent complications and the follow-up of NF2 patients must be organized throughout life in specialty centers.

[Ataxia with ophthalmoplegia: Miller-Fisher syndrome with anti-GQ1b antibody positivity]. / Ataxie avec ophtalmoplégie : syndrome de Miller-Fisher avec positivité des anticorps anti-GQ1b.

Miller-Fisher syndrome is defined as ophthalmoplegia, ataxia and areflexia. Considered as a variant of Guillain-Barré syndrome, it differs in its clinical presentation and by anti-GQ1b antibody positivity. The authors report a case of Miller-Fisher syndrome characterized by ataxia and complete ophthalmoplegia. Through this example, the range of ophthalmologic clinical manifestations are discussed.

[Dysautonomic syndrome of the face with Harlequin sign and syndrome: Three new cases and a review of the literature]. / Syndromes dysautonomiques du visage avec signe et syndrome d'Harlequin : étude de trois cas et revue de la littérature.

INTRODUCTION: Harlequin phenomenon is characterized by a strictly unilateral erythrosis of the face with flushing and hyperhydrosis, and controlaterally a pale anhydrotic aspect. This syndrome can occur alone or associated to other dysautonomic phenomena such as Horner syndrome, Adie syndrome or Ross syndrome. PATIENTS AND METHODS: We report three cases: two patients presented a Harlequin sign, associated with Horner syndrome for one and Ross syndrome for the second. The etiologic investigation was normal, allowing recognizing the idiopathic nature of the disorder. For the third patient, Harlequin syndrome was observed in a neoplastic context due to breast cancer, metastatic dissemination, and bone metastases involving the right side of the T2 body. DISCUSSION: We reviewed the literature: 108 cases have been described. This syndrome occurred alone in 48 patients and was associated with other dysautonomic syndromes such as Horner syndrome in 38 patients, Holmes Adie syndrome in six, and Ross syndrome in six; both Ross and Holmes Adie syndrome were associated five cases and associations were not reported in five patients. The pathophysiological mechanisms of this autonomic cranial neuropathy, the possible etiologies, and therapeutic management were discussed. CONCLUSION: Harlequin phenomenon with flushing and unilateral hyperhydrosis is rare, occurring alone or in combination with other autonomic syndromes of the face. Idiopathic in two-thirds of cases, Harlequin phenomenon does not require specific treatment; sympathectomy may be discussed in the severe cases with a significant social impact.

[Partial visual seizures induced by non-ketosic hyperglycemia: magnetic resonance imaging and visual evoked potential descriptions. A study of two cases reports with radiologic and electrophysiologic abnormalities]. / Crises partielles à point de départ visuel compliquant une hyperglycémie sans cétose: IRM et potentiels évoqués visuels; étude de deux cas avec anomalies radiologiques et électrophysiologiques.

INTRODUCTION: Non-ketosic hyperglycemia (NKH) may increase the likelihood of focal epileptic seizures, including commonly motor expression; rarely, they can have a visual expression. METHODS: The authors describe the observation of two men, who were hospitalized for visual manifestations; with episodes of homonymous hemianopia and hallucinations, revealing occipital seizure, secondary to NKH. Clinical data and characteristics of the investigations, including radiological imaging (MRI) and electrophysiological results of visual evoked potentials (VEP) are specified. RESULTS: MRI showed transitory low signal on T2 and FLAIR in occipital areas. Spectro-MR identified a moderate diminution of the NAA and lipids spikes, compatible with laminar necrosis. VEP revealed a transient decrease of the P100 amplitude. DISCUSSION: These two observations underline the existence of acute symptomatic seizures with a visual starting point which is often indicative of diabetes. Through these observations with a review of 28 patients from the literature, MR imaging characteristics and possible anomalies collected on VEP are discussed. Such seizures are resistant to anticonvulsant treatment and respond best to insulin and rehydration. CONCLUSION: The visual manifestations indicative of seizures with an occipital starting point in the context of NKH are possible enabling rapid initiation of effective symptomatic treatment with insulin.

Neuropathic complications after 157 procedures of continuous popliteal nerve block for hallux valgus surgery. A retrospective study.

