[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.

[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, 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.

[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.

[Syndrome of headache with neurologic deficits and CSF lymphocytosis]. / Syndrome de céphalées et déficits neurologiques transitoires avec lymphocytose du liquide céphalorachidien.

INTRODUCTION: A syndrome of headache with neurologic deficits and cerebrospinal fluid (CSF) lymphocytosis is uncommon and clinicians should be aware of this entity. EXEGESIS: We report a 28-year-old man without previous medical history of migraine, who presented with severe headache and temporary focal, neurological deficits. Lumbar puncture revealed aseptic lymphocytic pleiocytosis. The patient completely recovered within one month. This condition was suggestive of a transient syndrome of headache with neurologic deficits and lymphocytosis. The main characteristics and the pathophysiology of this uncommon disorder, generally with a benign course, are discussed. CONCLUSION: Such syndrome of headache, neurologic deficits and CSF lymphocytosis should be included in the differential diagnosis of meningo-encephalitis. The constant benign course of this affection should be emphasized.

[Reversible acute postpartum cerebral angiopathy]. / Angiopathie cérébrale aiguë réversible du post-partum.

Acute cerebral angiopathy is a rare neuro-vascular complication in postpartum. In this setting, the implication of vasoconstrictive drugs used for lactation or deliverance hemorrhage inhibition has been established. This review aimed 1) to describe, epidemiologic, clinical and diagnostic features of this pathological condition. 2) To put in perspective this condition within the scope of neurovascular clinical syndrome of pregnancy.

[Polyneuropathy involving cranial nerves associated with monoclonal IgM antibodies with anti-MAG/SGPG/SGPLG/sulfatides activity]. / Une polyneuropathie avec atteinte des nerfs crâniens associée à une IgM monoclonale à activité anti-MAG/SGPG/SGPLG/sulfatides.

INTRODUCTION: A typically distal and symmetrical, slowly progressive sensorimotor demyelinating neuropathy is caused by monoclonal IgM against myelin-associated glycoprotein (MAG) and SGPG, SGLPG glycolipids in the context of a benign IgM paraproteinemia. We studied a patient with a neuropathy that fulfilled the diagnostic criteria for CIDP in whom IgM kappa anti-MAG/SGPG/SGLPG were detected. OBSERVATION: The patient was a 57-year-old man who had developed a slowly progressive distal sensorimotor neuropathy, involving the lower then upper limbs, with cranial nerves palsies (oro-pharyngo-laryngo territory). ENMG showed a demyelinating neuropathy with a disproportionate slowing of conduction in distal segments of motor and axonal features in the lower limbs. The first routine laboratory analysis revealed negative or normal findings. Several serum protein electrophoreses were normal. The third cerebrospinal fluid examination demonstrated a moderate and late rise in CSF protein level with no cells. Monoclonal IgM-kappa against MAG/SGPG/SGLPG, was detected; anti-MAG antibody titre in the serum was 20 059 BTU (N<1000). A small IgM-kappa paraprotein was identified by immunofixation. Electron microscopy failed to show nerve fibers with widening of outer lamellae of the myelin. There is no clinical improvement after different treatments, immunoglobulins IV, cortisteroids, plasma exchange, rituximab. CONCLUSION: It is not known whether this neuropathy is an atypical form of PNMAG or an CIDP associated with anti-MAG. When ENMG show a disproportionate slowing of conduction in distal segments of motor nerves, one should screen the serum with immunofixation to identify small monoclonal components. If IgM-MGUS is present, search should be undertaken for anti-MAG/SGPG/SGLPG antibodies. Diagnosis enables optimal treatment using, in severe cases, expensive current strategies with immunoglobulins IV, plasma exchange, and corticosteroids, or, in the event of no response, rituximab before resorting to more toxic drugs like cyclophosphamide.

[Efficacy of a cognitive-behavioral program with pharmacological support to achieve smoking cessation]. / Effektivität eines kognitiv-verhaltenstherapeutischen Raucherentwöhnungsprogramms mit pharmakologischer Unterstützung.

BACKGROUND AND OBJECTIVE: In Germany over 30% of the population are smokers. Only very few smokers succeed in giving up smoking, because of the addictive nature of nicotine use. The objective of the present study was to evaluate a smoking cessation program which was recently established at a tertiary care clinic. PATIENTS AND METHODS: The median age of the 275 participants was 45.5 years; 60.4% of participants were female. Participants had tobacco dependence of 5.1 (Fagerstrom scale). The cognitive-behavioral smoking cessation program implemented in this clinic consisted of 8 sessions. Pharmacological support was recommended. A standardized evaluation was carried out after 6 and 12 months. RESULTS: All 275 smokers took part in the program over a period of 21 months. At the end of the course, 72% of participants had become non-smokers, after 6 months 37.6% and after 12 months 27.1% had stopped smoking. Those who have not yet been evaluated 12 months after the end of the program demonstrated a point prevalence of 45.7% after 6 months. The cigarette consumption of participants who were smoking at the end of the course was reduced to less than half. 6 months later they smoked an average of 18.0 cigarettes /day; 12 months after ending the course, 19.0 /day were consumed (p<0,001). Costs of about 250 Euro per gained life year were approximated. CONCLUSION: It is possible to establish a smoking cessation program at a large clinic. The results correspond to international published experiences and confirm that such a program is efficacious and cost-effective.

