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Interest in Morvan's disease or syndrome has grown, owing to its close links with various potassium channelopathies. Potassium is crucial for gating mechanisms (channel opening and closing), and especially for repolarization. Defective potassium regulation can lead to neuronal hyperexcitability. There are three families of potassium channels: voltage-gated potassium channels or VGKC (Kv1.1-Kv1.8), inward rectifier K+ channels (Kir), and two-pore channels (K2p). VGK channels are the commonest, and especially those belonging to the Shaker group (neuromyotonia and Morvan's syndrome, limbic encephalitis, and type 1 episodic ataxia). Brain and heart K+ channelopathies are a separate group due to KCNQ1 mutation (severe type 2 long QT syndrome). Kv7 channel mutations (in KNQ2 and KCNQ3) are responsible for benign familial neonatal seizures. Mutation of the Ca+ activated K+ channel gene causes epilepsy and paroxysmal dyskinesia. Inward rectifier K+ channels regulate intracellular potassium levels. The DEND syndrome, a treatable channelopathy of the brain and pancreas, is due to KCNJ1 mutation. Andersen's syndrome, due to KCNJ2 mutation, is characterized by periodic paralysis, cardiac arrythmia, and dysmorphia. Voltage-insensitive K2p channelopathies form a final group.

Limbic encephalitis is an inflammatory disease localized to the "grand lobe limbique" defined by Broca in 1878, sometimes restricted to the hippocampus, but sometimes including extralimbic abnormalities. The main features are subacute onset, short-term memory disorders and cognitive impairment, temporal seizures, and hippocampic changes on MRI. A list of underlying causes has recently been published Infectious causes used to be frequent (mainly herpes simplex virus). Paraneoplastic limbic encephalitis is characterized by the presence of various onconeural antibodies, such as AntiHu and ANNA3 (bronchial small cell carcinoma), AntiMa2 (testicular tumor), AntiCV2 (lymphoma, thymoma,...). No such antibodies are detected in 40% of patients. The prognosis of these forms is poor. Voltage-gated potassium channel-associated limbic encephalopathies are due to antibodies targeting potassium channels. Mutations of the genes encoding the Kv11 and Kv12 subunits are responsible for several Shaker syndromes, including neuromyotonia, Morvan's disease, type I episodic ataxia, and limbic encephalitis with hyponatremia. Plasma exchanges and immunotherapy are effective. In patients without detectable antibodies, hippocampic anti-neuropil antibodies should be sought, particularly those targeting N-methyl-D-aspartate receptors. Ovarian teratoma is the usual cause of this type of encephalitis. Surgery and immunotherapy are effective. These disorders have been categorized into those associated with antibodies targeting intracellular antigens (poor-prognosis paraneoplastic encephalitis) and those associated with antibodies targeting antigens reacting with cellular membranes (potassium channelopathies and antineuropil antibodies), which respond to immunotherapy and carry a better prognosis. Limbic encephalitis can also reveal Hodgkin's disease, as in a case observed by the authors.

Context: Progressive supranuclear palsy (PSP) is classically characterized by supranuclear ophthalmoplegia, paroxysmal imbalance with backward falling, axial dystonia, rigidity, pseudobulbar palsy and cognitive dysfunction. However, incomplete or atypical clinical presentation has been previously reported, but in all these cases, the patients had at least one of the main clinical features of the disease (ophthalmoplegia, parkinsonian syndrome or cognitive dysfunction)., Case Report: A 60-year-old woman presented with nocturnal agitation and choreiform movements. A few months later she developed severe swallowing disorders, caused by achalasia of the upper esophageal sphincter, and responsible for recurrent acute respiratory distress and pneumonia, prevailing to tracheotomy and gastrostomy. She died suddenly two years after the onset of the symptoms., Results: Postmortem examination of brain revealed a tauopathy, with deposition of abnormal phosphorylated tau in threads and in coiled-shaped as well as globose tangles in the brainstem, subthalamic nuclei and hippocampus. Nuclei of the medulla, including the vagus/solitarius complex and the region of the nucleus ambiguous were especially rich in tau positive inclusions. Ultrastructural analysis of globoid-shaped tangles in the brainstem revealed the presence of straight and paired helicoidal filaments compatible with a PSP., Conclusions: This case contributes to improve knowledge of the clinical phenotypic range of PSP. In this case, the neuropathological lesions accounted for most of the symptoms. However, the early death of the patient was probably related to the particular distribution of the neuropathological lesions. This case suggests that the initial neuropathological changes in PSP is located in the dorsal brainstem.

