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Biomedical subjects

J M Gomori

Publications and source records attributed to J M Gomori.

At least 19 recordsLinked to original sources

Giant lateral sinus pericranii. Case report.

A case of giant lateral sinus pericranii, which presented in a patient during early childhood as a soft, collapsible mass and gradually grew until it reached 13 x 9 cm when the patient was 36 years of age, is reported. The patient underwent successful surgery and the lesion was totally excised. The results of diagnostic tests (computerized tomography scanning, magnetic resonance imaging, cerebral angiography, and sinusography) and surgery-related problems are presented and discussed.

Adult

Progressive dystonia with optic atrophy in a Jewish-Iraqi family.

The combination of progressive dystonia and optic atrophy is extremely rare and its morphological, metabolic and genetic basis is unknown. In a family of 9 children (8 males) born to consanguineous Israeli-Jewish-Iraqi parents, we identified four brothers who developed the syndrome at the end of the first decade. Patients had hemi or bilateral dystonia associated with striatal, mainly putaminal, atrophy on CT and MRI, various degrees of optic atrophy, minimal corticospinal tract involvement, normal intelligence and no peripheral nervous system or systemic abnormalities. No causative metabolic defect was identified. None of the several known mitochondrial DNA mutations associated with Leber's hereditary optic neuropathy (LHON) or with LHON with dystonia were detected. Likewise, linkage to the idiopathic torsion dystonia region on chromosome 9q34 was excluded. It is suggested that this in our patients might be due to a yet unidentified genomic, autosomal recessive mutation.

Adolescent

Recurrent abducens nerve palsy caused by dolichoectasia of the cavernous internal carotid artery.

PURPOSE: To describe a 59-year-old patient who had seven recurrent, self-limited episodes of isolated ipsilateral abducens nerve palsy in the previous 4 years. Each episode lasted between 2 and 5 weeks. METHODS: Systemic examination and neuroimaging studies were performed. RESULT: Repeated comprehensive examination failed to demonstrate any ocular or systemic condition apart from a lateral protrusion by dolichoectasia of the posterior portion of the cavernous left internal carotid artery, compressing the posterior left cavernous sinus. CONCLUSION: Numerous self-limited episodes of sixth nerve palsy may be associated with structural abnormalities of the intracranial carotid vasculature.

Abducens Nerve

Thoracic aorta transobturator bipopliteal bypass as eventual durable reconstruction after removal of an infected aortofemoral graft.

A 36-year-old man was referred with aortofemoral graft infection and perigraft duodenal erosion. The aortofemoral graft was removed, and bilateral axillo-superficial femoral grafts were constructed. Recurrent failures of these grafts prompted us to convert to a more-durable reconstruction. A straight graft was anastomosed to the lower thoracic aorta, routed retroperitoneally, and attached to an inverted U-shaped bilateral transobturator bypass graft, which was anastomosed to both above-knee popliteal arteries. After 3 years, the patient has remained well and the grafts are patent. This operation represents a durable in-line reconstruction that avoids all previously infected areas after removal of an infected aortofemoral graft.

Adult

Echocardiographic sign for cerebral ischemia.

We describe a newborn who underwent balloon dilatation of critical aortic stenosis and surgical correction of coarctation of the aorta. Postoperative Doppler echocardiogram revealed diastolic retrograde flow in the distal aortic arch and increased systolic flow as well as diastolic forward flow in the left common carotid artery. Cranial computed tomography suggested increased left middle cerebral artery flow and a subacute infarction with luxury perfusion and damage to the blood brain barrier. Therefore, in the absence of another reason for diastolic "run-off" in the distal aortic arch, this flow pattern may represent the echocardiographic sign for cerebral hyperemia associated with subacute ischemia.

Aortic Coarctation

Utility of relative cerebral blood volume mapping derived from perfusion magnetic resonance imaging in the routine follow up of brain tumors.

