Chronic Axonal Neuropathy
Source: Teunissen LL, et al. Differences between hereditary motor and sensory neuropathy type 2 and chronic idiopathic axonal neuropathy: a clinical and electrophysiological study. Brain 1997;120:955-962.
Idiopathic axonal neuropathy is an all-too-frequent and vexing neurologic conundrum, with 20% of patients remaining unclassified despite intensive evaluation (McLeod JG. J Neurol Neurosurg Psychiatry 1995;58:274-283). Among those seen at a specialized medical center with hitherto undiagnosed neuropathy, the largest group, 42%, have an inherited disorder (Dyck PJ, et al. Ann Neurol 1981;10:222-226). However, hereditary axonal sensorimotor neuropathy (HMSN type 2) can be difficult to confirm in family members and may thus be difficult to differentiate from acquired chronic idiopathic axonal polyneuropathy (CIAP).
Among 48 patients with CIAP and 47 with HMSN type 2, Teunissen identified features that may allow clinical separation of these entities. (See Table.)
Table
Possible Differences in HMSN Type 2 and CIAP
HMSN type 2 CIAP
Onset age (mean) 39 years 60 years (P < 0.05)
Initial symptom motor (85%) sensory (60%) (P < 0.0001)
Hand Weakness 53% 25%
Arm atrophy 53% 29% (P < 0.05)
Leg atrophy 77% 44% (P < 0.05)
Fasciculations 49% 31%
Pes cavus 57% 13% (P < 0.0001)
Claw toes 30% 13% (P < 0.05)
Handicap more severe less severe
It would appear that in the absence of a family history of polyneuropathy, the diagnosis of HMSN type 2 is unlikely unless motor symptoms predominate. mr
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