RESUMEN
BACKGROUND: Diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP) have variable sensitivity and specificity. Newly published criteria by Koski et al combine clinical and electrophysiological components, either of which suffices to establish the diagnosis. European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) criteria require mandatory electrophysiology, as do other sets of criteria. METHODS: The value of the two above-mentioned sets of criteria, on 151 patients with CIDP, and 162 controls with axonal neuropathy, from four European centres was assessed. Results were compared with Van den Bergh and Piéret's criteria and those of the American Academy of Neurology (AAN). The utility of more extensive nerve-conduction studies was ascertained. RESULTS: Koski et al's criteria had a sensitivity of 63% and specificity of 99.3%. With unilateral, right-sided, forearm/foreleg, four-nerve studies, EFNS/PNS criteria offered a sensitivity of 81.3% and specificity of 96.2% for "definite/probable" CIDP. Van den Bergh and Piéret's criteria had a sensitivity of 79.5% and specificity of 96.9%. AAN criteria were poorly sensitive (45.7%) but highly specific (100%). "Possible" electrophysiological CIDP as per EFNS/PNS criteria were poorly specific (69.2%). More extensive studies increased the diagnostic sensitivity of EFNS/PNS criteria (96.7%) but reduced the specificity (79.3%). CONCLUSIONS: In our patient populations, the EFNS/PNS criteria were the most sensitive and allowed identification of a highly significantly greater number of patients than Koski et al's criteria. The latter were comparable in specificity with the "definite/probable" EFNS/PNS electrodiagnostic subcategories. More extensive nerve-conduction studies improved diagnostic yield but resulted in loss of specificity.
Asunto(s)
Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Bélgica , Errores Diagnósticos , Electrofisiología , Inglaterra , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Conducción Nerviosa/fisiología , Nervios Periféricos/fisiopatología , Guías de Práctica Clínica como Asunto/normas , Reproducibilidad de los Resultados , Estudios Retrospectivos , Sensibilidad y EspecificidadRESUMEN
INTRODUCTION: Paraneoplastic choreo-athetoses are rare. We report a case of anti-Hu syndrome with choreo-athetosis. CASE REPORT: A 48-year-old woman developed a small-cell lung carcinoma revealed by an anti-Hu syndrome. The neurological features included choreo-athetosis predominating in the upper limbs, chronic sensorimotor axonal polyneuropathy, and opsoclonus. The cerebrospinal fluid was acellular and contained several oligoclonal IgG bands, not found in the corresponding serum. Magnetic resonance imaging revealed bilateral high-intensity lesions on T2/FLAIR sequence in the corona radiata. Moderate transitory improvement of the paraneoplastic neurological syndrome was observed after several carboplatin-etoposid cycles. CONCLUSION: A paraneoplastic origin must be considered in all cases of unexplained choreo-athetosis. Paraneoplastic choreo-athetosis is most often associated with other neurological symptoms. The most frequent associated tumor is a small-cell lung carcinoma with anti-CRMP5 and/or anti-Hu antibodies. Our patient developed paraneoplastic choreo-athetosis related to an anti-Hu syndrome in the absence of anti-CRMP5/CV2 antibodies. Paraneoplastic choreo-athetosis might result from a central lesion, and/or from proprioceptive deafferentation subsequent to peripheral neuropathy.
Asunto(s)
Atetosis/etiología , Carcinoma de Células Pequeñas/patología , Corea/etiología , Neoplasias Pulmonares/patología , Enfermedades del Sistema Nervioso/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Atetosis/diagnóstico , Carcinoma de Células Pequeñas/diagnóstico , Carcinoma de Células Pequeñas/tratamiento farmacológico , Corea/diagnóstico , Femenino , Humanos , Neoplasias Pulmonares/diagnóstico , Neoplasias Pulmonares/tratamiento farmacológico , Imagen por Resonancia Magnética , Persona de Mediana EdadRESUMEN
We report on a 43-year-old woman who developed a fulminant thrombotic thrombocytopenic purpura (TTP) in the early course of an oral ciprofloxacin therapy. An acute bacterial meningo-encephalitis with disseminated intravascular coagulation was first suspected. She also presented with haemolytic anaemia and a severe thrombocytopenia. The lumbar puncture was delayed and only performed after platelets transfusions. CSF examination ruled out meningo-encephalitis. A diagnosis of TTP was made and she was given plasma exchanges. However, her neurological status worsened and the cranial CT revealed a subarachnoidal hemorrhage in left sylvian valley with diffuse oedema predominating in the left cerebral hemisphere and multiple ischemic lacunes. She died four days after admission.
