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1.
Fish Shellfish Immunol ; 100: 219-229, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-32160965

RESUMO

The use of terrestrial raw materials to replace fish meal (FM) and fish oil (FO) in marine fish diets may affect fish growth performance and health. In the last years functional additives have been profiled as good candidates to reduce the effects on health and disease resistance derived from this replacement, via reinforcement of the fish immune system. In the present study, three isoenergetic and isonitrogenous diets with low FM and FO (10% and 6% respectively) were tested based on supplementation either with 0.5% galactomannanoligosaccharides (GMOS diet) or 0.02% of a mixture of essential oils (PHYTO diet), a non-supplemented diet was defined as a control diet. Fish were fed the experimental diets in triplicate for 9 weeks and then they were subjected to a stress by confinement as a single challenge (C treatment) or combined with an experimental intestinal infection with Vibrio anguillarum (CI treatment). Along the challenge test, selected stress and immunological parameters were evaluated at 2, 24 and 168h after C or CI challenges. As stress indicators, circulating plasma cortisol and glucose concentrations were analyzed as well as the relative gene expression of cyp11b hydroxylase, hypoxia inducible factor, steroidogenic acute regulatory protein, heat shock protein 70 and heat shock protein 90 (cyp11b, hif-1α, StAR, hsp70 and hsp90). As immune markers, serum and skin mucus lysozyme, bactericidal and peroxidase activities were measured, as well as gene expression of Caspase-3 (casp-3) and interleukin 1ß (il-1ß). The use of functional additives induced a significant (p < 0.05) reduction of circulating plasma cortisol concentration when confinement was the unique challenge test applied. Supplementation of PHYTO induced a down-regulation of cyp11b, hif-1α, casp-3 and il-1ß gene expression 2h after stress test, whereas StAR expression was significantly (p < 0.05) up-regulated. However, when combination of confinement stress and infection was applied (CI treatment), the use of PHYTO significantly (p < 0.05) down-regulated StAR and casp-3 gene expression 2h after challenge test, denoting that PHYTO diet reinforced fish capacity of stress response via protection of head kidney leucocytes from stress-related apoptotic processes, with lower caspase-3 gene expression and a higher il-1ß gene expression when an infection occurs. Additionally, dietary supplementation with GMOS and PHYTO compounds increased fish serum lysozyme after infection. Both functional additives entailed a better capability of the animals to cope with infection in European sea bass when fed low FM and FO diets.


Assuntos
Ração Animal/análise , Bass/imunologia , Suplementos Nutricionais/análise , Óleos de Peixe/administração & dosagem , Prebióticos/administração & dosagem , Estresse Fisiológico , Animais , Aquicultura , Bass/genética , Caspase 3/genética , Caspase 3/imunologia , Resistência à Doença , Hidrocortisona/sangue , Interleucina-1beta/genética , Interleucina-1beta/imunologia , Óleos Voláteis/administração & dosagem , Oligossacarídeos/administração & dosagem
2.
Sci Rep ; 12(1): 1358, 2022 01 25.
Artigo em Inglês | MEDLINE | ID: mdl-35079033

RESUMO

Ascaridoids are one of the main parasitic hazards in commercial fish. Candling is the current industrial screening method whereby visible ascaridoid larvae are detected on a light table and manually removed. The aim of this study was to assess the sensitivity (Se) and negative predictive value (NPV) of this method. To make targeted recommendations to the fish industry, the Se was calculated per fish part, larval genus, and fish species. All fish parts (n = 615) were first candled, and larvae were collected, followed by enzymatic digestion to recover the remaining larvae. A fish part was considered positive if at least one larva was detected using candling and/or enzymatic digestion, with both methods combined as reference standard. The overall Se of candling was 31% (95% CI 23-41%) and NPV was 87% (95% CI 85-90%). The Se increased with higher numbers of larvae/100 g infected muscle. A low NPV was found for the belly flaps, therefore we either advise the removal or proper freezing of this part. Lastly, the Se and larval recovery was the highest for the darker and larger Pseudoterranova spp. larvae. Due to the low overall efficacy of candling, further assessment of its cost-benefit and impact on consumers' health risk should be conducted.


