Howard L. Steinbach

The Significance of Bilateral Basal Ganglia Calcification

Bilateral basal ganglia calcification was first recognized as a histologic entity in 1855 and described independently in that year by Virchow (1) and Bamberger (2). This phenomenon was encountered postmortem by many authors during the next eighty years but was not reported as a radiographic finding until 1935, when Fritzsche (4) and then Kasanin and Crank (5) described the roentgen appearance of this specific type of intracranial calcification in living patients. Four years later, in 1939, its occurrence in idiopathic hypoparathyroidism was described by Eaton, Camp, and Love (6). Its occurrence in pseudohypoparathyroidism, another disease characterized by abnormal calcium phosphorus metabolism, was first reported in 1945 by Sprague, Haines, and Power (14). Camp re-emphasized the association with hypoparathyroidism in 1947 (18). Anatomical Considerations The basal ganglia (Fig. 1) are masses of gray matter lying in the base of each cerebral hemisphere. They include three major structures—the caudate nucleu...

Measurement of the Heel-Pad as an Aid to Diagnosis of Acromegaly

Acromegaly is an insidious disease affecting all tissues of the body, not all, however, to the same extent. The involvement varies in different patients, probably the result of individual genetic make-up and the variable response of tissues to the stimulus of excessive growth hormone. Another factor may be the extent of imbalance of various hormones caused by the expanding lesion in the sella turcica. In general, the response of the tissues is one of hyperplasia and hypertrophy. The size of the bones may increase because of an accelerated endochondral ossification or appositional bone growth. In a previous investigation of the roentgen features of acromegaly (1), the most constant feature noted was an increase in thickness of the soft tissues of the fingers, toes, and scalp. This thickening diminished slightly after successful irradiation or surgical removal of the pituitary gland, but the tissues did not revert to normal size. The thickening of the skin is caused primarily by increased connective tissue,...

A mechanism of the production of pseudofractures in osteomalacia (Milkman's syndrome).

Osteomalacia is a metabolic disease of bone in which there is a deficient mineralization of the bony matrix. The amount of matrix present is normal or slightly increased. The defective deposition of calcium salts is due to the lowered concentration of calcium or phosphorus, or both, in the body fluids, as a result either of impaired absorption from the gastrointestinal tract or increased renal excretion. Because of their decreased mineral content, the bones are weakened, becoming soft and bending readily. Deformities of the extremities, pelvis, vertebrae, and skull may occur. As a result of the weakening of the bones, the osteoblasts are stimulated to lay down new bone matrix and elaborate alkaline phosphatase, which is usually present in abnormal concentration in the blood serum (1). In some cases of osteomalacia, multiple, almost symmetrical, radiolucent bands of diminished density, resembling fractures, occur in the cortex of the bone. These pseudofractures are seen most frequently in the axillary bord...

Roentgenology of Reiter's Syndrome

Abstract Roentgenograms of 55 patients with well-documented Reiters syndrome demonstrated a tendency toward asymmetric arthritic involvement, most commonly in the sacroiliac joints, toes, and heels. The distribution of these findings will often permit a presumptive roentgenographic diagnosis in the absence of a classic clinical history.

Evolution of Skeletal Lesions in Pseudohypoparathyroidism

Pseudohypoparathyroidism is a congenital, hereditary abnormality characterized by hypocalcemia and hyperphosphatemia, as in hypoparathyroidism. It does not, however, respond adequately to parathyroid hormone. The parathyroid glands are present and may be hyperplastic, but the hormones produced by them lack the ability to effect phosphate diuresis. This deficiency is the result of end-organ resistance (1), elaboration of defective hormone (6), or the occurrence of anti-enzymes in the serum or tissues (10). In some of these cases changes in bones have been described that indicate osteitis fibrosa cystica and the production of excessive quantities of a bone-resorbing hormone (8, 9). Most of these patients are short in stature, obese, and mentally retarded, and corneal or lenticular opacity and brachydactylia may be encountered (1, 3, 4, 10, 12). In many instances roentgenograms show calcification or ossification of the skin or subcutaneous tissues (5) or calcification of the basal ganglia or other parts of t...

Portal venipuncture; a percutaneous, trans-hepatic approach.

Conclusion This technic permits relatively easy access to the portal venous system in man. It should afford many opportunities for clinical investigations, including portal venography.

Roentgenographic Manifestations of the Prader-Willi Syndrome

The Prader-Willi syndrome consists of neonatal hypotonia, sexual infancy, obesity, and mental retardation. Five recent cases of this entity are presented to illustrate the roentgen findings, which include abnormalities of the calvarium and sella turcica, small hands and feet with thin cortices and over-tubulated bones, and unequal extremities. Other changes are coxa valga, scoliosis, advanced dental caries, and soft-tissue abnormalities. A review of the literature indicates that this syndrome is more common than generally recognized and that many of these patients had been categorized previously as having other similar conditions.

Shwachman's Syndrome: The Broad Spectrum of Bony Abnormalities1

Two cases are presented and previous reports reviewed to illustrate the wide range of osseous abnormalities associated with the syndrome of pancreatic exocrine insufficiency and bone marrow dysfunction (Shwachmans syndrome). Profound dwarfism with or without metaphyseal chondrodysplasia, abnormal tubulation of bone, focal lack of calcification of the metaphyses, retardation of maturation age, hypoplasia of the middle phalanx of the fifth digits with bilateral clinodactyly and narrowing of the sacrosciatic notches are among the osseous manifestations. Early radiographic recognition of the bony abnormalities of Shwachmans syndrome may facilitate diagnosis and prediction of the other diverse manifestations of this serious disorder.

The potential hazard of enemas in patients with Hirschsprung's disease.

It was recently our tragic experience to have one of our patients, a two-year-old girl with a megacolon, die while receiving a barium enema. The cause of death was not immediately apparent. A survey of the literature was then made and our own files were reviewed in an attempt to throw some light upon the cause of this accident and the frequency with which it happens. As early as 1893 a case was described in an eleven-year-old boy with a history of abdominal distention and constipation from birth who died suddenly during administration of an enema. At autopsy the transverse and descending colon were found to be dilated, gradually narrowing down to a normal distal sigmoid and rectum. The cause of death was thought to be “embarrassment of the enfeebled heart action by the distention of the abdomen” (7). In 1913, Neugebauer (5) reviewed a case described by Nepilly in which a child experienced convulsions and a cold sweat, and died shortly after passing a stool containing fluid that had been administered as an...

Roentgenographic Roentgenographic Features of the Neutrophil Dysfunction Syndromes

The neutrophil dysfunction syndromes comprise a group of rare, often fatal disorders, usually beginning in infancy. Common to all these syndromes is an impairment of the ability of peripheral blood neutrophils (3, 14, 15, 17, 26) and monocytes (12) to kill ingested, opsonized bacteria. Although the syndromes bear diverse patterns of inheritance (14), their clinical manifestations are strikingly similar. A proposed classification by Douglas, Davis, and Fudenberg (14) includes three syndromes whose roentgenographic features are here presented (Table I). Recognition of a specific constellation of these features permits the roentgenologist to assume a key diagnostic role. The roentgenographic findings in the neutrophil dysfunction syndromes are characterized by their great diversity, reflecting involvement of multiple organs. The unusual chronicity of many of the changes may result from the persistence of viable intracellular bacteria within granulomatous lesions. The pulmonary findings include chronic and re...