Abstract Description
Background: Carpal tunnel syndrome (CTS) is common in the patients with hereditary transthyretin amyloidosis (ATTRv) and known to occurred before the diagnosis. Since treatment is now available, the importance of early detection of ATTRv has recently been emphasized not only in sporadic diseases but also in carriers. The aim of this study is to identify the aspect of CTS seen in ATTRv in ultrasound and MRI.
Methods: Four patients with CTS symptoms and one asymptomatic carrier were enrolled. They were checked the clinical symptoms and signs of carpal tunnel syndrome through neurologic examination and underwent wrist ultrasonography (US) and wrist MRI to measure the cross-sectional area (CSA) of the median nerve and to observe morphology. The nerve conduction test results were used to grade CTS.
Results: CTS grade 5 was found in two patients with FAP stage 2, but one (Ala55Pro) had mild thickening of flexor tendon but normal median nerve in US and MRI while the other (Asp38Ala) had normal findings. In stage 1 patient (Lys35Asn) and carriers (Asp38Ala) who had normal NCS, US and MRI were also normal. Only one patient (Lys35Asn) with CTS grade 2 who was diagnosed with CTS prior to ATTRv diagnosis showed diffuse swelling of median nerve with increased intraneural fascicular signal on MRI.
Discussion: Although there are limitations due to the small number of patients, CTS associated with ATTRv did not show a correlation between NCS with US or MRI. Detailed morphological evaluation rather than measurement of CSA might help in the early diagnosis of ATTRv-related CTS.
Presenters
Authors
Authors
Prof. Jeeyoung Oh - Department of Neurology, Konkuk Univeristy Medical Center, Seoul, Korea , Prof. Dong Wook Kim - Department of Neurology, Konkuk University College of Medicine, Seoul, Korea , Prof. Yang-Ki Min - Department of Neurology, Hallym University Medical Center, Kangnam Sacred Heart Hospital, Seoul, Korea , Prof. Byoung Joon Kim - Department of Neurology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea , Prof. Byung-Jo Kim - Department of Neurology, Korea University College of Medicine, Seoul, Korea