Assuntos
Anisakis/isolamento & purificação , Doenças dos Peixes/enfermagem , Peixes/parasitologia , Parasitologia de Alimentos/métodos , Alimentos Marinhos/parasitologia , Animais , Doenças dos Peixes/parasitologia
3.
Food Waterborne Parasitol ; 22: e00108, 2021 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-33681486

RESUMO

The presence of Anisakidae at retail level, after the routine screening via candling, was investigated in cod, the most commonly consumed fish species in Belgium. A total of 780 pre-packed belly flap samples destined for one branch of retail shops were collected from a Belgian wholesale company. To recover all larvae, each sample was first candled and thereafter enzymatically digested. Larvae were morphologically identified to the genus level and a subset was additionally molecularly confirmed by amplification of the ITS fragment and HinfI/HhaI enzyme restriction. The PCR/RFLP profiles of Contracaecum spp. were determined and confirmed with sequencing by the European Reference Laboratory for Parasites (Istituto Superiore di Sanità). The positivity rate of Anisakidae in the individual cod samples was 18% [95%-CI: 15-21%], with a mean intensity of one larva [range: 1-6]. Belly flaps were sold packed primarily by two, with a one-in-three chance of buying an infected package. Pseudoterranova spp. infections (single infections) were most frequently detected (positivity rate 9% [95%-CI: 7-11]), closely followed by Anisakis spp. (7% [95%-CI: 6-9]). Co-infections of Pseudoterranova spp. and Anisakis spp. comprised 8% of the infections, with a positivity rate of 1% [95%-CI: 1-3%]. All belly flaps reportedly were candled prior to our sampling, nonetheless our results indicated that an additional candling screening before packaging would identify an extra third of the infections and larvae. In 19 of the 139 infected samples, all larvae were recovered by the additional candling, thereby removing the infection risk for consumers. In conclusion, this study shows that cod belly flaps infected with zoonotic parasites reach the Belgian consumer. Although a second candling step at retail level could be helpful in reducing the consumer risk, additional measures are needed since 66% of infections would still remain undetected.

4.
Int J Food Microbiol ; 318: 108456, 2020 Apr 02.
Artigo em Inglês | MEDLINE | ID: mdl-31821936

RESUMO

Anisakidae are marine zoonotic nematodes with most commercial fish species as intermediate hosts. Both public health risks and socio-economic problems are attributed to these larvae. Despite these concerns, the occurrence of Anisakidae in commercial fish species in Belgium remains unknown. Therefore, the main objective of this systematic review was to look into studies assessing the prevalence and intensity (level of infection) of Anisakidae in countries importing fish to the Belgian market. The databases of PubMed, Web of Science, Cordis, Google Scholar, Google, African Journals online and Asia Journals online were searched. Main eligibility criteria were: fish species consumed in Belgium; studies conducted in one of the main importing countries; and the availability of prevalence data. From the original 519 identified studies, 83 were included with data from Spain, Germany, Chile, Denmark, Turkey, France, China, England, Belgium, Norway, Iceland, Senegal and Sweden. Overall results show a widespread occurrence of Anisakidae with a high variability in prevalence between fish species and fishing sea. Cod (Gadus morhua) and Atlantic salmon (Salmo salar), the most consumed fish species in Belgium, have a mean prevalence of 33% and 5% respectively. Of all investigated fishing zones, fish caught in the Northeast Atlantic has the highest rate of infection (68%). Furthermore, higher prevalences were found when looking at the viscera (mean prevalence 59%) compared to the muscle (29%) and with superior techniques such as enzymatic digestion or UV press (46%) compared to candling, the routine method (23%). Farmed fish were found to be the least infected (2%) but were still not Anisakidae free. The widespread presence of Anisakidae and the associated food safety implications indicate the need to further investigate the presence of Anisakidae in fish in the Belgian market.