Introduction: Pompe's disease, also called glycogen storage disease type II or acid maltase deficiency, is an autosomal recessive disease caused by an enzymatic deficiency of acid-alpha-glucosidase (GAA). This deficiency causes an accumulation of intralysosomal glycogen in different organs. The classic form appears in the newborn with a very severe hypotonia and cardiomyopathy, which lead to death before age two. Less frequently, the disease appears only in childhood or in adult life, so called late-onset Pompe's disease. This form causes a very progressive limb-girdle myopathy and restrictive respiratory failure. The diagnosis is based on a low level of GAA either in the muscle biopsy or in the leucocytes. We report six cases of late-onset Pompe's disease from the Languedoc-Roussillon district., Method: Our work was a retrospective analysis of all cases of Pompe disease diagnosed in adults between 1975 and 2006 at the Montpellier and Nîmes University Hospital. We describe the clinical presentation and course of this form and explain the diagnostic approach. Results. The mean age at onset was 44.3 years (range: 36-60 years). The first symptom was fatigability (50%), gait difficulty (50%) and dyspnea (16%). The mean delay from symptom onset to diagnosis was 8.4 years (range: 17 years). Fatal outcome due to respiratory failure was noted in three patients. The mean time between symptom onset and death (four patients) was 20.75 years (range: 37 years). The diagnosis was made on the muscle biopsy showing a low level of GAA. Muscle was strictly normal on the morphologic study in one patient, pointing out the requirement for enzymatic analysis. Molecular confirmation was available in one patient., Discussion: Late-onset Pompe's disease is a possible cause of limb-girdle myopathy. Respiratory involvement is a characteristic feature. Enzymatic assay of GAA activity on the muscle biopsy is required for certain diagnosis., Conclusion: It is very important to recognize the adult form of Pompe's disease, a possible cause of limb-girdle myopathy, in order to search for respiratory failure and propose non-invasive ventilation if necessary. Moreover, substitutive therapy (recombinant acid-alpha-glucosidase) has shown efficiency for the classical infantile form of Pompe's disease and such treatment could be proposed for the adult form if larger studies confirm its efficacy.

Introduction: The most frequent acute and sub-acute complications of chronic alcoholism are delirium tremens, hepatic encephalopathy and Gayet-Wernicke encephalopathy. Morel laminar sclerosis is a rare and less known complication, often reported with Marchiafava-Bignami disease., Case Report: A 57-year-old alcoholic man presented delirium after surgery. Anterograde and retrograde amnesia as well as wrong recognitions appeared progressively and one generalized seizure occurred. He then developed mutism and became bedridden. Magnetic resonance imaging (MRI) showed high-intensity bilateral temporoparietal signals from white matter on T2-weighted images and high-intensity signals from the parietal cortex on T1-weighted images. The patient died four months after the onset of the delirium. Post-mortem examination of the brain showed cortical laminar necrosis with Alzheimer Type II gliosis but without demyelinisation of the corpus callosum., Conclusion: Cortical laminar necrosis with chronic ethylism is usually called Morel's laminar sclerosis. Nevertheless, histology is not typical of this diagnosis, because of necrosis especially of the second (and not the third) layer of the cortex, and because of the absence of lesion of the corpus callosum. MRI data are of interest here because they were rarely reported in cases of Morel's laminar sclerosis.

