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EnglishA 21-year-old man with a muscular atrophy of the left distal upper extremity is presented. The disorder had been progressive over a few years, showing an exacerbation of the hand's weakness when the patient worked in a chilled environment (i.e., in a cold room). The patient's diagnostic work-up was extensive and the MRI documented the presence of a cervical myelopathy, associated to an inversion of the physiological lordosis at the C5-C6 level, with a phenotype highly resembling Hirayama disease. This case indirectly supports the debated hypothesis that juvenile amyotrophy of the upper limb (Hirayama disease) is actually a type of cervical myelopathy, with a likely ischaemic pathogenesis of the ventral horns.
A 21-year-old man with a muscular atrophy of the left distal upper extremity is presented. The disorder had been progressive over a few years, showing an exacerbation of the hand's weakness when the patient worked in a chilled environment (i.e., in a cold room). The patient's diagnostic work-up was extensive and the MRI documented the presence of a cervical myelopathy, associated to an inversion of the physiological lordosis at the C5-C6 level, with a phenotype highly resembling Hirayama disease. This case indirectly supports the debated hypothesis that juvenile amyotrophy of the upper limb (Hirayama disease) is actually a type of cervical myelopathy, with a likely ischaemic pathogenesis of the ventral horns.
| Data di pubblicazione: | 2008 |
| Titolo: | A CERVICAL MYELOPATHY WITH A HIRAYAMA DISEASE-LIKE PHENOTYPE |
| Autori: | |
| Citazione: | Cerami, C., Valentino, F., Piccoli, F., & LA BELLA, V. (2008). A CERVICAL MYELOPATHY WITH A HIRAYAMA DISEASE-LIKE PHENOTYPE. NEUROLOGICAL SCIENCES, 29(6), 451-454. |
| Rivista: | |
| Digital Object Identifier (DOI): | http://dx.doi.org/10.1007/s10072-008-1058-3. |
| Abstract: | A 21-year-old man with a muscular atrophy of the left distal upper extremity is presented. The disorder had been progressive over a few years, showing an exacerbation of the hand's weakness when the patient worked in a chilled environment (i.e., in a cold room). The patient's diagnostic work-up was extensive and the MRI documented the presence of a cervical myelopathy, associated to an inversion of the physiological lordosis at the C5-C6 level, with a phenotype highly resembling Hirayama disease. This case indirectly supports the debated hypothesis that juvenile amyotrophy of the upper limb (Hirayama disease) is actually a type of cervical myelopathy, with a likely ischaemic pathogenesis of the ventral horns. |
| Settore Scientifico Disciplinare: | Settore MED/26 - Neurologia |
| Appare nelle tipologie: | 1.01 Articolo in rivista |
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