Assuntos
Ascaridoidea/isolamento & purificação , Peixes/parasitologia , Alimentos Marinhos/parasitologia , Animais , Anisakis/isolamento & purificação , Bélgica , Peixes/classificação , Inocuidade dos Alimentos , Larva/crescimento & desenvolvimento , Prevalência , Alimentos Marinhos/classificação
5.
Int J Food Microbiol ; 327: 108657, 2020 Aug 16.
Artigo em Inglês | MEDLINE | ID: mdl-32422589

RESUMO

Anisakidae and Raphidascaridae are marine nematodes present in a wide range of fish hosts, which may cause gastro-intestinal complaints and/or allergy in human, in addition to economic losses for the industry. Data regarding the presence of these parasites in fish for the Belgian market is currently missing; therefore, our aim was to investigate the presence and intensity of ascaridoids in a wide range of commercially fish species. A total of 415 fish samples, belonging to 36 different fish species, were collected from a Belgian whole-sale company. Ascaridoid larvae from the viscera (if present) and the muscles were collected by enzymatic digestion and the prevalence, median intensity, mean number of larvae per 100 g infected muscle, and localisation were determined. An overall prevalence of 53% [95%-CI: 42-63%] in the viscera and 27% [95%-CI: 23-32%] in the muscles was observed. Infection in the muscles varied between the fish species; no larvae were detected in 13 fish species, while a high prevalence (>78%) was observed in pollack, halibut, and gurnard. Most samples originated from the Northeast Atlantic Ocean, with the highest prevalence in the muscles observed in the Barents & Norwegian Sea (65% [95%-CI: 38-86]). Muscle samples were, if possible, divided in an anterior region, belly flap, medial region, and posterior region, with the most infections and larvae found in the belly flaps. In all samples, a total of 2569 larvae were recovered, with 1594 larvae originating from the viscera and 975 from the muscles; with an average of two larvae per 100 g infected fillet detected. Larvae were morphologically identified, and a subgroup was further confirmed using PCR/RFLP, resulting ultimately in the identification of Anisakis simplex s.s. (1853 larvae), A. pegreffii (137), A. simplex/pegreffii hybrid genotype (38), Pseudoterranova decipiens (160) and Hysterothylacium aduncum (380). This study demonstrates that ascaridoid larvae are highly prevalent in different fish species on the Belgian market.


Assuntos
Infecções por Ascaridida/veterinária , Ascaridoidea , Doenças dos Peixes/epidemiologia , Doenças dos Peixes/parasitologia , Parasitologia de Alimentos , Animais , Anisaquíase/parasitologia , Anisakis/genética , Anisakis/isolamento & purificação , Infecções por Ascaridida/epidemiologia , Infecções por Ascaridida/parasitologia , Ascaridoidea/genética , Ascaridoidea/isolamento & purificação , Oceano Atlântico/epidemiologia , Bélgica , Peixes/parasitologia , Parasitologia de Alimentos/estatística & dados numéricos , Humanos , Larva/genética , Músculos/parasitologia , Reação em Cadeia da Polimerase , Polimorfismo de Fragmento de Restrição , Alimentos Marinhos/parasitologia
6.
Eur J Hum Genet ; 7(8): 920-7, 1999 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-10602368

RESUMO

Hereditary neuralgic amyotrophy (HNA) is an autosomal dominant, recurrent focal neuropathy. HNA is characterised by episodes of painful brachial plexus neuropathy with muscle weakness and atrophy, as well as sensory disturbances. Single episodes are commonly preceded by non-specific infections, immunisations or parturition. Mild dysmorphic features and short stature are present in some HNA families, but absolute co-segregation with HNA has not been described. To refine the previously described HNA locus on chromosome 17q25, we performed a genetic linkage study in five HNA families with different geographic origins. Significant linkage was obtained with chromosome 17q24-q25 short tandem repeat (STR) markers in three HNA families and suggestive linkage was found in the other two HNA families. Analysis of the informative recombinations in affected individuals allowed us to reduce the HNA linkage interval to a candidate region of 3.5 cM.


