Abstract
Several conditions have been reported to mimic motor neuron disease (MND) and
misdiagnosis remains a common clinical problem. Peripheral neuropathy is a
classic feature of many vasculitic syndromes and in some patients it may be the
only manifestation of vasculitis. We report a case of ANCA-related vasculitic
neuropathy where the clinical presentation was suggestive of MND. A 42-year-old
woman was admitted to our centre to confirm a diagnosis of MND made elsewhere.
Clinical examination revealed postural tremor at the right hand, mild tongue
atrophy with diffuse fasciculations and brisk tendon reflexes without other
muscular weakness or atrophies. Electromyography demonstrated denervation in
tongue and in the first dorsal interosseous of right hand ; motor evoked
potentials disclosed normal central motor conduction time. Laboratory studies
revealed only a mild increase of p-ANCA. A muscle biopsy showed a small
inflammatory infiltrate around a vessel. The patient started high dosage of oral
steroids. After one year of follow-up the patient suspended oral steroids,
postural tremor of the right hand disappeared and tongue fasciculations were
reduced. Vasculitis may mimic a MND, particularly in the absence of sensory
involvement. Caution should be exercised in the clinical diagnosis of MND. Muscle
biopsy is indicated in patient with atypical MND especially in those with an
exclusive involvement of lower motor neuron.
Lingua originale | English |
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pagine (da-a) | 109-111 |
Numero di pagine | 3 |
Rivista | Acta Neurologica Belgica |
Stato di pubblicazione | Pubblicato - 2008 |
Keywords
- ALS
- Vasculitis