Among neuroeosinophilic syndromes, neuromuscular disorders are considered as a special group, including perimyosistis, polymyositis and fasciitis. These three disorders are considered as a continuum. They usually without a recognized cause, and are considered to be spontaneous or exercise-induced. We report the case of a 43 year-old woman who experienced angioedema followed by an histologically proven-fasciitis with eosinophilia after Ramipril (Triatec) use. Causal attribution to Ramipril was considered "plausible". To our knowledge this side effect has never been reported with this drug.

Leigh syndrome is a heterogeneous disorder, usually due to a defect in oxidative metabolism. Mutations in SURF1 gene have been identified in patients with cytochrome c oxidase deficiency. We report a homozygous splice site deletion [516-2_516-1delAG] in a young girl presenting with cytochrome c oxidase-deficient Leigh syndrome. Identification of molecular defect is indispensable for genetic counselling and prenatal diagnosis.

Andersen syndrome includes a clinical triad with periodic paralysis, cardiac arrhythmia and dysmorphic features most often mild but relevant. It is a potassium channelopathy due to mutation of KCJN2 gene coding for Kir 2.1 protein. We report a familial case with mutation R218W of Kir 2.1 and discuss the main phenotypic and genetic aspects of Andersen syndrome. Muscle manifestations are essentially a periodic paralysis most often of hypokaliemic type. Muscle biopsy reveals tubular aggregates but can be normal as it is shown in the same patient in our kindred. Our proband complained of paralytic attacks since childhood and at adult age she demonstrated a mild permanent deficit of pelvic girdle muscles as it has been described in other types of periodic paralysis after a long duration course. Cardiac manifestations may include in a variable manner a long QT syndrome, premature ventricular contractions, complex ventricular ectopy, polymorphic or bidirectional ventricular tachycardia. Imipramine had a positive effect on arrhythmia in our case. Dysmorphic features are often mild and have to be cautiously looked for as a clue to the diagnosis of Andersen syndrome. They can be easily overlooked if not systematically looked for. Clinical expressivity is variable including in the same family. In our observation, the daughter showed a complete triad, early expressed, which allowed the diagnosis. Her father was late diagnosed on ventricular dysrhytmia but without muscle manifestations and dysmorphic features. Since KCJN2 gene mutation identification, locus heterogeneity of Andersen syndrome was shown. Andersen syndrome kindreds without mutations in KCNJ2 were clinically indistinguishable from KCNJ2-associated subjects. KCNJ2 gene encodes the inward rectifier K+ channel Kir2.1 which plays an important role in maintaining membrane potential and during the terminal phase of cardiac action potential repolarization. Several studies showed a dominant negative effect of the mutation on Kir 2.1 channel function.

We present a case of renal BK virus infection with renal allograft dysfunction. Renal allograft biopsy showed mononuclear infiltrates in the interstitium and viral inclusions in the tubular epithelial cells. Infected cells were stained with an anti-polyomavirus antibody. The polymerase chain reaction (PCR) performed on blood, urine, and on the DNA extracted from renal tissue showed the presence of the BK virus DNA sequence. The immunosuppressive therapy including tacrolimus, prednisone, and mycophenolate mofetil was reduced leading to an improvement of the renal function. BK virus infection is now recognized as a cause of renal allograft dysfunction, and has been observed with increasing frequency in recent years. Reactivation of the latent virus occurs in immunocompromised hosts such as organ recipients with immunosuppressive treatment. Histologically, renal BK virus infection is characterized by a lymphocytic interstitial infiltrate, and could mimic acute rejection. The pathologist should diagnose the viral infection and may be helped by urine cytology and immunohistochemistry. An accurate diagnosis is important because antirejection therapy favors the decline of the renal function. Enhanced new immunotherapy protocols seem to be the main risk factor for this infection. The response to reduced immunosuppression is variable with reports of an end stage renal failure in 70% of the patients after 18 months.