It was recently demonstrated that imaging of brain tumors by relative cerebral blood volume (CBV) maps reconstructed from dynamic magnetic resonance (MR) data provide similar diagnostic information compared to positron emission tomography (PET) or 201Tl single-photon emission computerized tomography (201Tl-SPECT) scans. The authors used relative CBV mapping for routine follow-up evaluation of patients with brain tumors and compared its sensitivity to diagnostic MR imaging, 201Tl-SPECT and clinical assessment. Fifty-nine patients were prospectively followed using 191 concomitant studies of dual section relative CBV maps, MR imaging, 201Tl-SPECT, and neurological evaluations. Studies were repeated every 2 to 3 months (median three evaluations/patient). The relative CBV maps were graded as relative CBV 0 to 4, where Grades 3 and 4 are indicative of proliferating tumors (four = rapid leak). There were 44 high-grade and 15 low-grade tumors followed during treatment. During the follow-up period a change in relative CBV grade was observed in 56% of the patients, revealing an increasing grade in 72% of them. The rapid leak phenomenon was detected in 35% of all studies and in 81% of those with a worsening relative CBV grade. Tumor progression was detected earlier by relative CBV maps as follows: earlier than MR imaging in 32% of the studies (earlier by a median of 4.5 months; p < 0.01); earlier than 201Tl-SPECT in 63% (median 4.5 months; p < 0.01), and earlier than clinical assessment in 55% (median 6 months; p < 0.01). In 82% of studies with positive MR imaging but negative 201Tl-SPECT, the lesions were smaller than 1.5 cm. The relative CBV maps clearly delineated the appearance of rapid leak in these lesions. Routine use of relative CBV maps that can be implemented on any high-field MR unit and added to the regular MR evaluation provides useful functional information in patients with brain tumors. When used as an adjunct follow-up evaluation it proved more sensitive than the other modalities for early prediction of tumor growth. It is very sensitive to small regional changes, unlike functional imaging such as PET or SPECT scans. Based on previous experience with 76 regional CBV studies, the authors conclude that regional CBV mapping correlates with active tumor and it may separate enhancing scar and radiation injury from infiltrative tumor. A new effect named the rapid leak phenomenon was also observed; this phenomenon, as identified on the regional CBV maps, correlates with high malignancy.

Adolescent

Spinal echinococcal cyst: treatment using computerized tomography-guided needle aspiration and hypertonic saline irrigation. Case report.

A multilocular extradural cervical spinal hydatid cyst that causes severe spinal cord compression and quadriplegia is relatively rare and difficult to treat. In a patient with this disorder, computerized tomography-guided needle aspiration of the cyst loculations and irrigation using hypertonic saline eliminated the need for emergency surgery and provided complete resolution of the patient's quadriplegia. The subsequent course of the disease was controlled by treatment with albendazole. Magnetic resonance imaging performed 4 months after the procedure demonstrated collapsed cysts and absence of spinal cord compression.

Bone Cysts

Parapharyngeal abscess in children: the role of CT for diagnosis and treatment.

Parapharyngeal abscess (PPA) in children is an uncommon deep neck space infection. Computerized tomography (CT) is an important diagnostic aid. However, CT scan is not specific in differentiating an abscess from cellulitis or inflammatory edema. A retrospective review of eight children with a CT diagnosis of PPA was undertaken. In three patients an abscess was surgically confirmed. In two patients surgical exploration did not reveal an abscess and in three the infection resolved with intravenous antibiotic therapy only. Although CT is a useful diagnostic tool for PPA, surgical intervention should be based mainly on the clinical status of the patient and the non-responsiveness to i.v. antibiotic therapy.

Anti-Bacterial Agents

Use of 201Thallium brain SPECT, image registration, and semi-quantitative analysis in the follow-up of brain tumors.

OBJECTIVE: To assess the use of 201Thallium SPECT and of Thallium (Tl) uptake indices in the detection and follow-up of cerebral tumors. MATERIAL AND METHODS: Two-hundred eighty 201Tl, 135 99mTc-HMPAO scintigraphies and 280 MRI studies were acquired in 135 patients with cerebral tumors. Three types of Tl uptake indices were calculated by establishing the ratio of the lesion activity to an homologous ROI (I1), the contralateral hemisphere (I2), and the contralateral scalp (I3). Intermodal coregistration between Tl-SPECT and MRI when used, was performed using Pietrzyk visual interactive method. RESULTS: Tl uptake indices showed mean values of 2.0 +/- 0.5 (I1); 1.77 +/- 0.57 (I2) and 1.11 +/- 0.40 (I3) in positive scans, and of 1.05 +/- 0.22 (I1), 0.88 +/- 0.22 (I2) and 0.50 +/- 0.15 (I3) in negative scans, respectively. A linear combination of the two less correlated indices lead to a more powerful discrimination between positive and negative studies. Longitudinal follow-up studies in the same patients showed good concordance between the index values and the course of the disease. CONCLUSION: The use of Tl uptake indices improves the accuracy of Tl-SPECT in the follow-up of brain tumors.

