2010

Bilateral Ulnar Palsi İle Ortaya Çıkan Basınca Hassas Herediter Nöropati Olgusu

Hereditary neuropathy with liability to pressure palsy (HNPP) is a rare autosomal dominant disorder characterized by mononeuropathies at sites subject to compression. It causes recurrent, painless and transitory acute nerve palcies and rarely presents in two extremities. In this report, we describe a case of HNPP presenting as isolated, bilateral, acute, painless ulnar palsy. Neurologic examination revealed atrophy and weakness limited in intrinsic hand muscles bilaterally. Electrophysiological examination exhibited mild sensorimotor p o lyn e uro p a thy, bilateral conduction block of the ulnar nerve at the elbow and mild partial acute denervation findings on the abductor digiti minimi and first dorsal interosseus muscles. The diagnosis was based on clinical and electrophysiological features. This case is reported to emphasize the importance of exploring subclinic polyneuropathy in the patients who admit with multiple, acute, painless neuropathies and the value of electrophysiological examination in the diagnosis of HNPP. Basınca hassas herediter nöropati (BHHN) basıya maruz kalan bölgelerde mononöropatiler-le karakterize nadir görülen otozomal dominant geçiş gösteren bir hastalıktır. Ağrısız, tekrar-layıcı ve geçici akut sinir parezilerine neden olur ve nadiren iki ekstremitede meydana gelir. Biz; izole, bilateral, akut, ağrısız ulnar parezi ile ortaya çıkan bir BHHN vakası sunduk. Nörolojik muayenesinde bilateral tenar kaslar hariç olmak üzere, intrensek el kaslarında sınırlı atrofi ve güçsüzlük tespit edildi. Elektrofizyolojik incelemede hafif bir sensorimotor polinö-ropati, dirsekte iki yanlı ulnar sinirin tam ileti bloğu ile abduktor digiti minimi ve birinci dorsal interosseus kaslarında hafif parsiyel akut denervasyon bulguları mevcuttu. Tanı elektro-fizyolojik ve klinik özelliklere dayanılarak konuldu. Bu olgu birden fazla sayıda, akut, ağrısız nöropatilerle başvuran hastalarda subklinik polinöropati varlığını göstermenin önemini ve BHHN tanısında elektrofizyolojik incelemenin değerini vurgulamak için sunulmuştur.

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