Childhood dilated cardiomyopathies comprise a wide aetiological spectrum for which the prognosis and treatment sometimes vary considerably. We report the case of a patient affected by a rare form of mitochondrial cardiomyopathy in whom the diagnosis of acute myocarditis had initially been made. The progression was fatal even though the patient was awaiting a cardiac transplant. Beyond the difficulties of diagnosis and treatment of this pathology, this clinical case underlines the significance of an early aetiological diagnosis based on the results of an endo-myocardial biopsy. Cardiac transplantation should be envisaged for this type of patient based not only on the clinical status but equally by taking account of the prognosis of this disease for which deterioration can sometimes be very rapid.

Unlabelled: Early and severe cardiomyopathy may be related to myofibrillar myopathy., Case Report: We report a one-year-old child with early and severe restrictive cardiomyopathy. The diagnosis of myofibrillar myopathy was obtained on skeletal muscle and endomyocardial biopsies. The patient died despite inotropic support and mechanical ventilation., Conclusion: Myofibrillar myopathy must be considered when exploring the etiology of a restrictive cardiomyopathy in children. The diagnosis relies on examination of endomyocardial or skeletal muscle biopsy samples.

Neurological symptoms in a patient with large congenital melanocytic naevus are highly suggestive of cerebromeningeal melanoma metastasis. The presence of melanocytic cells in cerebrospinal fluid confirms this diagnosis If their malignant nature is shared with cutaneous naevocytic cells. Conversely, neurocutaneous melanosis is diagnosed when benign melanocytosis meningitis is found in patients with multiple and/or large congenital melanocytic naevus, whether cutaneous naevus cells are benign or not, or when cerebrospinal fluid cells are malignant with benign cutaneous melanocytic naevus. We report the case of a young man aged 19 presenting with multiple and large congenital melanocytic naevus who experienced transcient neurological signs and increased intracranial pressure. Cerebral neuroimaging evoked meningeal infiltration which benign melanocytic nature was supposed on CSF analysis and confirmed by necropsy findings, only 3 month after neurological onset, leading to neurocutaneous melanosis diagnosis. This rare neuroectodermal dysembryoplasia finds expression in various neurological signs, depending on patient's age and leptomeningeal and/or cerebral proliferation localization. Lumbar puncture, cerebral scanography and MRI may help diagnosis, but only histological examination can prove neurocutaneous melanosis, more often by necropsy because of poor prognosis.

Granulomatous inflammation is infrequently observed in muscle biopsy. We report a series of 7 patents presenting with granulomas in muscle. Two of them had a history of sarcoidosis In 4 other cases, muscle Involvement revealed systemic sarcoidosis. Among the 6 cases of sarcoidosis, we observed 2 with acute myositis and 4 chronic forms. The last patient presented with polymyositis in association with melanoma. In sarcoidosis, muscle biopsy showed a granulomatous inflammation of varying intensity, which was generally associated with mononuclear inflammatory cells. Most of granulomas were located in the perimysium and the endomysium and necrosis was absent. Inflammatory cells were predominantly macrophages and CD4 positive lymphocytes. On the contrary, in the case of paraneoplastic polymyositis,granulomas were rare, most of inflammatory cells were CD8 positive lymphocytes and numerous areas of necrosis were observed. Class I MHC molecules were expressed on the membrane of muscle fibers. As a general rule, requisite examinations must be performed to search for sarcoidosis in patients exhibiting granulomas on muscle biopsy.

Orbital myositis is idiopathic inflammation of the extraocular muscles in the absence of thyroid orbitopathy and often is included under broad description pseudotumor. We report here a series of three cases. Data from literature, combined with our own results yield a distinguishing pattern of orbital myositis suggesting that the term "orbital pseudotumor" is no longer a useful concept. The diagnostic criteria purposed here are: acute orbital pain exacerbated on eye movement; enlargement of one or more extraocular muscles with the respect of other orbital structures on muscle CT scan; absence of clinical and biological thyroid dysfunction: absence of signs of anterior uveitis or scleritis or visual decrease; rapid response to immunomodulator treatment.