BACKGROUND: Continuous peripheral nerve block (CPNB), in particular at the popliteal fossa, is widely used in orthopedic surgery, allowing good postoperative analgesia. Possible neuropathic complications, however, remain poorly known. OBJECTIVE: To review the characteristics of peripheral neuropathy (PN) after sciatic CPNB at the popliteal fossa, estimating prevalence, severity, evolution and possible risk factors, especially those relating to the procedure. METHODS: Retrospective study of PN associated with popliteal fossa CPNB for hallux valgus surgery, between November 1st, 2005 and November 1st, 2009. All procedures were analyzed (type of anesthesia, approach, nerve location technique, number of procedures by operator) with, for each case of PN, analysis of clinical and electromyographic data. RESULTS: One hundred and fifty seven sciatic CPNBs were performed (92% women; mean age, 55 years). The approach was lateral (n=62), posterior (n=74) or unknown (n=21). Ultrasound guidance was combined to neurostimulation for 69 patients (44%). Three women (prevalence=1.91%), aged 19, 24 and 65 years respectively, developed associated common superficial peroneal and sural nerve injury (2), axonal on electromyography, with motor (n=1) and/or sensory (n=3) residual dysfunction. DISCUSSION: The higher prevalence found in the present study than in the literature (0 to 0.5%) raises questions of methodological bias or technical problems. The common peroneal and sural nerves seem to be exposed, unlike the tibial. Several mechanisms can be suggested: anesthetic neurotoxicity, direct mechanical lesion, or tourniquet-related ischemia and conduction block. Further studies are necessary to determine the ideal anesthetic procedure. CONCLUSION: Patients should be informed of the potential risk, however rare, even during mild surgery. The best possible technique should be implemented, with reinforced surveillance.

[Ischemic stroke as the first manifestation of Wegener's granulomatosis]. / Une manifestation inaugurale inhabituelle de la granulomatose de Wegener : l'accident vasculaire cérébral ischémique.

The most characteristic clinical manifestations of Wegener's granulomatosis are upper and lower respiratory tract and renal involvement. The central nervous system manifestations are uncommon and occur usually late in the course of the disease. We report a 48-year-old man who presented with an ischemic stroke as the first manifestation of Wegener's granulomatosis. Wegener's granulomatosis should be considered in the differential diagnosis of ischemic stroke even in the absence of extraneurological involvement.

[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.

[Thyrotoxic hypokaliemic periodic paralysis revealing Graves' disease in a male Caucasian]. / Paralysie périodique thyréotoxique hypokaliémique révélatrice d'une maladie de Basedow chez un caucasien.

OBJECTIVES: Hypokaliemic thyrotoxic periodic paralysis (TPP) is an uncommon complication of hyperthyroidism. Mostly described among Asian patients, it is rare in other ethnic groups, in particular in Caucasian people. CASE REPORT: We present the case of a Caucasian male admitted to our unit after several paretic episodes. Tachycardia, goiter and mild proptosis led to the diagnosis of Graves' disease. CONCLUSION: Rare in the Caucasian population, TPP involves dysfunction of the NA-K-ATPase pump. Beta-blockers should be associated with medical or surgical treatment of hyperthyroidism.

[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.

[Brain abscess as the first clinical manifestation of isolated pulmonary arteriovenous malformation without Rendu-Osler disease]. / Abcès cérébelleux révélant une malformation artérioveineuse pulmonaire isolée en dehors de la maladie de Rendu-Osler.

INTRODUCTION: Brain abscesses occur in 5 to 13 % of patients with pulmonary arteriovenous malformation (PAVM), more often present in Rendu-Osler-Weber disease or hereditary hemorrhagic telangiectasia (HHT). CASE REPORT: A 51-year-old man with a history of transient Parinaud syndrome at 37 years complained of headache for 2 months before acute onset of a left cerebellar syndrome without fever. CT-scan and MRI of the head revealed a heterogeneous left cerebellar lesion. A brain abscess was drained and all signs resolved. CT-scan of the chest revealed a left lingual PAVM; occlusion was incomplete after coil embolization. He had no feature of HHT and no mutation in ENG and ACVRL1 genes. A second embolization was performed 5 months later, but the malformation was not occluded at 6 months. DISCUSSION: We report the seventh case of PAVM complicated by a cerebellar abscess. The right to left shunt in PAVM results in hypoxemia, secondary polycythemia and paradoxical embolization of infective organisms bypassing the pulmonary filter. CONCLUSION: Combining different MRI techniques (in particular diffusion and proton MR spectroscopy) provides invaluable data for the diagnosis of brain abscess. Careful search for PAVM must be undertaken, particularly in adults with cryptogenic abscess, to avoid further abscess formation or stroke.

[Susac syndrome: variable onset modes and disease courses: two case reports]. / Syndrome de SUSAC. Modes d'entrée et évolutions variables: à propos de deux cas.

