Autopsy findings in three family members with a presumably acquired immunodeficiency syndrome of unknown etiology.

This paper presents clinical, immunological and post-mortem findings in three family members (husband, wife and daughter) who all died in 1976 after having had chronic and recurrent opportunistic infections for many years. In all of them a progressive, presumably acquired T-lymphocyte defect associated with B-lymphocyte dysfunction had been diagnosed several years before death. The clinical and immunological findings are compatible with those seen in acquired immunodeficiency syndrome (AIDS) caused by HTLV-III/LAV infection, but examinations of stored blood samples from the three patients were negative with regard to the presence of HTLV-III/LAV antibodies. This immunodeficiency may therefore have been caused by an infectious agent of unknown nature. The most remarkable finding on post-mortem examination was the presence of a granulomatous encephalomyelitis with multinucleated giant cells in the husband and his wife. In addition, the wife's CNS revealed scattered microglial nodules. No infectious agents could be demonstrated, and the etiology of this peculiar CNS affection therefore remains obscure.

Brainstem lesion with coma for five years following manipulation of the cervical spine.

A woman, aged 38, lost consciousness immediately after manipulation of the cervical spine and remained in coma for nearly 5 years in a combined decorticate and decerebrate state. Cerebral angiography revealed impaired circulation in the vertebrobasilar system. The EEG initially showed generalized cerebral dysrhythmia but tended, over the years, to become more normal with desynchronized fast activity of low voltage. Neuropathological examination postmortem revealed a large cystic lesion in the pressure equalization area of the carotid and basilar circulation in the upper pons, mesencephalon, posterior hypothalamus and basal thalamus. No definite stenosis or occlusion was seen in the cerebral vessels on angiography or at the postmortem examination. The mechanism of the lesion is considered to have been temporary interference with the blood flow in the vertebrobasilar system during manipulation of the cervical spine sufficient to cause ischemia and subsequent infarction of the brainstem. The pathogenesis of vascular lesions of the brain stem following manipulation of the cervical spine is discussed briefly.

Experimental hydronephrosis. Influence of infection on the histological picture.

The present paper is concerned with a histological examination of 110 pairs of rat kidneys from an experimental investigation on unilateral hydronephrosis previously published. Urine sampled by puncture of the obstructed pelvis remained sterile in cultures from most of the cases, though in 22 of a total of 184 cases growth of bacteria occurred. Intravenous injection of E. coli resulted in substantial growth of the bacteria in similar samples. In 2 cases the untreated control kidney displayed histological evidence of infection. The histology indicated that damage to the renal parenchyma in hydronephrotic kidneys probably predisposes the kidney for hematogenous infection.

Treatment of rheumatoid pachymeningitis involving the entire thoracic region.

A 67-year-old woman developed a spinal cord compression syndrome due to rheumatoid pachymeningitis with a roentgenological block from T2 to T11. The dura was surgically split from T1 to T12 and carefully dissected away from the spinal cord and then left open without grafting. The patient imporved and 3 years after the operation the spinal subarachnoid space and the circulation of cerebrospinal fluid were normal.

Ependymoma: a follow-up study of 101 cases.

One hundred and one patients with histologically confirmed ependymomas were studied over a 22-year period. Choroid plexus papilloma and sub-ependymoma were not included. About half of the tumors were intracranial, with the majority of these infratentorial. The intraspinal tumors were equally divided between intramedullary and the "cauda" group. The majority of the intracranial tumors occurred in children, while almost all the intraspinal tumors were in adults. The histologic classification consisted of "typical ependymoma" (cellular, papillary and myxopapillary patterns) and "anaplastic ependymoma". The intracranial and intramedullary tumors showed a predominantly cellular pattern, while the myxopapillary type was found only in the "cauda" group. The histology seems to be of limited value in assessing the prognosis in an individual patient with ependymoma. The postoperative prognosis was poor in the intracranial tumors, although radiotherapy increased the survival time without affecting the eventual fatal outcome. The prognosis in the intraspinal group was much better, with three-fourths of the patients living for at least 10 years. No patient with an anaplastic tumor survived for more than 6 years.

X-linked aqueductal stenosis.

A family is reported in which eight members of one generation were affected by the syndrome hydrocephalus with aqueductal stenosis. With the exception of one child who lived for several weeks, they all died at or within 10 days of birth. Autopsy of a pair of affected twins showed marked stenosis of the aqueduct of Sylvius with fusion of the lamina quadrigemina. There were no signs of previous or present inflammatory changes or neoplasia. All the affected individuals were males, and the familial and pathological data presented support the concept that aqueductal stenosis in this family was due to an X-linked gene, and may have a developmental origin.