Adolescent

Antiphospholipid syndrome manifested by ischemic stroke in a patient with Crohn's disease.

Cerebrovascular accidents are rare but well documented in patients with Crohn's disease. Up to 10% of hypercoagulable state manifestations reported in association with inflammatory bowel disease are ischemic strokes. However, no clear mediating factor has thus far been suggested. A 44-year-old woman with Crohn's disease for 25 years developed a left temporal stroke associated with anticardiolipin antibody and lupus anticoagulant suggesting antiphospholipid syndrome. A thorough evaluation did not reveal any other risk factor for ischemic stroke. No possible sources of emboli were found in the carotids and heart, and no deficiencies of protein C and activated protein C, protein S, and anti-thrombin III leading to hypercoagulable state were present. There may be a possible association between antiphospholipid syndrome and hypercoagulable state in Crohn's disease.

Adult

Treatment of secondary progressive multiple sclerosis with the immunomodulator linomide: a double-blind, placebo-controlled pilot study with monthly magnetic resonance imaging evaluation.

Linomide (quinoline-3-carboxamide) is a synthetic immunomodulator that increases the natural killer cell activity. We previously demonstrated that linomide effectively inhibited the clinical and histopathologic signs of acute and chronic relapsing experimental autoimmune encephalomyelitis. We report a double-blind, placebo-controlled study to evaluate tolerability and to obtain preliminary indications of the clinical efficacy of linomide on secondary progressive MS. Thirty patients suffering from clinically definite and laboratory-supported secondary progressive MS, with an expanded disability status scale (EDSS) of 3.0 to 7.0, were included in this study. Patients were treated daily with linomide (2.5 mg) or placebo orally and were followed up for side effects and changes in their neurologic status; monthly MRI scans were taken throughout the treatment period. Twenty-four patients completed at least 6 months of treatment. Mild to moderate side effects, including muscle pains, arthralgia, and edema, were present in 11 of the 15 patients receiving placebo and in 13 of the 15 patients treated with linomide. At 24 weeks, the mean shift in EDSS was +0.272 +/- 0.156 in the placebo group versus -0.166 +/- 0.167 in the linomide group (p = 0.0451). The percentage of patients with evidence of "activity" on their MRI (new, enlarging, or new gadolinium diethylenetriaminepentaacetic acid [Gd-DTPA]-enhancing lesions) throughout the treatment period was 75% in the placebo group and 33% in the linomide group (p = 0.0205). The mean total number of new Gd-DTPA-enhancing lesions per MRI scan for the same period was 0.42 +/- 0.143 in the placebo group and 0.19 +/- 0.114 in the linomide group (p = 0.0387). In this study, linomide proved to be safe and well tolerated in patients with secondary progressive MS. In addition, our results indicate that linomide tends to inhibit the progression of the disease, especially preventing the appearance of new active lesions in the MRI scans. Based on these results, two multicenter phase III trials are currently under way in the United States and in Europe and Australia.

Adjuvants, Immunologic

Clinical significance of diffuse dural enhancement detected by magnetic resonance imaging.

