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Introduction: Acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) is an increasingly recognized CIDP subtype. Differentiating A-CIDP from Guillain–Barre syndrome (GBS) is challenging but important, because there are different treatment outcomes. Methods: We report 3 patients with A-CIDP who were initially diagnosed with severe GBS but were later confirmed to have CIDP based on their clinical course and electrodiagnostic (EDx) studies. We also report on the long-term treatment of these patients and review the literature on EDx studies in this syndrome. Results: Three patients were initially diagnosed with GBS and responded to treatment. However, all 3 had arrest in improvement or deterioration during their rehabilitation phases. EDx studies showed prominent demyelinating changes many months after the initial presentation. All responded very well to immunotherapy. Conclusion: Although several features may suggest the diagnosis of A-CIDP at initial presentation, close follow-up of GBS patients during the recovery phase is also needed for accurate diagnosis. EDx studies may distinguish patients with A-CIDP from GBS patients. Muscle Nerve 52: 900–905, 2015

길랑 바레 증후군은 주로 말초, 척수, 뇌신경을 침범하여 급성 혹은 아급성 마비를 일으키는 다발성 신경염으로 대칭적인 진행성 운동 마비와 심부 건반사 소실, 뇌척수액 단백질 수치 상승을 특징으로 한다. 길랑 바레 증후군은 다양한 병리 기전을 가진 질병군으로 현재는 면역 병리학적 조사나 전기 생리학적 검사 소견에 따라 급성 염증성 탈수초성 다발성 신경병과...

Resume Int'eret.Il s'agit de faire le point sur les donnees therapeutiques actuelles et, notamment, sur les indications des echanges plasmatiques au cours du syndrome de Guillain-Barre. Donnees actuelles. Le syndrome de Guillain-Barre est la cause la plus frequente des paralysies aigues flasques. La mortalite est actuellement d'environ 5%, et 10% des malades garderont des sequelles motrices tres invalidantes apres 1 an de recul. La prise en charge de ces malades necessite des equipes entrainees, disposant de moyens de reanimation, de reeducation et pouvant pratiquer l'ensemble des traitements specifiques. La corticotherapie per os ou par voie intraveineuse est inefficace. Les echanges plasmatiques (EP) sont le premier traitement dont l'efficacite a ete demontree par rapport a un groupe temoin. Les indications sont maintenant mieux connues. Les formes benignes (marche possible) doivent etre traitees par deux EP, deux EP supplementaires doivent etre faits en cas d'aggravation. Dans les formes intermediaires (marche impossible) et dans les formes severes (recours a la ventilation mecanique) quatre EP doivent etre pratiques. Il n'est pas utile d'augmenter le nombre d'EP dans les formes severes ou en cas d'absence d'amelioration. De fortes doses d'immunoglobulines donnees par voie intraveineuse (0,4 g/kg/j pendant 5 jours) sont aussi efficaces que les EP dans les formes intermediaires et severes. Conclusion. Dans l'etat actuel des connaissances, l'indication des immunoglobulines et/ou des echanges plasmatiques doit tenir compte de leurs contre-indications respectives. II n'est pas utile d'associer ces deux traitements, ce qui en accroit le cout et les risques.

To establish the efficacy of intravenous immunoglobulins (IVIG) in the treatment of acute Guillain-Barré syndrome (GBS), we treated nine consecutive pediatric cases (age 2.5-13.5 years) fulfilling the criteria for GBS with a single dose of IVIG (Sandoglobulin; 2 g/kg/BW). None of the patients experienced any IVIG related side-effects. The mean time required to improve by at least one grade on the functional GBS scale after IVIG treatment was 3.5 days, while the mean period to regain ambulation was 11.2 days. Full mobilization without evidence of relapse in the follow-up period (mean 14.5 months) was noted in all but one patient who relapsed after 5 months. We conclude that the early use of a single IVIG dose may prevent further progression of the disease, thus shortening the clinical course of childhood GBS. The most beneficial IVIG dose regimen remains to be determined by controlled trials.

Four children with the Guillain-Barresyndrome seen during the autumn of 1968 had high complement-fixing antibody titres to Mycoplasma pneumoniœ, and in two this mycoplasma was isolated from the nasopharynx during the neurological illness. These observations indicate that M. pneumoniœ is yet another determinant of the syndrome, and perhaps more common than hitherto recognised.

The effect of corticosteroids on the Guillain-Barresyndrome was studied in thirty-eight episodes of the disease. Twenty-five patients received corticosteroids and thirteen did not. The two groups were similar as regards age, sex, and severity of illness. In mild and moderately severely affected cases the recovery-time and length of stay in hospital was prolonged in the group receiving corticosteroids. In severely affected cases, however, these times were not influenced. It is recommended that corticosteroids should not be prescribed in the Guillain-Barresyndrome.

The case-records of 71 patients from previous therapeutic trials were used to determine features in clinical presentation that might be useful in predicting the outcome of the Guillain-Barre syndrome. The major features adversely affecting prognosis were severe maximum deficit requiring ventilation and failure to improve within 3 weeks of reaching peak deficit. The risk of poor outcome in a patient who required ventilation and failed to improve within 3 weeks was 86%.

A patient with the Landry-Guillain-Barresyndrome required 266 days of complete ventilatory support. The complete respiratory and extremity paralysis then improved remarkably over the next 2 weeks, and the patient could be removed from the ventilator and the tracheostomy was removed. Further neuromuscular recovery followed. This case represents the longest period of paralysis, with ultimate recovery, on record.

Sixty patients with suspected lumbar herniated disk and/or canal stenosis were studied prospectively with surface coil MRI, CT, and/or myelography, and the results were compared with the surgically confirmed abnormality. Forty-eight patients had lumbar surgery at 62 levels. There were no negative explorations. Thirty-nine patients had a myelogram and CT. Thirty of the CTs were performed following the injection of metrizamide for myelography. Nine patients had a CT without intrathecal contrast material 1 to several days before the myelogram. Six patients had myelography only, and three patients had CT only. All studies were evaluated for the location and type of disease in a forced choice fashion. Independent of the surgically correlated levels, there was 86.8% agreement between the MR and CT studies in all patients at 151 levels and 87.2% agreement between MR and myelography at 218 levels. At the operative levels, there was 82.6% agreement between MR and surgical findings for both type and location of disease; 83% agreement between CT and surgical findings; and 71.8% agreement between myelography and surgical findings. There was 92.5% agreement when MR and CT were used jointly, and 89.4% agreement when CT and myelography were used jointly. The results of this study indicate that a technically adequate MR examination was equivalent to CT and myelography in the diagnosis of lumbar canal stenosis and herniated disk disease. CT and MR can be complementary studies, and surface coil MR can be viewed as an alternative to myelography.