Dermatomyositis (DM) and polymyositis (PM) are the two main forms of idiopathic inflammatory myopathies. They have in common a proximal muscle weakness, but skin manifestations, juvenile forms and increased incidence of malignancies are clinical characteristics of DM. The follow up of creatine-kinases is the best biological test in spite of their possible normality. The significance of antibodies titers is uncertain, except the association Jo-1 interstitial and lung disease indicating a poor prognosis. The association with HLA haplotypes expresses a genetic predisposition of a dysimmunity to develop DM or PM. Pathological changes are well known with a humoral immune effector mechanism in DM, and a muscle fibre aggression by CD8 + T cells in PM. Non inflammatory forms of DM and PM and rhabdomyolytic forms of PM are not very rare, they are recognized by the HLA class 1 immunoreactivity. Pathophysiological processes involve muscle fibers, inflammatory cells and endothelial cells of capillaries, with a complex intervention of cytokines, adhesion molecules, MHC classe 1, membrane attack complex, anti endothelial cells antibodies, perforin secretion and sometimes apoptotic Fas-mediated mechanisms. Despite these recent advances, causal antigens and activator processes of endothelial cell lysis and autoinvasive cytotoxicity of muscle fibers remain to be identified.

Over the past 10 years, the impact of modern microscopic pathology and molecular genetics on the knowledge of myopathies has been enormous. Dysferlinopathy is a good example. Dysferlin is a surface membrane protein without homology with known mammalian protein excepted otoferlin. It is encoded by a gene on chromosome 2. Miyoshi myopathy and limb girdle muscular dystrophy 2B have been reported to arise from defects in the same genetic locus (chromosome 2p 13). Some personal different examples are presented with typical features, high level of creatine kinase. Gene mutations, immunoblot and immunohistochemistry allow the diagnosis. Three clinical phenotypes are separated: distal myopathy, proximal myopathy, entire lower limbs posterior compartment amyotrophy. Structural changes are mild. Inflammation is not unusual and leads to the diagnosis of polymyositis. There are no correlation genotype-phenotype.

We report 13 French patients with proximal myotonic myopathy. PROMM is a recently delineated multisystem disorder with dystrophic myopathy, myotonia and cataracts. This syndrome is genetically distinct from myotonic dystrophy (DM) by the absence of abnormal CTG repeat expansion. The geographical origin varies but 4 families originated from Poland. Of late onset, muscle weakness is diffuse and predominantly affected proximal and axial muscles. Facial involvement and myotonia were moderate or absent, but in all cases myotonic discharges were detected on EMG. 6 patients suffered from myalgia. Cataracts occurred in 11 patients, mainly indistinguishable from those in DM. Cardiac arrythmia occurred in 7 patients. Muscle biopsy revealed rare structural changes of the muscle fibers and selective type I atrophy, common in DM, could not be found on morphometric analysis. PROMM has a distinct clinical spectrum from DM which includes a predominantly proximal muscle weakness, with troubling pain, a more favourable prognosis and a different histopathological pattern.

Unlabelled: Epstein-Barr virus does not belong to the principal causative agents of acute myocarditis, whose diagnosis and pathogenesis are often difficult to determine. Treatment is also controversial regarding the use of anti-inflammatory or immunosuppressive therapy., Case Report: We describe a 13-month-old girl, admitted for acute heart failure, in whom cardiac catheterization with endomyocardial biopsy revealed an acute myocarditis. Acute viral titers indicated infectious mononucleosis caused by Epstein-Barr virus, and the virus genome was identified with a polymerase chain reaction in the patient's serum. The patient had clinical improvement after corticosteroid administration., Conclusion: The different diagnostic tools and the screening examinations to determine the causative agent of myocarditis are discussed. The frequency of Epstein-Barr virus in pathogenesis is also considered. The favorable outcome with immunosuppressive therapy suggests its administration in cases of acute myocarditis.

