Herbert L. Cares

Ossification in the region of the posterior longitudinal ligament as a cause of cervical myelopathy

In 1967, we operated on a patient for cervical cord compression caused by an unusual type of ectopic bone formation in the spinal canal which we tentatively identified as ossification within the posterior longitudinal ligament. Until that time we had believed that calcification of the vertebral ligaments is a phenomenon secondary to spondylosis (Oppenheimer, 1942) and does not cause symptoms by itself. However, about the same time, a description of no less than 18 similar cases was published by Onji, Akiyama, Shimomura, Ono, Hukuda, and Mizuno (1967). Additional cases were also found in Japan (Yokoi, 1963). It seems, therefore, that these cases, while probably rare, constitute a distinct pathological entity. Although calcification and ectopic bone formation have been described before in various ligaments of the spine (Oppenheimer, 1942; Cloward, 1967), they were found accidentally without giving rise to clinical symptoms. A search for similar cases was undertaken and six additional patients with such abnormalities were found.

Olfactory meningiomas. Report on a series of twenty-five cases.

SummaryThe clinical features of 25 cases of olfactory groove meningiomas operated between 1950 and 1970 are presented, discussed and compared with earlier series. Particular attention is focused on early symptoms and diagnostic studies that might lead to earlier recognition of these tumors. The surgical mortality rate was 12 per cent. However, this improvement of operative results over some of the previously reported series does not seem to be related to earlier recognition of the tumors; most of them were very large at the time of operation and the average patients complaints extended over a long period of time before the correct diagnosis was established. Although radioactive brain scanning and carotid angiography are very helpful in outlining these tumors, early surgical treatment is still prevented by the lack of careful and timely evaluation of patients who present the initial symptoms of anosmia, mental abnormalities or visual deterioration.

Giant cell lesions of the skull

SummaryGiant cell tumor, chondroblastoma, brown tumor of hyperparathyroidism, aneurysmal bone cyst, fibrous dysplasia, and reparative giant cell granuloma have been discussed with reference to their behavior in the skull. They have been compared with special regard to clinical presentation, roentgenographic appearance, gross and microscopic pathologic characteristics, treatment, and prognosis.

Histopathology of the nervous system in carnosinase enzyme deficiency with mental retardation

A few years ago, Perry e t al.’ reported a heretofore unknown metabolic disorder associated with progressive motor dysfunction, mental retardation, deafness, and blindness in two siblings who had appeared normal during the first few months of life. In the course of their physical and mental deterioration, which h a d b e e n initiated by seizures, increased amounts of carnosine were found in the urine and an elevated carnosine concentration in the serum and a deficiency of serum carnosinase activity were demonstrated.2 These higherthan-normal levels of serum carnosine persisted even when the children were on diets free of meat products. Recently, two physically and mentally retarded boys were observed in the follow-up studies of infants born a t the Children’s Hospital (in the Child Development Program) with a disease pattern similar t o that described by Perry e t al. The boys were severely mentally retarded; however, their younger sister appeared normal. In all three siblings carnosinuria, carnosinemia, and serum carnosinase deficiency were demonstrated. Extracts of liver, kidney, and spleen obtained after death of the older boy were examined by electrophoresis. There was only one of the two forms of carnosinase normally present in these tissues. In the sera of the parents carnosinase activity was detectable but a t a lower level than normal. (A detailed account of the methods used for the assay of the enzyme carnosinase will be given elsehere.^) We shall report briefly the clinical history of