Assuntos
Neurite do Plexo Braquial/genética , Cromossomos Humanos Par 17 , Bandeamento Cromossômico , Feminino , Ligação Genética , Marcadores Genéticos , Humanos , Escore Lod , Masculino , Linhagem , Penetrância
7.
Neuromuscul Disord ; 11(4): 400-3, 2001 May.
Artigo em Inglês | MEDLINE | ID: mdl-11369192

RESUMO

Hereditary neuropathy with liability to pressure palsies (HNPP) is an autosomal dominant, demyelinating peripheral neuropathy. Clinical hallmarks are recurrent painless focal neuropathies mostly preceded by minor trauma or compression at entrapment sites of peripheral nerves. In the majority of the patients, HNPP is caused by a 1.5 Mb deletion on chromosome 17p11.2-p12 containing the peripheral myelin protein 22 (PMP22) gene. Point mutations within this gene are reported in only a few families. We report a novel mutation in the PMP22 gene in a Spanish family with HNPP. The mutation is a 3' splice-site mutation, preceding coding exon 3 (c.179-1 G>C), causing a mild HNPP phenotype.


Assuntos
DNA Recombinante , Neuropatia Hereditária Motora e Sensorial/genética , Mutação/genética , Proteínas da Mielina/genética , Paralisia/genética , Adulto , Sequência de Bases/genética , Criança , Eletrofisiologia , Feminino , Neuropatia Hereditária Motora e Sensorial/patologia , Neuropatia Hereditária Motora e Sensorial/fisiopatologia , Humanos , Masculino , Linhagem , Pressão , Nervo Sural/patologia
8.
Rev Esp Cardiol ; 51 Suppl 3: 93-8, 1998.
Artigo em Espanhol | MEDLINE | ID: mdl-9717410

RESUMO

Patients with severe angina pectoris, refractory to medical treatment, in which conventional revascularization (PTCA or bypass surgery) is not possible because they present advanced coronary artery disease with a poor distal bed, account for an important clinical problem due to an increasing incidence, combined with poor quality of life, an elevated risk of severe complications, repeated hospital admissions and high mortality rate. Laser transmyocardial revascularization provides a new therapeutic alternative for these patients. Although up to now there are only a few published series, with a small number of patients, the results obtained in the two ongoing multicentric studies in Europe and the United States (including more than 500 patients at present) are quite promising. It is a simple surgical procedure, but its associated mortality is not to be dismissed (in the beginning 12% and currently 5%), because patients are in an advanced evolutionary stage. In Spain this procedure has been available since April 1996 and the results have been encouraging. In our small series we have noted a significant symptomatic improvement and better quality of life.


Assuntos
Terapia a Laser/métodos , Isquemia Miocárdica/cirurgia , Revascularização Miocárdica/métodos , Idoso , Angina Pectoris/cirurgia , Diástole , Feminino , Seguimentos , Humanos , Terapia a Laser/efeitos adversos , Masculino , Pessoa de Meia-Idade , Revascularização Miocárdica/efeitos adversos
9.
Med Clin (Barc) ; 93(4): 121-4, 1989 Jun 24.
Artigo em Espanhol | MEDLINE | ID: mdl-2552235

RESUMO

The authors report two male patients with inclusion body myositis (IBM) and review the features of this condition in the literature. This is an uncommon type of idiopathic myositis which involves males more often than females; it usually develops in elderly patients; its course is very slow; it is usually associated with distal weakness; neurophysiological studies show a mixed "myogenic" and "neurogenic" pattern; and it is usually unresponsive to corticosteroids. The diagnosis is basically made on the basis of the histological features, mainly consisting of vacuoles surrounded by a basophilic haze in histochemical stains of frozen tissue and, particularly, by the presence of characteristic microfilaments in ultrastructural studies.


Assuntos
Corpos de Inclusão/patologia , Miosite/patologia , Humanos , Masculino , Pessoa de Meia-Idade , Miosite/diagnóstico , Miosite/genética , Linhagem
10.
Rev Neurol (Paris) ; 147(1): 17-27, 1991.
Artigo em Francês | MEDLINE | ID: mdl-1901663