This study was performed to determine the clinical significance of diffuse dural enhancement (DDE) detected by magnetic resonance (MR) imaging and to typify enhancing patterns related to inflammatory or metastatic causes. The authors retrospectively evaluated the clinical, imaging, and laboratory characteristics of 20 consecutive patients with DDE. Those with DDE and an underlying neoplastic disease (13 patients) were compared to 11 consecutive patients with cytological evidence of neoplastic leptomeningeal metastasis evaluated by MR imaging. The DDE was often associated with an underlying malignancy (13 (65%) of 20 patients) but it coexisted with leptomeningeal metastasis in only one patient. Skull metastases were evident in 10 (77%) of 13 patients and cranial nerve palsies in six (46%) of 13. Other causes of DDE were related to cerebrospinal fluid (CSF) leak or shunting (five (25%) of 20), with or without symptoms of intracranial hypotension, and to dural sinus thrombosis and pachymeningitis. Dural biopsies obtained in two patients with DDE showed a narrow rim of granulation-like tissue adherent to the dural surface facing the inner skull table. Magnetic resonance subtraction, diffusion, and perfusion studies revealed unique characteristics in patients with metastatic causes as compared to those with DDE secondary to CSF leak. None of the patients with proven leptomeningeal metastasis had DDE, but four of them presented with focal dural enhancement and two displayed apparent leptomeningeal enhancement. The findings indicate that DDE is not a radiographic hallmark of leptomeningeal metastasis in spite of the similarities in clinical manifestations (for example, headache and cranial polyneuropathy). Nonetheless, DDE is most frequently associated with metastatic malignancies and particularly with skull metastases and CSF leak. Special MR techniques can discern the underlying cause and elucidate the disparity in the pathophysiological mechanisms leading to DDE.

Aged

Spontaneous involution of an intra-axial brain stem lesion: a case report.

This report describes the spontaneous involution of an intrinsic brain stem lesion whose clinical and MRI characteristics strongly suggest a tumor etiology. Nonoperative experiences with central nervous system tumors have shown that they may sometimes exhibit prolonged periods of dormancy. There are several reports of 'disappearing' CT lesions which have all been inflammatory, infective, or immunological. No histologically proven tumor has been shown to spontaneously involute, nor have 'disappearing' lesions been described for MRI.

Brain Neoplasms

Superficial hemosiderosis of the central nervous system.

Superficial hemosiderosis (SH) of the CNS is a rare disease caused by repeated subarachnoid hemorrhage, with progressive superficial siderosis of the CNS. We report a patient with SH whose clinical picture was marked by progressive gait ataxia, hearing loss, dysarthria, and recurrent episodes of hemifacial spasm. Iron and ferritin levels in the CSF were significantly higher than in a control group of patients. Six month's treatment with the iron-chelating agent trientine dihydrochloride led to clinical improvement, with a concomitant reduction of CSF iron level. We suggest that, in addition to magnetic resonance imaging findings, CSF levels of iron and ferritin should be used as diagnostic criteria for SH, as well as to estimate the efficacy of iron chelation treatment.

Astrocytoma

Magnetization transfer: theory and clinical applications in neuroradiology.

Magnetization transfer, a new technique for improving image contrast in magnetic resonance (MR) imaging, is based on application of off-resonance radio-frequency pulses and observing their effects on MR images, as well as measuring the signal intensity with and without application of the pulses (ie, magnetization transfer ratio [MTR]). MTRs can be used to detect changes in the structural status of brain parenchyma that may or may not be visible with standard MR techniques. Use of MTRs may allow subcategorization of multiple sclerosis lesions into those with very low MTR (demyelinated lesions) and slightly decreased MTR (edematous lesions). In cases of wallerian degeneration, use of MTRs appears to allow reliable detection of changes undetectable with MR imaging or even light microscopy. In cases of infection with human immunodeficiency virus, MTRs seem to indicate that the macromolecular structure of white matter remains intact until relatively late in the course of disease. In cases of metastatic disease, MTRs of brain lesions indicate structural changes beyond the extent of the lesions seen on standard MR images. These findings may be due to chronic edema, myelin loss, and perhaps previous undetected tumor. In addition to being a new method of providing contrast, the magnetization transfer technique enables semi-quantitative, reproducible characterization of tissue and pathologic entities, which could substantially improve the specificity of MR imaging.

Brain Diseases

Acute postpartum inflammatory sacroiliitis. A report of four cases.

We report four patients with unilateral postpartum sacroiliitis presenting with agonising unilateral pain, an elevated ESR, elevated alkaline phosphatase levels, leucocytosis and positive bone scans. The diagnosis of a non-infectious inflammatory cause was supported by the postpartum onset, the response to non-steroidal anti-inflammatory drugs, negative aspiration cultures in two cases and the lack of changes in the sacroiliac joints on long-term follow-up radiographs.

Acute Disease