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Microgyria and Cytomegalic Inclusion Disease in Infancy by L

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Microgyria and Cytomegalic Inclusion Disease in Infancy by L J Clin Pathol: first published as 10.1136/jcp.12.5.427 on 1 September 1959. Downloaded from J. clin. Path. (1959), 12, 427. MICROGYRIA AND CYTOMEGALIC INCLUSION DISEASE IN INFANCY BY L. CROME AND N. E. FRANCE From the Fountain Hospital and the Mothers' Hospital (Salvation Army), London (RECEIVED FOR PUBLICATION APRIL 13, 1959) Many different malformations of the central neonatal jaundice, ascites with hepatomegaly, and nervous system can now be produced in the splenomegaly, and died at 24 days. Inclusion offspring of animals exposed during pregnancy to bodies were found in the kidneys, liver, and brain. x rays, drugs, and dietetic deficiency or excess of The brain weighed 130 g. and showed microgyria vitamins, but proof of environmental causation of of the neocortical areas. Necrosis was present in analogous human conditions is understandably the medial basal regions of both temporal lobes scarce. Occasional neural, somatic, or gonadal and in the cerebellum. The optic nerves were maldevelopment has been reported after the atrophic, there was marked hydrocephalus, and administration to pregnant women of aminopterin the corpus callosum was thin. On microscopy, (Thiersch, 1956), thiouracil (Morris, 1953), the necrotic areas showed infiltration by methyltestosterone (Nellhaus, 1958), and pro- lymphocytic and plasma cells with many gesterone (Reilly, Hinman, Pickering, and Crane, compound granular corpuscles, macrophages, and copyright. 1958; Wilkins and Jones, 1958). Nevertheless, calcified particles. The olfactory nerves were the maternal rubella and ionizing radiation remain seat of marked inflammatory change. Typical the only widely known extrinsic causes of human inclusions of cytomegalic inclusion disease were embryopathy. Hence the importance of the seen in the leptomeninges, ependymal, and glial suggestion that another, probably infective, cells, as well as in some of the undifferentiated condition, cytomegalic inclusion disease, may be matrix cells arranged in numerous dense also responsible for neural malformation (Diezel, periventricular foci. The only mature neurons 1954). to show inclusions were a few Purkinje cells, http://jcp.bmj.com/ It has been known for some time that the brain found after prolonged search. Architectonically, is one of the organs which may display the the palaeocortex and archicortex were normal and characteristic inclusions of cytomegalic inclusion the relatively older occipital cortex was better disease. Haymaker, Girdany, Stephens, Lillie, developed than the rest of the neocortex. It and Fetterman (1954) collected seven such cases appeared therefore to the author that the virus of in the literature and added one of their own. In cytomegalic inclusion disease attacked the foetus addition to the inclusions, some of these brains at a time when migration of nerve cells from the on September 29, 2021 by guest. Protected showed hydrocephalus, periventricular calcifica- periventricular matrix to the periphery was tion, focal softening, haemorrhages, astrocytic complete in the older, and still afoot in the newer. proliferation, perivascular inflammatory infiltra- parts of the brain, i.e., the beginning of the fourth tion, and ependymal granulations. These are, of month of gestation. course, evidence of neural disease, but not Diezel was able to re-examine histological necessarily of malformation. Diezel was the first preparations from the brains of the two cases of to report the association of cytomegalic inclusion cytomegalic inclusion disease previously reported disease with microgyria, or micropolygyria. by Hartmann (1948) and by Haymaker and his Microgyria is a true cerebral malformation co-workers. He found microgyria in one of these, characterized by an excess of small abnormally whilst the other had an anomaly of gyri, possibly formed gyri (Crome, 1952). Histological and of microgyric nature. Microgyria was possibly embryological considerations suggest that the present in a case of cytomegalic inclusion disease cause of this condition operates before the sixth presented by Mercer, Luse, and Guyton (1953); month of foetal development. the brain of this child had an irregular, thin-walled The patient described by Diezel was an infant cyst, 1.5 cm. in diameter, in the right parietal lobe, weighing 2,500 g. at birth who developed the cortical surface around the cyst was slightly J Clin Pathol: first published as 10.1136/jcp.12.5.427 on 1 September 1959. Downloaded from 428 L. CROME and N. E. FRANCE depressed and firm, while the irregular narrow and poorly aerated. The spleen weighed 14 g. gyri in this area had a "wrinkled " surface. A (average normal for age, 6 g.) and showed patchy further definite example of cytomegalic inclusion capsular thickening. The liver weighed 112 g. disease with microgyria was recently demon- (average normal for age, 88 g.). It was light green strated in this country by Strich (1958, and had a few small subcapsular groups of cysts personal