Increased prevalence of obstructive sleep apnoea in CMT

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J Neurol Neurosurg Psychiatry. 2008 Jul;79(7):829-31

Increased prevalence of obstructive sleep apnoea in patients with

Charcot-Marie-Tooth disease: a case control study.

Dziewas R, Waldmann N, Böntert M, Hor H, Müller T, Okegwo A,

Ringelstein EB, Young P.

Department of Neurology, University Hospital of Münster, Albert-

Schweitzer-Strasse 33, 48129 Münster, Germany. dziewas@...

Obstructive sleep apnoea (OSA) has previously been described in a

large family suffering from Charcot-Marie-Tooth disease type 1

(CMT1). In the present study, we used a case control design to

establish whether this suggested link between OSA and CMT1 may also

be found when studying genetically non-related patients.

12 patients with CMT1 and 24 control patients matched for age, sex

and body mass index (BMI) were included in the study. Neurological

disability was graded with a previously established 6 point score.

All patients underwent overnight polysomnography. The mean apnoea-

hypopnoea index (AHI) of patients with CMT1 was 10.5 (16.3) which was

significantly higher than that of the control group (1.5 (1.3)).

Five out of 12 patients with CMT1 had an AHI > or =10/h compared with

1 of 24 control patients (p<0.01).

In patients with CMT1, a significant correlation between AHI and

neurological disability was found (Spearman r = 0.62; p = 0.031)

while BMI and age were not related to AHI. CMT1, in particular CMT1A,

predisposes with disease progression to the development of OSA.

Pathophysiologically, one may assume that CMT1 related pharyngeal

neuropathy increases the collapsibility of the upper airway which in

turn leads to recurring obstructive respiratory events.