Asunto(s)
Antiinfecciosos/efectos adversos , Encéfalo/diagnóstico por imagen , Ciprofloxacina/efectos adversos , Púrpura Trombocitopénica Trombótica/inducido químicamente , Púrpura Trombocitopénica Trombótica/diagnóstico por imagen , Infecciones Urinarias/tratamiento farmacológico , Adulto , Resultado Fatal , Femenino , Humanos , Tomografía Computarizada por Rayos XRESUMEN
The inflammatory demyelinating neuropathies constitute a significant proportion of the acquired polyneuropathies. Major progress in finding the causes and in the treatment of these neuropathies has been made over the last decade. Early recognition is of paramount importance, because timely and appropriate treatment can largely reduce morbidity and disability. Electrodiagnosis plays a key role in the detection and characterization of the inflammatory demyelinating neuropathies. Electrodiagnostic criteria for primary demyelination have therefore been developed. They are empirically based on changes of nerve conduction parameters in populations of patients with a confirmed clinical and laboratory diagnosis of inflammatory demyelinating neuropathy. The challenge consists of defining criteria sets that are highly specific but also as sensitive as possible. Most of the hereditary demyelinating neuropathies are part of Charcot-Marie-Tooth disease type 1. The pattern of nerve conduction abnormalities usually provides valuable clues for the distinction from chronic inflammatory demyelinating neuropathies.
Asunto(s)
Potenciales de Acción/fisiología , Enfermedad de Charcot-Marie-Tooth/diagnóstico , Electrodiagnóstico , Conducción Nerviosa/fisiología , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Animales , Enfermedad de Charcot-Marie-Tooth/genética , Enfermedades Desmielinizantes , Electrodiagnóstico/métodos , Humanos , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/genéticaAsunto(s)
Hemangioma Cavernoso/diagnóstico , Hemangioma Cavernoso/inmunología , Inmunoglobulinas/líquido cefalorraquídeo , Neoplasias de la Médula Espinal/diagnóstico , Neoplasias de la Médula Espinal/inmunología , Femenino , Hemangioma Cavernoso/líquido cefalorraquídeo , Humanos , Imagen por Resonancia Magnética , Persona de Mediana Edad , Bandas Oligoclonales , Neoplasias de la Médula Espinal/líquido cefalorraquídeoRESUMEN
A 44-year-old maniacodepressive woman developed acute encephalopathy due to the association of lithium carbonate and haloperidol. She was treated with lithium salts for many years and the serum level of lithium was within the therapeutic range. The encephalopathy was worsened by hyperthermia, dehydration, and reintroduction of haloperidol, 5 days after the first discontinuation of the neuroleptic. The clinical features were characterized by a persistent cerebellar syndrome, more than one year after the interruption of these medications.
Asunto(s)
Antidepresivos/envenenamiento , Antipsicóticos/envenenamiento , Encefalopatías/inducido químicamente , Enfermedades Cerebelosas/inducido químicamente , Haloperidol/envenenamiento , Carbonato de Litio/envenenamiento , Enfermedades Musculares/inducido químicamente , Enfermedad Aguda , Adulto , Encefalopatías/fisiopatología , Enfermedades Cerebelosas/fisiopatología , Femenino , Humanos , Enfermedades Musculares/fisiopatología , SíndromeRESUMEN
The authors describe the clinical and biological data of seven patients with anti-Hu antibodies. Six of them displayed a small cell lung carcinoma (SCLC), but no cancer was detected in the 7th patient in spite of an extensive workup. The clinical heterogeneity of the anti-Hu syndrome is emphasized. The major symptoms were linked to a severe sensory neuropathy in three cases, to cerebellitis in two cases, to dysautonomia in one case, and to gastro-intestinal pseudo-obstruction in one case. One patient also displayed EMG abnormalities characteristic of the Lambert-Eaton myasthenic syndrome. Two patients developed opsoclonus or ocular flutter associated with severe confusion in the late stage of their disease. In four patients, the neurological signs and symptoms preceded the discovery of the SCLC, and in two cases the initial detection of anti-Hu antibodies prompted the successful search for this tumor. Immunopathological events injuring the peripheral and central nervous system are briefly discussed.