Objective: To assess the usefulness of frozen sections (FS) on endocervical margin in surgical conization or loop electrosurgical specimens., Material and Methods: In a prospective study, 150 patients were treated from October 1995 to December 1997: 69 cases without FS, 81 cases with FS. CIN on frozen section resulted in an immediate additional resection., Results: In the group without FS, 13 patients had involved endocervical margin by high-grade CIN (18.8%). Frozen section was impossible in a conization specimen that was too short. FS revealed 64 normal glandular epitheliums, seven squamous metaplasias in which two lesions were under-evaluated (being in fact CIN on permanent sections), eight high-grade CIN followed by additional resection in six cases and two invasive carcinomas. Endocervical margin on additionals section were always free of disease. The rate of failure was 2.6% among 77 cases. This rate corresponded to two under-evaluations. Invasive carcinoma and CIN without additional resection were excluded because frozen section only allowed a peroperative diagnosis. The average height of the cone and the rate of complications were similar. Repeat surgery was necessary in nine cases in the group without frozen section, in which five showed residual lesions, absent in the other group., Conclusion: The ultimate histological interpretation was never difficult after frozen section. This method permits reduction of cases with involved cone margin and residual lesions and, despite some limitations, it may be useful for surgical management.

Unlabelled: MACROPHAGIC MYOFASCIITIS: A most unusual inflammatory myopathy, first described by Germmad had been reported with increasing frequency since 1993 in the leading French myopathology centers. We present our experience with this new disease: macrophagic myofasciitis., Clinical Features: By November 1999, 70 cases of macrophagic myofasciitis had been recorded since our first description. The first 22 patients (sex ratio M/F = 1:3) referred with the presumptive diagnosis of polymyositis (n = 11), polymyalgia rheumatica (n = 5), mitochondrial cytopathy (n = 4), and congenital myopathy or muscle dystrophy (n = 1 each). Symptoms included myalgia (91%), anthralgia (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%)., Laboratory Findings: Abnormal laboratory findings included elevated CK levels (50%), markedly increased erythrocyte sedimentation rate (37%), and myopathic EMG (35%). Muscle biopsy showed a unique myopathological pattern characterized by: i) centripetal infiltration of epimysium, perimysium and perifascicular endomysium by sheets of large cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-, with a PAS-positive content; ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; iii) presence of occasional CD8+ T-cells; iv) inconspicuous muscle fiber damage. The picture was easily distinguishable from sarcoid myopathy and fasciitis-panniculitis syndromes. The infectious diseases know to be associated with reactive histiocytes, including Whippleís disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients improved under corticosteroid therapy and/or immunomodulatory therapeutic, Conclusion: A new inflammatory muscle disorder, characterized by a distinctive pathological pattern of macrophagic myofasciitis is emerging in France.

Purpose: A new type of inflammatory myopathy of unknown etiology has recently been described in France. The myopathy, called macrophagic myofasciitis, had never been described in the literature., Methods: In December 1998, 35 cases of macrophagic myofasciitis were reported, showing an increase in its incidence since the description of the first case in 1993. The first 22 cases are described., Results: The 22 patients were each referred with a presumptive diagnosis of either polymyositis (11 patients), polymyalgia rheumatica (5 patients), mitochondrial cytopathy (4 patients), or congenital myopathy or muscle dystrophy (1 patient for each). Clinical symptoms included myalgias (91%), arthralgias (68%), marked asthenia (55%), muscle weakness (45%), and fever (32%). Laboratory findings included elevated CK levels (50%) and a marked increased in the erythrocyte sedimentation rate (37%). Electromyographic recordings showed the existence of myopathy (35%). Muscle biopsy showed a unique pattern characterized by: (i) centripetal infiltration of the epimysium, perimysium and perifascicular endomysium by non epitheloid, cells of the monocyte/macrophage lineage (CD68+, CD1a-, S100-) with both large cytoplasm and PAS-positive content; (ii) absence of necrosis, of both epithelioid and giant cells, and of mitotic figures; (iii) occasional CD8+ T-cells; and, (iiii) minimal myocyte suffering. The disease symptoms were easily distinguishable from those of sarcoid myopathy and fasciitis-panniculitis syndromes. Infectious diseases known to be associated with reactive histiocytosis, including Whipple's disease, Mycobacterium avium intracellulare infection and malakoplakia, could not be documented. Patients' condition improved under corticosteroid therapy, associated or not with non-specific antibiotic therapy., Conclusion: A new inflammatory muscle disorder of unknown etiology, characterized by a distinctive pathological pattern of macrophagic myofasciitis, is emerging in France. Diagnosis is based on muscular biopsy. Numerous clinical, epidemiological and etiopathologic studies initiated by the GERMMAD (Groupe d'études et de recherche sur les maladies musculaires acquises) are in progress.

