A Japanese male developed deafness, pyramidal signs and ataxia at age 50. A cerebrospinal fluid examination showed elevated levels of iron, transferrin and ferritin. Brain MRI showed atrophy of the cerebellum and pons as well as potential iron deposits on the surface of the brain. At autopsy, the brain weighed 1090 g and showed severe atrophy and necrosis of the cerebellum. No vascular malformation was observed. Extensive deposits of hemosiderin that were well stained with Berlin blue and ferritin immunohistochemistry were present at the surface and in the superficial layers of the cerebrum, brainstem, cerebellum and spinal cord. In these regions, numerous AT8 (p-τ)-immunopositive deposits were present in neurons and glia. In addition, phosphorylated α-synuclein-immunopositive Lewy bodies and neurites were observed in the brainstem nuclei. In the present report, the authors derive the novel insight that superficial siderosis is a distinctive entity associated with tauopathy and synucleinopathy.

A 22-year-old male presented with granulomatous angiitis manifesting as headache. Computed tomography (CT) demonstrated a low-density area and ring-like enhancement in the right fron toparietal region. Steroid therapy caused rapid improvement in the signs and symptoms. Withdrawal of steroid was followed by recurrence of the headache and low-density area. The steroid treatment was resumed until he became asymptomatic 3 months after admission. He was followed up as an outpa tient once every 2 weeks. One month after discharge, he complained of mild headache, and CT again demonstrated a mild increase in the low-density area. He was followed up continuously without steroid treatment. Two months after discharge, he suddenly lost consciousness, and CT demonstrated multiple intracerebral hematomas in the bilateral parietal regions. Removal of an intracerebral hematoma in the right parietal region and external decompression were carried out, but he died 3 days later. Histological examination of tissues from the right parietal cortex and leptomeninges demonstrated granulomatous inflammation with several giant cells in the vessels of the cerebral cortex and leptomeninges.

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Objective: To analyze the correlation between neuropathology and autopsy images (AIs) using newly‐introduced 3‐Tesla (3T) MRI. Methods: In the six cases here under investigation‐‐two amyotrophic lateral sclerosis (ALS), two spinocerebellar degeneration (SCD), a dementia with Lewy bodies (DLB) and a mild cognitive impairment (MCI)‐‐AIs of 3T MRI (GE Signa) were administered just before each autopsy using T1, T2, FLAIR and T2*. Fixed brains were dissected at the exact same levels of AIs of MRI. Sections were stained using conventional and immunohistochemical methods. Results: Clear MRI images of the brain and spinal cord without motion and pulsation artifacts were obtained. In ALSs, neuropathology showed degeneration of the anterior horn cells and pyramidal tracts. MRI demonstrated those pathologic alterations as the atrophy and hyperintense lesions of the pyramidal tract in the spinal cord and the brainstem. In SCDs, the atrophy of the brainstem and cerebellum exactly corresponded to the results of MRI. In MCI, numerous neurofibrillary tangles and neuropil treads were seen in the hippocampal formation that was atrophic on MRI. Although Lewy bodies were present in the neocortex and brainstem in DLB, only mild cerebral atrophy was seen by MRI. Conclusions: Since AIs of 3T‐MRI reflect the final conditions of the central nervous system, this method may enable us to analyze neuropathologic alterations, precisely.