INTRODUCTION: Susac syndrome is a rare microangiopathy of unknown etiology, which involves the clinical triad of encephalopathy, visual loss, and hearing loss. Several onset and progression patterns are possible. OBSERVATION: Case 1: a 43-year-old woman developed subacute encephalopathy, which had not been diagnosed and had been evolving for 2 months, as well as left sensorineural hearing loss. The fundus exam found central artery branch occlusion in the left retina. The treatment was based on corticosteroids combined with cyclophosphamide and immunoglobulins. Angiographic monitoring revealed persistent asymptomatic arterial alterations despite positive neurological progression. Case 2: a 27-year-old woman presented visual loss in the right eye after recurrent neurological episodes. The triad was completed by deafness in the right ear. Treatment with corticosteroids led to favorable neurological progression and stabilized the ophthalmologic symptoms. DISCUSSION: This syndrome preferentially affects young women. The nearly constant neurological symptoms can differ. Branch occlusions are frequently bilateral and often come with the appearance of vasculitis. Deafness is bilateral, asymmetrical, and of endocochlear origin. Brain MRI shows lesions of the corpus callosum that are distinctive of the syndrome. The disease mainly evolves in a monocyclic way, self-limited in time, and it rarely becomes chronic. Treatment, which has not been codified to date, is based on corticosteroids and, in severe cases, immunosuppressive drugs. Other therapies have not proved to be effective. CONCLUSION: The diagnosis is based on the triad of neurological, ophthalmic, and ENT damage, but sometimes it can be difficult to formulate because of the chronology of symptom onset. Neurological damage, the first manifestation, will help make therapeutic decisions.

[Susac syndrome, retinocochleocerebral microangiopathy: contribution of MRI]. / Le syndrome de Susac, microangiopathie rétinocochléocérébrale : apport de l'IRM.

This is a new case of Susac syndrome in a 27-year-old woman with polymorphic neurological disorders, her brain MRI showed multifocal hyperintense signals on T2-weighted images with possible effects on the corpus callosum. However, visualization of an occlusion in the retinal arterial branch of the right eye and hypoacusia on the right side allowed confirmation of the diagnosis. In this case report, we describe the imaging aspects of Susac syndrome and demonstrate that brain MRI allows the syndrome to be diagnosed at an early stage.

[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.

[Supervision of low-grade gliomas with multiparametric MR imaging: research of radiologic indicators of malignancy transformation]. / Intérêt de l'IRM, avec séquences de diffusion, de perfusion et de la spectrométrie dans le diagnostic et la surveillance de gliomes d'aspect initial de grade 2 : recherche de marqueurs radiologiques orientant vers une aggravation tumorale de grade.

We assessed the contribution of diffusion, perfusion and spectroscopy imaging for the diagnosis and follow-up of intraaxial tumors, suspected to be grade II gliomas. Twenty-four patients were included from April 2005 to July 2006, 17 initially and seven during their follow-up. The diagnosis was reconsidered in a first group of six patients: a high-grade tumor was suspected and confirmed in five. These patients presented a lipid peak; the perfusion results and the CHO/Cr and CHO/NAA ratios were not pathological. The second group included patients with grade II gliomas: these 18 patients had a radiographic work-up, initially, then at three months and every six months. For this group, no evidence of a change of grade were observed. Abnormal findings were noted in seven patients: among these patients, one developed radiographic progression, one other had radiographic progression associated with a spectroscopy lipid peak; only spectroscopy changes were noted in the third patient; the last patient had radiographic progression with perfusion and spectroscopy abnormalities; these four patients were treated. These observations suggest that diffusion, perfusion and spectroscopy can provide supplementary information for diagnosis and follow-up of glial tumors. The presence of a lipid peak is of particular value. The limitations of this work must also be taken into consideration: the follow-up was too short for slow-growing gliomas; the population was small and patients may have undergone surgery during the study, leading to structural modifications which may have compromised comparisons. This work should be continued with new examinations every six months and inclusion of new patients.

[Adie's tonic pupil and migraine: a chance association?]. / Pupille tonique d'Adie et migraine: une association fortuite?

INTRODUCTION: Adie s tonic pupil is defined as a dilated pupil, constricting poorly to light and exhibiting light-near dissociation. CASE REPORT: The authors report an 18-year-old woman diagnosed with transient bilateral Adie's tonic pupil during a migraine attack. Work-up including neuroiaeaging disclosed no underlying detectable abnormalities. DISCUSSION: The association between tonic pupil and migraine is rarely reported. This type of transient mydriasis could result from postganglionic parasympathetic dysfunction, affecting the iris sphincter. This situation is different from the heterogeneous group with benign episodic unilateral mydriasis. CONCLUSION: Transient Adie's tonic pupil may be associated with migraine attacks. This association should be known to avoid unnecessary examinations.