We investigated the immunohistochemical expression of endothelial cell adhesion molecules in 17 cases of idiopathic inflammatory myopathies. The present study revealed: 1) a constitutive expression of ICAM-1, ICAM-2 and PECAM-1, but not VCAM-1, LFA-3 and E-selectin, on endothelial cells in normal muscles; 2) a modification of cell adhesion molecules expression in idiopathic inflammatory myopathies with an increased expression of the expressed molecules constitutively; an expression of LFA-3 and VCAM-1 on capillary endothelial cells in polymyositis/inclusion body myositis, these molecules being only observed on endothelial cells of some arterioles in dermatomyositis; 3) an expression of all these molecules on inflammatory cells in inflammatory myopathies. In dermatomyositis, the expression of ICAM-1 and VCAM-1, predominated on mononuclear cells located near vessels. In polymyositis/inclusion body myositis, a strong expression of ICAM-1, VCAM-1 and LFA-3 was present on inflammatory cells invading non necrotic muscle fibers; 4) absence of E-selectin on endothelial and inflammatory cells in all cases.

Malignant Leydig cell tumours of the testis occur in only 0.1-0.3% of patients with testicular tumours. Less than 50 cases of malignant Leydig cells tumours have been previously reported. A report of a new case is presented. This tumour was unusual because of high progesterone level. We analyzed malignant pathologic signs of Leydig cell tumours as immunohistochemical proliferation index. Management of this tumour, for which chemotherapy is not yet available, is discussed.

We describe the main methods of gross examination of renal tumours. The pathologic result should refer to the TNM staging, which was achieved by UICC and AJCC in March, '97.

Neuromuscular diseases with eosinophilia include a number of disorders associated with variable degrees of muscle, peripheral nerve, and connective tissue involvement. Eosinophilic infiltration in blood and/or tissue is a consistent finding. In addition to the neurologic manifestations of systemic vascularitis, in particular Churg and Strauss syndrome, there are three main forms of neuromuscular disease. Diffuse fasciitis or Shulman syndrome which can be limited to the fascia or associated with perimyositis is sensitive to corticosteroids. Eosinophilic myositis corresponds to focal muscle involvement and is also sensitive to corticosteroids. Eosinophilic polymyositis is a manifestation of essential hypereosinophilic syndrome and is life-threatening. Eosinophilia-myalgia syndrome and toxic oil syndrome are separate entities that occur in outbreaks and involve poisoning by ingestion of L-tryptophan and adulterated oil containing aniline respectively. The key to diagnosis of these neuromuscular diseases is muscle biopsy to detect the presence of polynuclear eosinophils.

The incidence of neurosyphilis has been increasing since AIDS has appeared but acute gummatous necrotizing progression is so far exceptional. We report a second case of "quaternary neurosyphilis" in a 29-year-old patient without serologic nor CT scan evidence of treponema. Diagnosing neurosyphilis is particularly difficult in HIV-1 infected patients, specially in spirochetes-poor injuries.

