We recently observed a patient with neurofibromatosis complicated by bilateral upper extremity elephantiasis and a Brown-Séquard type of hemiplegia with ipsilateral diminution of pain and temperature response. Four cases of Brown-Séquard syndrome directly associated with this disease have been reported.1 To our knowledge, there have been no previously reported cases of bilateral upper extremity elephantiasis in neurofibromatosis. This case, in addition, demonstrates the ability of an apparently long-standing cervical deformity to produce a sudden hemiplegia.
The recognition of "multiple neuromas" as a disease entity preceded Recklinghausen's classical description of neurofibromatosis by 33 years.2 Bruns described the myriad clinical manifestations of the disease and called attention to the incidence of large distorted limbs.3 The bone alterations in multiple neurofibromatosis were not fully appreciated until after clinical roentgenology was firmly established. The early work by Gould4 and the observations of Brooks and Lehman5 emphasized the almost universal
Hudson LH, Cox TR. BROWN-SÉQUARD SYNDROME WITH BILATERAL ELEPHANTIASIS IN NEUROFIBROMATOSIS. JAMA. 1956;161(4):326–328. doi:10.1001/jama.1956.62970040003007a
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