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• Vol.42 No.4
• CLINICA NEUROL, 42: 293−298, 2002
• Vol.42 No.4 contents

Original Article

Multiple system atrophy with a-/hypo-ceruloplasminemia: distribution of iron in brains of 2 autopsy cases

Hiroshi Kurisaki, M. D. ¹⁾, Harumi Yomono, M. D. ¹⁾, Shigeo Murayama, M. D. ²⁾ and Akira Hebisawa, M. D. ³⁾

¹⁾Department of Neurology, National Tokyo Hospital
²⁾Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology
³⁾Department of Pathology, National Tokyo Hospital

Objective: We presented first two cases of multiple system atrophy (MSA) with a-/hypo-ceruloplasminemia (hypo-Cp). To know whether hypo-Cp was a cause of MSA, we investigated distribution of iron in brains.
Methods: Investigating history, neurological sings and symptoms, neuroimagings, and neuropathological findings of the 2 cases, we demonstrate that these 2 cases were typical MSA. Serum ceruloplasmin (Cp) values of two cases were investigated, as well as those of 14 MSA patients diagnosed after the 2 cases. In the 2 cases, we compared distribution of lesions and distribution of iron depositions revealed by Berlin blue stain (iron stain). Further, we compared depositions of iron in substantia nigra, putamen, and dentate nucleus of the 2 cases with those of 4 control MSA, 2 Parkinson's disease (PD), 2 amyotrophic lateral sclerosis (ALS), and 3 controls.
Results: Case 1 was 68-year-old man who developed gait disturbance, and had anti-Parkinson disease drugs after diagnosis of PD. Parkinsonism was progressed, and became bed-ridden after 6 years when he died. Neuropathological finding was typical MSA from distribution of lesions, as well as existence of GCIs and NCIs. Case 2 was 61-year-old man who developed parkinsonism. After 9 years, he had tracheostomy, and after 11 years died of renal failure. Neuropathological finding was typical MSA.
With an investigation of serum Cp values of clinically diagnosed 14 MSA patients, we found 2 other cases of MSA with hypo-Cp. Iron stain of the 2 brains revealed that iron depositions were found in substantia nigra and putamen, but were not found neither in pontine base, cerebellum, nor inferior olive. Iron depositions were also seen in substantia nigra and putamen of control MSA cases as same degree as MSA with hypo-Cp, but iron depositions were fewer in PD, ALS and controls.
Conclusion: Clinico-pathological findings of the the 2 cases were those of typical MSA, but were not same as those of previously reported hypo-Cp. Previous reports suggested iron depositions as a cause of brain lesions, but, we concluded that, in the 2 cases, iron depositions were not a direct cause of MSA lesions. However, high incidence of association of hypo-Cp and MSA shown in our study suggests a relation between hypo-Cp and MSA.

(CLINICA NEUROL, 42: 293−298, 2002)
key words: aceruloplasminemia, multiple system atrophy, brain iron deposition

(Received: 10-Apr-02)