Herzlia Hadar

Brain imaging in late‐onset GM2 gangliosidosis

We describe brain CT and MRI characteristics of 10 patients with late-onset GM2 gangliosidosis. Cerebellar atrophy, particularly of the vermis, was a prominent feature in all patients with normal-appearing cerebral hemispheres. The severity of these findings did not correlate with the age of onset, disease duration, severity of neurologic impairment, or mode and distribution of the various clinical presentations. In particular, no cerebral abnormality was found by neuroimaging in seven patients with intellectual decline and in six patients with recurrent psychosis, while prominent cerebellar atrophy was present in the only patient who was free of cerebellar signs.

Widespread dissemination of gelfoam particles complicating occlusion of renal circulation.

Embolization of renal circulation by Gelfoam particles in a thirty-two-year-old man with renal carcinoma was followed by retrograde regurgitation of the injected particles upstream, with resultant occlusion of the celiac trunk and the superior mesenteric artery. This was followed by a stormy clinical course, which the patient survived. Several possible explanations are offered for the occurrence of this complication.

Computed tomography of renal agenesis and ectopy

The position and shape of the organs normally adjacent to the kidney were evaluated by computed tomography in seven patients with renal agenesis and in two patients with unilateral renal ectopy. The major findings were displacement of colonic flexures, small intestine loops, duodenum, spleen, and tail of the pancreas. The shape and position of the adrenal gland was found abnormal in cases with left-sided anomaly. Genital abnormalities such as absent seminal vesicle were frequently encountered. The presence of abnormally shaped adrenal glands in patients with left renal agenesis and ectopy is a new diagnostic radiologic sign for these conditions.

Spontaneous extravasation of urine in chronic ureteric obstruction.

Two cases of spontaneous extravasation of urine in chronic ureteric obstruction are presented. In 1 case the long-standing partial ureteric obstruction was caused by enlarged lymphomatous glands, and extravasation was followed by a perinephric abscess. In the second case, believed to be unique, there was a moderate chronic ureteric obstruction post irradiation, and extravasation was induced by a sudden rise in intra-abdominal pressure due to vomiting.

Pitfalls in the follow-up of cervical and mediastinal goitres: role of CT imaging

Four patients with cervical and mediastinal goitres were described. Routine investigation in these patients, including chest X-rays, oesophageal and tracheal X-rays and scintigraphy of the thyroid gland, failed to show significant changes over the years, or underestimated the true extent of the goitres. Three of the goitres caused severe mediastinal compression, evident only on computed tomography (CT) imaging, and requiring urgent surgery. The pros and cons of different diagnostic modalities for imaging cervical and mediastinal goitres are discussed, stressing the importance of CT imaging as exemplified in the cases presented.

Primary CT diagnosis of gastrointestinal masses

Four cases of gastrointestinal masses (3 tumorous and 1 inflammatory) diagnosed primarily by CT, are presented. It is stressed that the radiologist should be familiar with the CT appearance of these conditions. It is recommended that in most cases barium studies and endoscopy should follow. Case 4 is unique because it represents the rare possibility of CT diagnosis of a tumor of the bowel where barium studies and endoscopy may have failed.

Computed tomographic diagnosis of abdominal aortic aneurysm

Diagnosis of abdominal aortic aneurysms has been facilitated by the use of computed tomography (CT). This is illustrated by four cases, presented here, two of which were diagnosed by angiography and CT, and two having been operated upon on the basis of CT diagnosis alone. We feel that CT can replace angiographic studies by supplying the surgeon with all the pre-operative details needed. CT is more accurate than angiography, since the latter may underestimate the true diameter of aneurysms, or can even give false-negative results. With CT it is possible to detect signs of rupture or pre-rupture states of aneurysms that have not been suspected on angiographic or clinical grounds.

Thickened renal fascia—A sign of retroperitoneal pathology☆

Abstract Twenty-two cases of thickened renal fascia diagnosed by i.v. urography and/or CT scan are summarized. In 15 cases the pathological process was renal and in the other 7 it was non-renal. The anatomy of the retroperitoneal compartments has been reviewed. The authors wish to stress that thickened renal fascia is not only a valuable sign in locating renal pathology, but is also very useful in diagnosing retroperitoneal pathology

Renal sinus lipomatosis: differentiation from space-occupying lesion with aid of computed tomography.

Forty-two cases of renal sinus lipomatosis were diagnosed by intravenous urography and nephrotomography. The differential diagnosis from a malignant process necessitated selective renal angiography in 9 cases. With the aid of computed tomography (CT), an invasive technique such as renal angiography appears to be unnecessary in the differential diagnosis of renal sinus lipomatosis. CT seems to be more accurate since it revealed a concomitant cyst in 2 of 4 cases.

Muscle computed tomography features of motor neuron disease in late-onset GM2 gangliosidosis.

Late‐onset GM, gangliosidosis, a rare inherited neuronal storage disease, is characterized by a vanety of clinical manifestations. The common clinical picture comprises neuromuscular, spinocerebellar, extrapyramidal, cognitive, and psychiatric abnormalities. Details of the extent of muscle involvement have never been reported. Eight patients with this syndrome were evaluated for the existence and extent of motor neuron disease using routine electrodiagnosis and systematic evaluation of skeletal musculature by computed tomography. Motor neuron disease was present in each and every patient regardless of the clmical manifestations and to a degree beyond that suspected on neurological examination. Muscle imaging disclosed a diffuse wasting and fatty replacement of muscles with predilection of pelvic and thigh muscles, and especially the quadriceps group. It seems that progressive motor disability in this syndrome IS mainly due to motor neuron disease, as manifested by muscle atrophy, which can be easily demonstrated by muscle computed tomography.