CMT 1A and 2A: Different clinical and magnetic resonance imaging features betwee

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Neuromuscul Disord. 2008 Jul 3.

Different clinical and magnetic resonance imaging feature sbetween

Charcot-Marie-Tooth disease type 1A and 2A.

Chung KW, Suh BC, Shy ME, Cho SY, Yoo JH, Park SW, Moon H, Park KD,

Choi KG, Kim S, Kim SB, Shim DS, Kim SM, Sunwoo IN, Choi BO.

Department of Biological Science, Kongju National University, Gongju,

Republic of Korea.

Charcot-Marie-Tooth disease type 1A (CMT1A) is the more frequent

cause of demyelinating CMT, and CMT2A is the most common cause of

axonal CMT. We conducted a magnetic resonance imaging (MRI) study on

39 CMT1A and 21 CMT2A patients to compare their neuroimaging patterns

and correlate with clinical features.

CMT1A patients showed selective fatty infiltration with a preference

for anterior and lateral compartment muscles, whereas CMT2A patients

showed a preference for superficial posterior compartment muscles.

Early-onset CMT2A patients showed more severe leg fatty atrophy than

late-onset CMT2A patients. In late-onset CMT2A, soleus muscle was the

earliest, and most severely affected than the other leg muscles.

Selective involvement of intrinsic foot muscles is a characteristic

pattern of minimal CMT1A and CMT2A. Our MRI study demonstrates

different patterns of fatty infiltration involving superficial

posterior compartment muscles in CMT2A (partial T-type), and peroneal

nerve innervated muscles in CMT1A (P-type).