RESUMO

The National Institute of Health Consensus Panel on Neurofibromatosis (NF) recently recognized 2 distinct forms of NF (NF-1 and NF-2) and stated that variant forms may exist. We selected 30 patients who fulfilled the criteria of NF-1 or whose condition was consistent with NF-2. All patients showed pathological magnetic resonance images (MRI), and in 19 cases confirmation was obtained from histopathology. We established correlations between the site and nature of the lesions on the one hand and the diagnostic criteria of NF on the other hand, there by hoping to contribute to a better knowledge and classification of neurofibromatosis. Nineteen patients had only intraparenchymatous lesions of the central nervous system (CNS) and fulfilled the criteria of NF-1; histopathological examination demonstrated pilocytic astrocytoma in 8 cases. Eleven patients showed only extra-axial lesions; 8 of them had criteria suggestive of NF-2, except for familial history. Pathological examination revealed either acoustic, pluriradicular, peripheral or mixed schwannomas (7/8) or pluriradicular ganglioneuromas (1/8). Two patients had unilateral extra-axial pluriradicular cervical lesions and fulfilled the diagnostic criteria of NF-1; pathological examination revealed neurofibroma in both cases. One female patient had both intra- and extra-axial lesions that fulfilled the criteria of NF-1 and NF-2, suggesting the existence of a mixed form (NF-3).


Assuntos
Doenças do Sistema Nervoso Central/complicações , Neurofibromatose 1/etiologia , Neuroma Acústico/etiologia , Adolescente , Adulto , Doenças do Sistema Nervoso Central/patologia , Criança , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neurofibromatose 1/classificação , Neurofibromatose 1/patologia , Neuroma Acústico/classificação , Neuroma Acústico/patologia
11.
Rev Neurol (Paris) ; 150(1): 22-32, 1994.
Artigo em Francês | MEDLINE | ID: mdl-7801036

RESUMO

Acute spinal cord infarction (ASCI) occurs infrequently and may have diverse causes. The diagnosis of ASCI, and particularly of an anterior spinal artery syndrome (ASAS) can be confirmed nowadays by MRI, whereas in the past only necropsy confirmation was possible. Pathophysiology and long-term prognosis may be better known at present and treatments more consistent. We present the longitudinal study and clinical features of 8 patients suffering from ASCI. All of them were personally studied and had MRI examinations, often with sequential studies. three groups must be considered: one included 4 cases of ASAS at cervical level, the second 2 cases of ASAS at thoracic level and the third group with infarction of the conus medullaris (ICM), one of them developed during surgical repair of an infrarenal aortic aneurysm. Motor and sensory sequelae were assessed in each case together with possible etiological factors. In conclusion, recovery after ASAS tends to be dependent on the severity of the initial deficit. At cervical level, clinical and morphological findings argue in favour of an extrinsic selective compression of the C7 right radiculo-medullary artery as responsible for the ASA. At thoracic level, the artery preferentially occluded seems to be the sulco-commisural artery as a consequence of disc compression. Finally, an underlying peculiarity of the pattern of arterial supply is a probable predisposing factor for ICM. Generally, the long-term prognosis of ASCI is not necessarily bad.


Assuntos
Infarto/diagnóstico , Isquemia/diagnóstico , Imageamento por Ressonância Magnética , Doenças da Medula Espinal/etiologia , Medula Espinal/irrigação sanguínea , Adulto , Idoso , Artérias , Feminino , Seguimentos , Humanos , Infarto/complicações , Isquemia/complicações , Estudos Longitudinais , Masculino , Pessoa de Meia-Idade , Paraplegia/etiologia , Paresia/etiologia , Prognóstico , Doenças da Medula Espinal/diagnóstico
12.
Rev Neurol (Paris) ; 144(3): 181-93, 1988.
Artigo em Francês | MEDLINE | ID: mdl-3368693

RESUMO

Twenty-two patients with syringomyelia were investigated clinically and by MR imaging using clearly defined clinical and radiologic protocols. Intramedullary cavities were identified by MR images in 20 cases and by CT scanning after intrathecal injection of Metrizamide in two cases. In all but one case, when the clinical picture was purely motor, concordance was established between clinical findings and morphologic type of intramedullary cavity. Syringomyelic syndromes should now be defined not only on the basis of clinical data but on clinicoradiologic features. The examination of choice is MR imaging, because of its reliability and safety and because it allows tridimensional analysis of cavities and associated malformations. Results of MR imaging exploration suggest a possible improved physiopathologic approach to the disease and more effective therapeutic procedures.


