Chronic inflammatory demyelinating polyneuropathy and ventilatory failure: report of seven new cases and review of the literature

Abstract

Clinical ventilatory dysfunction in CIDP is usually not an indicator of poor prognosis, and many patients recover without significant permanent disability. The mortality rate is similar to intubated patients with GBS. Patients with cardiopulmonary comorbidities and acute GBS-like onset of CIDP may be at higher risk of ventilatory failure which typically responds to 'standard' treatments of CIDP. Larger prospective studies are needed to define the prevalence, clinical spectrum and significance of ventilatory involvement in CIDP and to establish guidelines for evaluation and treatment.

The recurrent Guillain-Barré syndrome: a long-term population-based study

“…GBS is preceded in 60–70% of cases by an infectious illness 1–2 weeks before the onset of neurological symptoms, which is unusual in CIDP . However, some authors include relapsing–remitting cases into the CIDP category , with intermittent episodes associated with respiratory failure and recurrence after 18 months of full recovery . Moreover, CIDP with acute onset (A‐CIDP) was described with <4 weeks to nadir .…”

Chronic Inflammatory Demyelinating Polyradiculoneuropathy: From Bench to Bedside

“…Some patients may have a more acute onset or may present with multiple AIDP episodes with respiratory failure. 24 Most patients present with proximal and distal weakness, paresthesias, and sensory loss. The course may be slowly progressive or relapsing/remitting.…”

“…CIDP has been recognized worldwide, with varying prevalence (partly due to diagnostic criteria) from 1.0 to 8.9 per 100,000 persons. 24,[26][27][28][29][30] CIDP has also been recognized to have a higher prevalence in patients with human immunodeficiency virus (HIV) infection. 31,32 Whether the prevalence of CIDP is increased in diabetes mellitus is controversial.…”

“…36,37 Respiratory failure requiring ventilator support is rare, but can occur. 24 CNS lesions can also occur, varying from T2 hyperintense white matter lesions, atrophic cervical cord, and abnormal brainstem evoked potentials, although prominent upper motor neuron features are atypical and suggest an alternative diagnosis. [38][39][40][41] Other CIDP Variants Pure Sensory CIDP: Pure sensory CIDP has been reported by several authors as a CIDP variant.…”

Avaliação genotípica de pacientes com polineuropatia inflamatória desmielinizante crônica: estudo da duplicação/deleção do gene PMP22

“…As neuropatias são doenças do sistema nervoso periférico que podem determinar importante morbidade, por vezes severa, resultando em incapacitações intensas e freqüentemente permanentes, levando em alguns casos ao êxito letal (THOMAS et al, 1969;ŽIVKOVIĆ et al, 2011;SANTOS et al, 2014). Estas afecções possuem etiologia variada e todas as faixas etárias, raças e sexos podem ser acometidos (GRAVESON, 1959;MILLER, 1966;THOMAS, 1970;FREITAS et al, 1990;MARQUES Jr e BARREIRA, 1992;MYGLAND e MONSTAD, 2001;HUGHES, 2002;ENGLAND e ASBURY, 2004;MISRA, KALITA e NAIR, 2008;LOZERON, TROCELLO e KUBIS, 2013).…”