[Crossed anarthria and a dissociated lateralisation of language]. / Anarthrie croisée et latéralisation dissociée du langage.

INTRODUCTION: Crossed anarthria cases are uncommon and rather old. OBSERVATION: We report the case of a right-handed 55-year-old man who presented crossed pure anarthria due to a hemorrhage in the premotor cortex (feet of F1 and F2) and in the high part of Pierre-Marie's quadrangle. CONCLUSION: The study of different tasks (articulation, verbal fluency, direct object word-generation from a verb) showed a dissociated lateralisation of his language. Lexico-semantic and grammatical tasks are processed in the left hemisphere. Articulation programming occurs in the right hemisphere.

[Split-cord malformation (diastematolmyelia) presenting in two adults: case report and a review of the literature]. / Diastématomyélie révélée chez l'adulte: étude de deux cas et revue de la littérature.

INTRODUCTION: Split cord malformation (SCM) is an uncommon developmental anomaly characterized a cleft spinal cord. In type I, each of the hemicords is contained within an individual dural tube whereas in type II there is a common dural tube housing both hemicords. Commonly diagnosed in childhood, adult presentation is exceptional. METHODS: We report the case of two women whose type II SCM was discovered at the age of 40 and 54 years. RESULTS: The first patient complained of chronic lombar and radicular chronic pain with dysuria. Physical examination revealed a radicular syndrome with abolition of the left Achille reflex and a lombar hair tuft. MRI showed a disc herniation at the L5-S1 level, with a partial SCM at the level of the L2 vertebra, spina bifida and tethered cord. The second patient complained of lombar pain with perineal irradiation for 6 years. Physical examination showed a lombar cutaneous angioma. MRI revealed a thoraco-lombar SCM at the T12 to L1 level, with spina bifida. No spur could not be identified in either patient. No further surgical treatment was given. CONCLUSION: Based on these two observations, we propose a review of literature reporting 90 cases of adult SCM.

[Paraneoplasic pandysautonomia with anti-Hu antibodies: a presentation of pulmonary adenocarcinoma identified by PET scanning]. / Pandysautonomie paranéoplasique avec anticorps anti-Hu: révélation d'un adénocarcinome bronchique par le PET scan.

A 74-year-old man developed pandysautonomia with severe orthostatic hypotension. Search for a paraneoplastic etiology was confirmed with the positivity of anti-Hu antibodies. [18] Fluorodeoxyglucose positron emission tomography (PET) enabled recognition of a lung adenocarcinoma. We emphasize the usefulness of PET scans for early diagnosis of cancer.

[Acute severe leukoencephalitis with posterior lesions due to Borrelia burgdorferi infection]. / Leuco-encéphalite aiguë à tropisme postérieur révélant précocement une maladie de Lyme.

INTRODUCTION: Central nervous system manifestations represent 0.54 to 8% of neurological complication in Lyme disease. OBSERVATION: A 78-year-old woman presented a severe meningo-encephalitis with visual disorders (agnosia, alexia) progressing towards coma. Cranial magnetic resonance imaging revealed large areas of hypersignal T2 in the white matter of the lower, parieto-occipital lobes and left temporal lobe. The cerebrospinal fluid (CSF) contained 16 then 293 white corpuscles/mm3 of lympho-monocytes, increased protein level from 2.67 to 5.83 g/l and an increase in IgG index with oligoclonal distribution of IgG. Serological Elisa analysis for Lyme disease was slightly positive in blood (confirmed by western blot) but clearly in the CSF (IgG and IgM). Treatment with ceftriaxone followed by methylprednisolone provided clinical improvement 3 months later. DISCUSSION: Acute meningo-encephalitis is often benign, protein-like and of good prognosis: the gnosic visual disorders with posterior leukoencephalopathy are unusual. A blood level of specific antibodies slightly positive on Elisa at the early stage of the infection warrants confirmation by Western blot in the blood and by Elisa in the CSF. Additional corticosteroid therapy may be required in the severe forms that evoke acute disseminated encephalomyelitis.

[Hashimoto's thyroiditis associated with recurrent transverse myelopathy]. / Myélite transverse à rechutes associée à une thyroïdite d'Hashimoto.

A 55-year-old woman presented first episode of paraplegia at D6 level with a marked improvement of neurological signs, but relapse occurred five months later. Hashimoto's thyroiditis with euthyroidism was found. Long-term 9 months treatment with oral corticosteroids was applied. Also she was able to walk with help. Two years after the second palsy she developed a new paraplegia at D6 level with a severe residual deficit five months later in spite of oral corticosteroids. Thyroid antibodies remained positive. Devic's disease without optica neuritis associated with thyroiditis or spinal cord localisation of Hashimoto's encephalopathy was discussed.

[Calcified brain metastases of a pulmonary neuroendocine carcinoma]. / Métastases cérébrales calcifiées d'un carcinome bronchique neuro-endocrine.

We describe an unusual case of pulmonary neuroendocrine carcinoma of intermediate cell type, revealed by calcified metastatic nodules of the brain in a 49-year-old man. Histological examination of the brain lesions disclosed necrosis and calcification in the center with small and medium-sized carcinoma cells. Pathophysiological mechanisms of such calcifications in metastasis lesions are reviewed.