Assuntos
Imageamento por Ressonância Magnética , Siringomielia/diagnóstico , Adulto , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Dor/etiologia , Siringomielia/diagnóstico por imagem , Siringomielia/patologia , Tomografia Computadorizada por Raios X
13.
Rev Neurol (Paris) ; 149(8-9): 481-4, 1993.
Artigo em Francês | MEDLINE | ID: mdl-8009146

RESUMO

Several forms of peripheral neuropathy occur in Sjögren's syndrome (dryness of eyes, mouth and other mucous membranes). Symmetrical sensorimotor polyneuropathy occurs most frequently followed by sensory neuropathy. Pure sensory neuronopathy, trigeminal sensory neuropathy and autonomic neuropathy are also common. We report three rare forms of peripheral neuropathy related to Sjögren's syndrome and analyze their clinical course and response to therapy. There were a case of mononeuropathy multiplex (MM), a case of recurrent cranial polyneuropathy (RCP) and a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). The patients were females, 70, 63 and 81 years old respectively. All of them fulfilled the criteria of Sjögren's syndrome, without clinical or serological abnormalities of systemic disease. Only one patient presented with positive antinuclear antibodies. In two patients the onset of neuropathy occurred before the sicca syndrome long. The sural biopsy of the patient with MM exhibited vasculitis of the small vessels and a mild sensory polyneuropathy was superimposed. The patient with RCP had 8 episodes of isolated third nerve palsy and 5 episodes of multiple cranial neuropathy. The patient with CIDP responded poorly to treatment while in the patients with MM and CRP the response to corticotherapy was good. In conclusion, Sjögren's syndrome must be considered in neuropathies of unknown cause not only when they are sensory, autonomic or trigeminal but also when they are recurrent suggesting an ischemic mechanism. Even a CIDP requires a search for Sjögren's syndrome before being considered idiopathic.


Assuntos
Doenças Desmielinizantes/etiologia , Doenças do Sistema Nervoso Periférico/etiologia , Síndrome de Sjogren/complicações , Idoso , Doenças dos Nervos Cranianos/etiologia , Doenças Desmielinizantes/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/patologia , Vasculite/complicações , Vasculite/patologia
14.
Rev Neurol (Paris) ; 144(6-7): 456-8, 1988.
Artigo em Francês | MEDLINE | ID: mdl-3187301

RESUMO

A patient presented with what was clinically diagnosed as a solitary medullary haemangioblastoma. One year later she was operated from a cerebellar haemangioblastoma. The neuro-radiologic findings showed all the characteristics of von Hippel-Lindau's disease, except the retinal haemangioblastoma. The patient died five years later as a result of deglutition problems with aspiration pneumonia. Post-mortem examination showed that the tumor level was in the posterior right side of the dorsal spinal cord. A syringomyelic cavity spread above the tumor up to the medulla - without communicating with the fourth ventricle and beneath the tumor down to the lowest part of the thoracic spinal cord. A large venous pia mater stasis and a thick glial wall of the cavity - mostly below the tumor - suggested a lack of reabsorption of liquids produced by the tumor.


Assuntos
Angiomatose/complicações , Hemangiossarcoma/complicações , Neoplasias da Medula Espinal/complicações , Siringomielia/complicações , Doença de von Hippel-Lindau/complicações , Neoplasias Cerebelares/complicações , Neoplasias Cerebelares/patologia , Feminino , Hemangiossarcoma/patologia , Humanos , Bulbo , Pessoa de Meia-Idade , Neoplasias da Medula Espinal/patologia , Siringomielia/patologia , Doença de von Hippel-Lindau/patologia
15.
Rev Neurol (Paris) ; 156(12): 1126-35, 2000 Dec.
Artigo em Francês | MEDLINE | ID: mdl-11139729

