Hemochromatosis of the Central Nervous System

Superficial Siderosis Mimicking Amyotrophic Lateral Sclerosis

“…18 Autopsies of patients with superficial siderosis have shown anterior horn cell degeneration and pigmentary changes presumably resulting from hemosiderin deposition. 19,20 However, despite these striking abnormalities at autopsy, LMN weakness may not be evident clinically. 18 Progressive deposition of hemosiderin of anterior horn cells or at the cervical nerve root entry zone is a possible mechanism for the LMN syndrome in our patient.…”

Superficial haemosiderosis of the central nervous system

“…The author concluded that this condition was due to the diffusion of blood pigment into the marginal zone of the central nervous system and suggested that the condition was probably the result of chronic subarachnoid bleeding. Subsequently, however, additional cases were reported by Lewey and Govons (1942), Cammermeyer (1947), Neumann (1948Neumann ( , 1956, Garcin and Lapresle (1957), Rosenthal (1958), and McGee, van Patter, Morotta, and Olszewski (1962). Neumann (1948,1956) concluded that the pathological changes in the central nervous system which she observed in her two cases were the result of haemochromatosis of the central nervous system.…”

Superficial hemosiderosis of the central nervous system

“…Superficial hemosiderosis (SH) of the CNS is a rare condition, with only 49 cases reported in the literature (1)(2)(3)(4)(5). The clinical manifestations of SH are remarkably consistent and include cerebellar signs, hearing loss, and spastic quadriparesis (6,7), at times accompanied by dementia and sphincter disturbances (7)(8)(9). In about half of the reported cases, the etiology was recurrent subarachnoid hemorrhage secondary to a vascular malformation, tumor, or posthemispherectomy (1)(2)(3)(4)(5)(6)(7)(8)(9).…”