communication), and it is the object of 1-2 mm. in diameter. The gall-bladder contained this communication to record another case. colourless mucoid material. The peritoneal cavity contained about 200 ml. of clear yellow Cae Report fluid. The stomach showed a small mucosal erosion. The small intestine contained meconium This girl was the issue of the second pregnancy by fine fibrous adhesions. of a 25-year-old unmarried mother. Two years and was bound together previously, she had had a miscarriage at 3 months, The large intestine was collapsed, containing only followed a few months later by possible glandular pale green mucus. The thymus weighed 5 g. and fever with lymphadenopathy and a rash. The Paul- the left femur was normal. The occipital bone Bunnell test was not done. Apart from slight was firmly united with both parietal bones and vaginal bleeding at the 12th week of gestation, her showed a prominent posterior projection. There second pregnancy was normal and the labour began were small haemorrhages in the dura. spontaneously at 32 weeks. Labour was complicated Histologically, the lungs showed extensive by mild ante-partum haemorrhage and resulted in aspiration of amniotic fluid and areas of well- delivery by the vertex of a female infant weighing marked hyaline membrane formation with 2,020 g. The placenta (760 g.) was large and friable. At birth, the baby showed numerous skin occasional alveolar haemorrhages. A single large haemorrhages of face, trunk, and lower limbs. The cell was seen in one of the sections which was abdomen was huge and lax with considerable attached to the alveolar lining and contained a separated by a enlargement of the liver and spleen. Respirations large oval intranuclear inclusion copyright. were moaning and grunting and there was much rib clear halo from the nuclear membrane showing recession. The submaxillary salivary glands were not condensation of chromatin. Its cytoplasm enlarged. In spite of oxygen therapy and treatment contained many small basophilic inclusions. with penicillin and streptomycin, her condition The spleen showed diffuse generalized rapidly deteriorated; moaning respirations continued death haemopoiesis. Many haemopoietic foci were also and occasional convulsive movements preceded present in the liver, which showed a considerable at 31 hours. accumulation of haemosiderin in the parenchymal Investigations and Kupffer cells. The subcapsular cysts were http://jcp.bmj.com/ Mother.-The blood group was 0, Rh negative (to lined by fibrillary material and were separated anti-D). The serum contained no atypical antibodies, from the capsule by a thin zone of liver cells. A and anti-A and anti-B titres were within normal few degenerate inclusion cells were seen in the bile limits. duct of one portal tract. Baby.-The blood group was B, Rh positive (to anti- Both kidneys showed cortical foci of interstitial D). The direct Coombs test was negative. Serum inflammatory cell infiltration mainly by bilirubin (at birth) was 2.3 mg. per 100 ml. Capillary lymphocytes and plasma cells. Dilated tubules blood contained 10.36 g. haemoglobin per 100 ml. containing many typical inclusion cells were on September 29, 2021 by guest. Protected (Haldane). Colour index was 1.32; R.B.C.s 2.64m. foci per c.mm. ; total nucleated cells, 12,700 per c.mm. present in some of these inflammatory platelets, 142,000 per c.mm. (Fig. 1). Glomeruli were mostly normal, but some were hyalinized and one contained an inclusion cell in a glomerular tuft. There was a Pathological Findings moderate amount of haemorrhage into the The body weighed 1,910 g., and was 43.5 cm. medulla and excessive haemopoiesis. Many (crown-heel) long. The head was microcephalic, typical inclusion cells without accompanying the circumference measuring 27 cm. compared inflammation were present in the submaxillary with 34.7 cm. which is the mean for this age. salivary gland. The parotid glands were normal. The abdominal circumference was 37 cm. There was generalized mild oedema and slight jaundice, Central Nervous System.-The unfixed brain with numerous haemorrhages into the skin, weighed 87 g., the range for a baby of 2,000 g. conjunctivae, and umbilical cord. being about 250 to 350 g. from our unpublished The heart showed marked dilatation of the observations. right side, and patency of the ductus arteriosus. The brain, brain-stem, and cerebellum had been The lungs (R. 13 g., L. 11 g.) were pale purple initially cut sagittally through the midline and, J Clin Pathol: first published as 10.1136/jcp.12.5.427 on 1 September 1959. Downloaded from copyright. FIG. 1.-Kidney, showing dilated tubules with typical inclusion cells and interstitial inflammatory cell exudate. Haematoxylin and eosin x 350. http://jcp.bmj.com/ on September 29, 2021 by guest. Protected MM G 2a 3 4 6 I 2 3 4 5 6 7 o5 FIG. 2.-External aspect of right cerebral hemisphere. FIG. 3.-Medial aspect of right cerebral hemisphere. J Clin Pathol: first published as 10.1136/jcp.12.5.427 on 1 September 1959. Downloaded from when re-examined for pur- poses of the present study#" five years after death, the material was in two parts.
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