RESUMO

Twenty patients with multiple sclerosis (MS), 19 women and 1 man, with acute proprioceptive sensory disturbances related to the presence of plaques on the posterior columns (posterior column syndrome) at the cervical or thoracic levels of the spinal cord, were selected among 138 new patients with MS assisted in our neurological unit over the past five years. In 17 of these patients, the acute posterior cordonal syndrome was responsible for the first clinical manifestations of the disease. The other 3 patients had a history suggestive of MS. These 20 patients were followed with a minute analysis of neurological function with repeated clinical evaluation combined with repeated MRI study of the spinal cord. Brain MRI (strongly suggestive of MS in 15 patients), evoked potentials (EP) and cerebrospinal fluid electrophoresis analysis (with oligoclonal bands present in all patients were it was performed) were also obtained at least once in each patient. Spinal cord MRI demonstrated more lesions in the cervical region (90 p.100) than in the thoracic regions (10 p.100). Eighty percent of the cervical lesions were located high, between C1 and C4. The most characteristic clinical expression was the deafferentation of one upper limb, preferentially the "useless hand" (Oppenheim) or even a pseudoathetosic or dystonic limb. Propioceptive ataxia or spontaneous cervical or brachial pain were other forms of clinical expression. No major motor deficit or sphincter disorders were noted at any time in the clinical course in any of the patients. There was a good correlation between localization and morphology of the plaques detected by spinal cord MRI and clinical signs. Intrinsic medullary lesions were seen as high intensity signals on T2-weighted images which were enlarged more than the same lesion visualized on T1-weighted images after injection of paramagnetic contrast agents. This reflected the presence of edema extending beyond the main inflammatory lesion. There was also a good correlation between improvement of clinical symptoms and total or, mor frequently, partial reduction of the plaques, analyzed morphologically by successive spinal cord MRI series. The diagnosis of MS was clinically definitive in 60 p.100 of cases and laboratory-supported definitive in 40 p.100. During the follow-up period (average 36 months), 15 patients (75 p.100) presented one or more exacerbations, all of them presenting a favorable course: at last follow-up, 9 patients were asymptomatic, EDSS was 1 in 6 patients, 1.5 in 4 patients and 2 in 1 patient. This study confirms the contribution of serial spinal cord MR studies to understanding the natural history and pathophysiology of medullary forms of MS presenting as a cordonal posterior syndrome. It also shows a good relationship between the clinical manifestations and course of this form of MS and the localization and variable morphology of plaques. Finally, our results suggest the predictive benign course for this medullary form of MS that seems to be almost exclusively restricted to the female gender.


Assuntos
Esclerose Múltipla/patologia , Esclerose Múltipla/fisiopatologia , Medula Espinal/patologia , Adulto , Potenciais Evocados , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/líquido cefalorraquidiano , Propriocepção , Estudos Retrospectivos
16.
Rev Neurol (Paris) ; 152(12): 744-7, 1996 Dec.
Artigo em Francês | MEDLINE | ID: mdl-9205698

RESUMO

Bilateral palsy of the common ocular motornerve (III) was observed in two patients with an intra-axial lesion due ti hemorrhage in one and ischemia in the other. The lesions involved the cerebral peduncle in the periaqueductal region and the nuclear complex of the III in the first case. Bilateral infarct of the thalamus was seen in the second. Clinical manifestations were transitory except for the oculomotor impairment. In the first patient, oculomotricity was dissociated as intrinsic mortricity was spared. These exceptional cases demonstrate a syndrome with unique oculomotor expression resulting from intra-axial oculomotor lesions. Prognosis varies and is related to the ischemic or hemorrhagic nature of the causal lesion and its localization.


Assuntos
Isquemia Encefálica/complicações , Hemorragia Cerebral/complicações , Doenças do Nervo Oculomotor/etiologia , Idoso , Idoso de 80 Anos ou mais , Isquemia Encefálica/diagnóstico , Hemorragia Cerebral/diagnóstico , Humanos , Imageamento por Ressonância Magnética , Masculino , Mesencéfalo/irrigação sanguínea
17.
Rev Neurol (Paris) ; 147(6-7): 497-500, 1991.
Artigo em Francês | MEDLINE | ID: mdl-1962056

RESUMO

Two 30-year old twins, one male, the other female, were followed up for 20 years for predominantly proximal muscular deficit without increase of muscle enzymes. The lactic acid level was elevated at rest and further increased during exercise. Muscle biopsy revealed mitochondrial abnormalities. Encephalopathy was also present. The female patient had been treated, at the age of 10 years, for myoclonic attacks which regressed when she was over 18 years. None of the two patients had dementia. CT and MRI showed very extensive and symmetrical lesions of the white matter which did not involve the basal ganglia. These two cases are interesting on three scores: (1) clinically, the woman exhibited symptoms of the MELAS syndrome (without cerebral vascular accidents) and symptoms of the MERRF syndrome, which suggests the existence of borderline cases; (2) genetically, our cases were in favour of a so-called "maternal" heredity (boys are affected in all cases): here both sexes were involved but the phenotype varied; (3) biochemically, we found no enzyme activity deficit likely to explain the clinical features. The significance of a selective increase of cytochrome c oxidase in both mother and daughter is unclear.


