cpc9705

The 100th Clinicopathological Conference at Odawara Municipal Hospital on May 12 1997

SN-824 OOOO, XXX 73 y. female.

Date of Autopsy; October 25 1996 (14 hrs. p.m.)

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Clinical diagnosis by the Dept of Int Med:

1. Idiopathic superficial siderosis of the brain

2. Aspiration pneumonia

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Patho-anatomical diagnosis by Dr. Hasegawa of the Dept of Pathology:

1. Idiopathic superficial siderosis of the brain and spinal cord

2. Aspiration pneumonia

3. [Neurogenic urinary bladder] with hydroureteronephrosis

A. Idiopathic superficial siderosis:

1. Brown staining of the pia along the Sylvian fissure, measuring 10 x 4 cm in extension, left, and 10 x 5 cm, right, the orbital surface along the olfactory bulbs of the frontal lobes, 4 x 3 cm in extension, the base of the brain, the cerebellum, and around the superior and inferior colliculi. Moderate dilatation of the lateral ventricles. Marked atrophy with degeneration and necrosis of the cerebellar vermis and hemispheres, especially severely damaged at the upper surface and cerebellopontine angle, covered with thickened dark brown leptomeningeal tissue, in association with atrophy of the pons. No tentorial and tonsillar herniation. Pineal gland rather whitish. Grayish brown staining of the pia of the whole length of the spinal cord, with unstained and apparently normal root nerves and cauda equina. Clear cerebrospinal fluid without subdural, nor subarachnoid hemorrhage. Histologically,

a. Deposition of hemosiderin pigments in the pia matter and the perivascular spaces of Virchow-Robin of the subpial cortex of the cerebral hemispheres, moderate-to-severe, and in the wall of both the lateral and third ventricles, very slight.

b. Deposition of hemosiderin pigments in the pia matter and the perivascular spaces of Virchow-Robin of the white matter of the spinal cord, especially in the outer layer of the whole circumference of the white matter increasingly prominent bellow the upper thoracic level down to the lower lumbar level, in association with demyelination of the same layer.

c. Deposition of hemosiderin pigments in the pia matter and the perivascular spaces of Virchow-Robin under the damaged portion of the cerebellar cortex, in maximum degree, in association with degeneration and infarctoid necrosis of the crests of the cortical folia and neuronal cell loss of the dentate nuclei.

d. Round-to-oval granular and eosinophilic bodies (so-called "ovoid bodies") distributed in the densely siderotic zones of the white and gray matter of the cerebellum, in the molecular and external granular layer, i.e., the second and third layer of Brodmann, of the cerebral cortex, and spinal cord, including the tractus opticus and acoustic nerve root.

e. Psuedocalcium deposition in the wall of the small vessels of the globus pallidus and medulla oblongata, mild.

f. Unremarkable neuronal cells of the pigmented nuclei of substantia nigra and locus coeruleus.

2. No apparent AV malformation, aneurysm, neither complete occlusion due to arteriosclerosis, of the cerebral arteries. Histologically, fibrous intimal thickening of the basilar artery with up-to-20% stenosis.

B. Sequels and findings related to A:

1. Chronic cystitis of the urinary bladder with marked dilatation of the lumen and trabeculation (neurogenic bladder), containing pyuria.

2. Hydroureteronephrosis of both sides, with dilatation of the renal pelvis and the whole length of the ureter, measuring 3 cm in average in circumference. Pressure atrophy of the renal cortex, mild. Histologically, diffuse lymphocytic infiltrate in the submucosal layer of the urinary bladder, ureter, and renal pelvis, with fairly well retained renal glomeruli and moderate intimal thickening of the interlobular arteries.

3. Unremarkable ganglion cells of the Auerbach's plexus of the small and large intestines.

4. Unremarkable pituitary gland without discernible siderosis.

5. Microcystic encephalomalacia of the anterior left portion of the pons (fibrae corticopontinae, corticonucleares et corticospinalis), measuring 8 x 2 mm in size on glass slide, accompanied by mobilization of lipid-laden macrophages and without discernible siderosis.

C. Other findings:

1. Aspiration pneumonia; Bronchiectasis with mucus packing of both the upper lobes, moderate (260; 540 g). No adhesion of the pleurae. Histologically, necrotic foci of various sizes and shapes surrounded by mobilization of multinucleated giant cells of Langerhans' type and foreign body type, epithelioid cells and lymphocytes, scattering in the dilated lumen of bronchi and mainly subpleural lung parenchyma, most prominent in the left S-1 and S-6. Acid-fast stain and Rhodamine with fluorescein negative.

2. Regurgitation of gastric content to the esophagus.

3. Congestion of the liver (1,250 g) and the spleen (75 g).

4. Coronary arteriosclerosis of the heart (310 g), mild, with sporadic patchy atheromas.

5. Atherosclerosis of the aorta, mild, with calcification at the lower abdominal portion. Elastic thoracic aorta.

6. Normocellular mixed bone marrow of the vertebrae, sternum and upper 1/3 of the femur, with a few lymphocytic follicles in the sternum.

7. Atrophy of the adrenal cortexes (6.0; 5.5 g), and thyroid with mild lymphocytic infiltrate (15 g).

8. Atrophic uterus and ovaries.

9. Emaciated aged female cadaver (147 cm/ 37 kg).

Clinicals: Familial and past medical history was unremarkable except for hypertension. She had noticed impaired hearing of the left side and dysarthria since 1985, and also gait disturbance since 1986. She was referred to the dept. of internal medicine in 1987 for ataxic gait and sensory hearing loss, when CT-scan revealed marked atrophy of the cerebellum. She was diagnosed with spinocerebellar degeneration, and administered TRH on an outpatient basis and 6 times of admissions in 1987 to 1992. Superficial siderosis was suspected with MRI images during the 7th admission in August and September 1995. CSF was positive for iron and ferritin. She was admitted on April 29 for high fever (9th admission), and prescribed with PIPC etc. on a diagnostic basis of pneumonia of the right upper lung lobe. She was brought to the hospital on ambulance car in state of unconsciousness on November 24 1996, followed by exitus lethalis in that night.

Comments: This is a very rare case of idiopathic superficial siderosis. Although there was no identifiable source of subarachnoid hemorrhage, maximum tissue destruction was observed in the cerebellum, so it seems reasonable to suppose that hypothetical bleeding occurred in this area. This assumption of hemorrhage as a cause of superficial siderosis, though, remains open to question. The direct cause of death was obviously aspiration pneumonia, which histologically mimics tuberculosis in particular in this subject.

References:

1. Hughes JT, Oppenheimer DR. Superficial siderosis of the central nervous system. Acta neuropathol 1969; 13:56-74.

2. Koeppen AHW, Barron KD. Superficial siderosis of the central nervous system. A histological, histochemical and chemical study. J neuropathol exp neurol 1971; 30:448-469.

3. Grunshaw ND, Blandhard KS, Hussain SSM, Grace ARH. Superficial siderosis of the central nervous system - diagnosis by magnetic resonance imaging. Clincal Radiol 1993; 48:186-188.

4. Bauer, E.: Uber einen Fall von Hamochromatose mit besonderer Beteiligung von Gehirn und Ruckenmark. Inaug Diss Wurzburg 1928.

5. Noetzel, H.: Diffusion von Blutfarbstoff in der inneren Randzone und auseren Oberflache des Zentralnervensystems bei subarachnoidaler Blutung. Arch Psychiat Nervenkr 1940; 111: 129-138.

May 12, 1997

Akio Hasegawa, MD, PhD

Certified Pathologist (JSP #832), Odawara/Tokyo, Japan

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