Differences between acute-onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients.

Acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed. Demographic characteristics, clinical manifestations, cerebrospinal-fluid (CSF) findings, treatment and clinical evolution were analyzed. Nerve conduction studies were performed in all patients with at least 12 months follow-up. A total of 91 patients were included (AIDP, n = 77; A-CIDP, n = 14). The median age was 55.5 years in patients with A-CIDP vs 43 years in AIDP (P = .07). The history of diabetes mellitus was more frequent in A-CIDP (29% vs 8%, P = .04). No significant differences between groups were observed with respect to: human immunodeficiency virus (HIV) status, presence of auto-immune disorder or oncologic disease. Cranial, motor and autonomic nerve involvement rates were similar in both groups. Patients in the A-CIDP group showed higher frequency of proprioceptive disturbances (83% vs 28%; P < .001), sensory ataxia (46% vs 16%; P = .01), and the use of combined immunotherapy with corticoids (29% vs 3%; P = .005). There were no significant differences in CSF findings, intensive care unit (ICU) admission, or mortality rates. During the first 8 weeks both entities are practically indistinguishable. Alterations in proprioception could suggest A-CIDP. Searching for markers that allow early differentiation could favor the onset of corticotherapy without delay.

Chronic inflammatory demyelinating polyneuropathy: quality of life, sociodemographic profile and physical complaints.

UNLABELLED: Whereas an evaluation of quality of life and possible impacts on the mental state of a patient may help to evaluate the evolution of chronic inflammatory demyelinating polyneuropathy (CIDP), the aim of this study was to study the psychological profile of patients, and evaluate quality of life associated with the disease. METHOD: 41 patients were evaluated using a Mini-Mental State Examination (MMSE) and a Short-Form Health Survey (SF-36). RESULTS: The mean age of the patients was 50.6 years, 63.4% men. Of the participants, 65.9% had other health problems, 39% reported needing help with activities of daily living, 49% slept less than 8 hours per night, and 34.1% complained of some memory deficit. The average MMSE score was 26. Impairment of functional capacity and pain were the more important altered health states. CONCLUSION: CIDP has important social and economic impacts, owing to functional impairments that can lead to professional and personal limitations.

Chronic inflammatory demyelinating polyneuropathy: quality of life, sociodemographic profile and physical complaints / Polineuropatia inflamatoria desmielinizante cronica: qualidade de vida, perfil sociodemografico e queixas fisicas

Whereas an evaluation of quality of life and possible impacts on the mental state of a patient may help to evaluate the evolution of chronic inflammatory demyelinating polyneuropathy (CIDP), the aim of this study was to study the psychological profile of patients, and evaluate quality of life associated with the disease. Method 41 patients were evaluated using a Mini-Mental State Examination (MMSE) and a Short-Form Health Survey (SF-36). Results The mean age of the patients was 50.6 years, 63.4% men. Of the participants, 65.9% had other health problems, 39% reported needing help with activities of daily living, 49% slept less than 8 hours per night, and 34.1% complained of some memory deficit. The average MMSE score was 26. Impairment of functional capacity and pain were the more important altered health states. Conclusion CIDP has important social and economic impacts, owing to functional impairments that can lead to professional and personal limitations. .

A avaliação da qualidade de vida (QV) e dos possíveis impactos dos déficits funcionais sobre o estado mental de pacientes com polirradiculoneuropatia inflamatória desmielinizante crônica (PIDC) pode contribuir para a melhor compreensão de aspectos evolutivos da doença. A presente investigação teve como objetivo estudar as atividades da vida diária depacientes com PIDC e avaliar a sua QV. Método Foram avaliados 41 pacientes através do Mini Exame do Estado Mental (MEEM) e do inventário de saúde SF-36®. Resultados A média de idade dos participantes foi 50,6 anos, 63,4% homens. Problemas adicionais de saúde foram referidos por 65,9%: 39% relataram necessitar de ajuda para atividades de vida diária, 49% dormiam menos de 8 horas por noite e 34,1% referiam alguma dificuldade de memória. A média do MEEM foi 26. Através do SF-36 foi verificado maior prejuízo na capacidade funcional; a referência a dor foi proeminente. Conclusão A PIDC pode ter importante impacto social e econômico em decorrência dos prejuízos funcionais primários e secundários que podem levar ao afastamento do trabalho. .

Coexistence of two chronic neuropathies in a young child: Charcot-Marie-Tooth disease type 1A and chronic inflammatory demyelinating polyneuropathy.

We report an 18-month-old Charcot-Marie-Tooth type 1A (CMT1A) patient who developed a rapid-onset neuropathy, with proximal and distal weakness, and non-uniform nerve conduction studies. The neuropathy responded well to immunomodulation, confirming the coexistence of an inherited and an inflammatory neuropathy. Unexpected clinical and/or electrophysiological manifestations in CMT1A patients should alert clinicians to concomitant inflammatory neuropathy. In addition, this association raises reflections about disease mechanism in CMT1A.

Chronic inflammatory demyelinating polyradiculoneuropathy in a boy with systemic lupus erythematosus.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, autoimmune peripheral neuropathy. Systemic lupus erythematosus (SLE) is a multisystemic, autoimmune disease that can affect the central nervous system in about 40% of patients, with prevalence and incidence unknown in the pediatric population due to lack of multicenter studies. We report the case of a 13-year-old Mexican boy, diagnosed with CIDP at the onset of SLE, beginning with progressive muscle weakness of lower and upper limbs, without affection of the central nervous system. The patient had positive ANA, antiDNAdc, antiBeta2glycoprotein, anti-cardiolipin, ANCA-C and X. He received intravenous immunoglobulin, cyclophosphamide, steroids, and azathioprine and showed clinical improvement. It is important to take into account the presence of peripheral neurological disorders in patients with pediatric SLE, considering CIDP as an uncommon presentation, making the diagnosis important for better treatment and evolution.

[Differences in the electrophysiological patterns in the distinct variations of the presentation of chronic inflammatory demyelinating polyneuropathy]. / Diferencias del patrón electrofisiológico en las variantes de presentación de la polineuropatía desmielinizante inflamatoria crónica.

INTRODUCTION: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a mixed (predominantly motor) neuropathy, with an autoimmune aetiology and different forms of clinical presentation including the idiopathic form and one related to concurrent diseases. Neurophysiological studies play a decisive role in its diagnosis. AIM: To evaluate the differences in the electrophysiological patterns in the two forms of presentation of the disease, based on the variables that were analysed by studying nerve conduction and the F wave. PATIENTS AND METHODS: The study involved 27 patients with a diagnosis of probable CIDP, according to American Academy of Neurology criteria from 1991. Of all the patients included in the study group, 17 presented primary CIDP and in 10 cases it was related to other diseases, such as diabetes mellitus, adenocarcinoma, multiple sclerosis and human immunodeficiency virus infection. Motor and sensory nerve conduction studies were performed, as well as F wave studies of the nerves in the four limbs. Statistical methods were applied to compare the neurophysiological variables in the two groups of patients. RESULTS: Significant differences were found to exist between the two groups. The sensory study was more affected in patients with primary CIDP, whereas the motor and F wave study were affected to a greater extent in patients with secondary CIDP; these cases displayed a predominantly axonomyelinic pattern, with greater involvement of the proximal motor segment. CONCLUSION: The different variant forms of presentation of CIDP display distinct electrophysiological behaviours.