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.

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.

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 [10.1007/s10072-008-1058-3.].

A CERVICAL MYELOPATHY WITH A HIRAYAMA DISEASE-LIKE PHENOTYPE

CERAMI, Chiara;VALENTINO, Francesca;PICCOLI, Federico;LA BELLA, Vincenzo
2008

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 MED/26 - Neurologia
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 [10.1007/s10072-008-1058-3.].
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Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10447/71828
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