The authors report two cases of aortic angiosarcomas. These cases were similar and characterized by a typical pejorative clinical course, with peripheric emboli and many osteolytic metastasis. Immunohistochemical study was in favour of an endothelial origin, electronic microscopy indicated a mixed tumor with myofibroblasts and poorly differentiated endothelial cells. Differential diagnosis between angiosarcoma, leiomyosarcoma and intimal sarcoma are reviewed.

The clinical and pathological findings of a 43-year-old woman, diagnosed as having acute hemorrhagic leukoencephalitis at postmortem examination, are presented. The acute hemorrhagic leukoencephalitis affects mainly young adults and is the most fulminant from of demyelinating disease. It is frequently preceded by a respiratory infection. Diagnosis is facilitated by CT scanning and MRI, which reveal the massive lesion in the cerebral white matter. Many cases terminate fatally in 2 or 4 days, but in others survival is longer. The pathological findings are distinctive.

Muscle biopsy is a simple procedure of great diagnostic interest when correct processing is obtained. Specimens for molecular genetics are very important. Paraffin embedded material is not sufficient in most of the cases except in some inflammatory processes. Histochemical techniques on frozen material are of major interest. Immunohistochemistry and electron microscopy can also be used. The aim of this paper is to describe the procedure of the muscle biopsy, the various techniques and the possible artefacts.

Acute myopathy occurred in a 49-year-old woman hospitalized in the intensive care unit for status asthmaticus. She was given high-dose intravenous steroid therapy and intubated. Pancuronium bromide was used for prolonged curarization. Flaccid quadriplegia developed with preservation of the deep tendon reflexes. Muscle biopsy showed a myogenic process with disorganized myofibrils and selective loss of thick myosin filaments. This mainly myogenic process would result from the toxic effect of corticosteroids favored by prolonged curarization although the effect of other factors still remains unknown.

The viscoelastic properties of the arterial wall are responsible for the blood pressure wave propagation throughout the arterial system. Arterial diseases may cause disorders in this propagation. We have developed a mock circulation system that allows assessment of viscoelastic properties in fresh or cryopreserved human arteries. It includes the following components:--a hydrodynamic generator that can simulate physiological pressure variations in fresh segments of human arteries;--a lightweight miniature flexible probe that can be placed around the artery to measure changes in the external diameter during systolo-diastolic cycles;--a computer program to analyse pressure and diameter data measured during a cardiac cycle. Using this system, it is possible to evaluate the main viscoelastic properties of the arterial wall (arterial compliance, arterial stiffness, midwall aortic stress, Young elastic modulus, incremental modulus). Human arterial samples were collected during organ harvesting in subjects from 18 to 35 years of age. Correlation between viscoelastic properties, arterial wall status, and histological aspect in nitrogen vapor (-140 degree C) were established. No statistically significant difference was observed in femoral arteries characteristics. Compliance decreased, while stiffness increased with statistically significant difference after cryopreservation in carotid arteries. No histological difference was observed in both arteries before and after cryopreservation.

The case of a 41 years old man presenting with mitochondrial myopathy associated with calcification of the basal ganglia (Fahr's disease) neurosensorial and endocrine-deficits is reported. These different symptoms could share a common physiopathological process.

Cerebral angiotropic large cell lymphoma is a rare fatal neurologic disorder characterized by multifocal intravascular proliferation of large pleomorphic cells within vessels of all caliber, predominantly skin and nervous system. Clinical manifestations in previously reported cases were dominated by focal neurologic signs, epilepsia and progressive dementia. We report a case of a 70 year-old man with subacute dementia, epileptic seizures and cerebrovascular events. There was no evidence of a systemic disease outside the nervous system. Cerebrospinal fluid contained 13 leukocytes/mm3 (49% of lymphocytic cells) and more than 100 mg/dl of protein. Cytology was negative. Cranial MRI demonstrated cerebral atrophy and an increased paraventricular signal in T 2 weighted images. A frontal brain biopsy revealed only neuronal dystrophy and astrocytic gliosis. Despite treatment with corticosteroids the patient died 18 months after the onset of the first symptoms. Autopsy was performed and revealed B cell lymphoma.