Assuntos
Encefalopatias/patologia , Doenças em Gêmeos , Doenças Musculares/patologia , Adulto , Encefalopatias/diagnóstico por imagem , Encefalopatias/genética , Epilepsias Mioclônicas/genética , Epilepsias Mioclônicas/patologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Doenças Musculares/genética , Tomografia Computadorizada por Raios X , Gêmeos Dizigóticos
18.
Rev Neurol (Paris) ; 135(8-9): 633-8, 1979 Oct.
Artigo em Francês | MEDLINE | ID: mdl-161057

RESUMO

The authors report 2 cases of aneurysmal cysts of the spinal cord with neurological manifestations. The first case was a young girl of 16 years of age with a lesion at the D6 level and spasmodic paraparesis. The second case was a boy aged 5 years with a lesions at the L3 level and lumbar pains. The characteristic "one-eyed vertebra" appearance was present in the radiological images in both cases, and was confirmed histologically on operation, which produced good results.


Assuntos
Cistos Ósseos/complicações , Doenças do Sistema Nervoso/etiologia , Neoplasias da Coluna Vertebral/complicações , Adolescente , Dor nas Costas/etiologia , Cistos Ósseos/diagnóstico por imagem , Cistos Ósseos/cirurgia , Criança , Feminino , Humanos , Vértebras Lombares , Masculino , Paraplegia/etiologia , Radiografia , Compressão da Medula Espinal/etiologia , Neoplasias da Coluna Vertebral/diagnóstico por imagem , Neoplasias da Coluna Vertebral/cirurgia , Vértebras Torácicas
19.
Rev Neurol (Paris) ; 134(4): 295-302, 1978 Apr.
Artigo em Francês | MEDLINE | ID: mdl-725401

RESUMO

We report a case of a boy of 13 who was born with muscular hypotony which has tended to improve over the course of the years. We was examined for the first time when he was 4 years old and a diagnosis was made of "congenital myopathy". Examination showed curved feet, gothie palate, severe laxity of the ligaments, and spider fingers which evoked Marfan's syndrome. The present myopathy is concentrated in the shoulder girdle and the E.M.G. showed "myogenic" tracings. Biopsy of the deltoid showed the presence of large numbers of rods in all muscle fibers stained with Engel's modified trichromic stain, localized mainly under the sarcolemma. Histochemical staining showed no evidence of differences in the fibers, all of which had the same enzymatic activity. A diagnosis was made of nemaline myopathy. As we were able to follow clinical progress in this case for nine consecutive years we could confirm that not only was the disease non-progressive but that it was regressing in this patient.


Assuntos
Doenças Musculares/congênito , Braço , Biópsia , Pré-Escolar , Diagnóstico Diferencial , Seguimentos , Humanos , Masculino , Síndrome de Marfan/diagnóstico , Hipotonia Muscular/congênito , Hipotonia Muscular/patologia , Músculos/patologia , Doenças Musculares/patologia , Miofibrilas/patologia , Síndrome
20.
Rev Neurol (Paris) ; 144(12): 820-2, 1988.
Artigo em Francês | MEDLINE | ID: mdl-3070695

RESUMO

A hemifacial spasm was symptomatic of a neurinoma of the hypoglossal nerve in a 50 year-old woman. It disappeared after the surgical cure of the tumor. Hemifacial spasm is not mentioned among the 19 cases of neurinoma of the hypoglossal nerve which have been reported up to now. The spasm could have been due to the compression of the 7th nerve by the anterior inferior cerebellar artery, displaced by the tumor.


Assuntos
Neoplasias dos Nervos Cranianos/complicações , Músculos Faciais , Nervo Hipoglosso , Neurilemoma/complicações , Espasmo/etiologia , Feminino , Humanos , Pessoa de Meia-Idade
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