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Biomedical subjects

A C Mamourian

Publications and source records attributed to A C Mamourian.

At least 19 recordsLinked to original sources

Association between size of the lateral ventricle and asymmetry of the fornix in patients with temporal lobe epilepsy.

PURPOSE: Our goal was to determine whether the size of the lateral ventricle influences asymmetry of the fornix in patients with mesial temporal sclerosis. METHODS: The cross-sectional area of the lateral ventricle was measured along with the thickness of the fornix in 18 patients with pathologically proved mesial temporal sclerosis. Two additional patients with ventricular asymmetry and seizures but without mesial sclerosis were also studied. RESULTS: Ten of 18 patients with mesial temporal sclerosis had equally sized ventricular areas or less than a 25% difference between the two sides. In seven of these 10, the left and right fornices were equal; in the other three patients, the fornix was thinner on the side of mesial sclerosis. Among the eight patients with ventricular area differences greater than 25%, the larger ventricle was on the side of mesial sclerosis in five cases. In all of these, the ipsilateral fornix was thinner. In three of eight cases, the contralateral ventricle was larger; in two, the left and right fornix were equal; and in one, the fornix contralateral to the side of mesial sclerosis was smaller. The greatest difference in fornix thickness was noted in patients with ventricular asymmetry greater than 40%, regardless of side or presence of mesial temporal sclerosis. CONCLUSION: The size of the lateral ventricle may influence the apparent thickness of the fornix.

Cerebral Ventricles

Colloid cyst of the third ventricle: sometimes more conspicuous on CT than MR.

We present two cases of surgically proved colloid cysts that were more apparent on CT scans than on MR images. These cysts, while hyperdense on CT scans, were nearly isointense with brain on multiple MR sequences. This relative lack of visibility represents a potential pitfall when imaging a patient with headache.

Adult

Delayed sequelae after acute overdoses or poisonings: cranial neuropathy related to ethylene glycol ingestion.

A 31-year-old woman came to the hospital with breathlessness, confusion, and a refractory anion gap metabolic acidosis; acute renal failure subsequently developed. Her blood ethylene glycol concentration was 390 mg/L, and she was treated with an intravenous ethanol infusion and hemodialysis. During the tenth and eleventh day after admission bilateral seventh cranial nerve paralysis developed, as well as bilateral dysfunction of cranial nerves II, V, VIII, IX, X and XII. Magnetic resonance imaging of her head showed gadolinium enhancement of the fifth cranial nerve bilaterally and a communicating hydrocephalus. Over the subsequent 11 months she recovered full function of her cranial nerves V, VII, IX, X, and XII, and she had subjective clinical improvement to baseline function in cranial nerves II and VIII. This case serves to introduce a discussion of agents that cause delayed complications after their acute toxic ingestion.

Adult

Aqueduct compression from venous angioma: MR findings.

Vascular compression as the cause of aqueductal stenosis is rare. In a 16-year-old girl with hydrocephalus, MR imaging provided evidence of aqueductal stenosis caused by a venous angioma in the tectum and midbrain. This indicates the usefulness of MR imaging for the evaluation of obstructive hydrocephalus.

Adolescent

Glutaric aciduria: improved MR appearance after aggressive therapy.

Advances in understanding the metabolic abnormalities which cause glutaric aciduria allow biochemical diagnosis on the basis of deficient enzyme and the potential for therapy. Brain abnormalities associated with this inborn error of metabolism have been demonstrated with CT and MR. The findings typically described are atrophy of the fronto-temporal regions with large insular cisterns and diffuse white matter hypodensities. We present a patient with glutaric aciduria, confirmed by enzymatic assay, who had these findings on CT and MR examination. Repeat imaging demonstrated significant improvement after dietary therapy and aggressive prevention of catabolism during febrile illness.

Amino Acid Metabolism, Inborn Errors

MR of giant arachnoid granulation, a normal variant presenting as a mass within the dural venous sinus.

We report three cases of masses within the cerebral dural venous sinuses shown with either MR or angiography. The dural venous sinuses of 10 patients without known venous disease were examined at autopsy. In two patients, three giant arachnoid granulations were identified. On the basis of the literature and our limited autopsy series, we suggest that these lesions identified at imaging are giant arachnoid granulations, normal variants of no known clinical significance.

Adult

The asymmetric mamillary body: association with medial temporal lobe disease demonstrated with MR.

PURPOSE: To determine whether mamillary body atrophy is caused by deafferentation of the mamillary body in patients with mesial temporal sclerosis. METHODS: We studied 36 patients with thin-section MR to assess mamillary body symmetry. These patients included 10 control subjects without seizures and 26 patients with a history of seizures. Thin-section T1 scans were available for all cases. The patients with epilepsy underwent axial and coronal T2 scans as well. RESULTS: In five of eight cases with prior medial temporal lobe resection for intractable epilepsy, there was evidence of unilateral mamillary body atrophy ipsilateral to the resection. Similar findings were evident in three of six patients with MR findings of mesial temporal sclerosis without surgery. Two patients with medial temporal stroke or tumor also had ipsilateral mamillary body atrophy. CONCLUSION: These findings provide support for the proposed mechanism of mamillary body atrophy caused by prior medial temporal lobe injury.

Adult

Dystonia, hyperintense basal ganglia, and high whole blood manganese levels in Alagille's syndrome.

Hyperintensity of the globus pallidus on T1-weighted magnetic resonance imaging (MRI) has been reported in patients with chronic liver disease. This abnormality has been associated with the severity of liver disease and tremor, but its cause is unknown. Similar MRI signal abnormalities have been reported in experimental models of manganese neurotoxicity. This case report describes a child with Alagille's syndrome and end-stage liver disease who developed dystonia and tremor associated with an elevated whole blood manganese level and symmetric hyperintense globus pallidi and subthalamic nuclei on T1-weighted but not T2-weighted MRI. Liver transplantation was performed; 2 months later, neurological function was improved, manganese levels were normal, and the MRI signal abnormality had completely resolved. This child had neurological findings described in manganese neurotoxicity with compatible laboratory and radiological findings. Manganese is excreted by the liver in bile, and toxicity may have resulted from the inadequacy of this mechanism, subsequently corrected by liver transplantation.

Alagille Syndrome

Symptomatic manganese neurotoxicity in a patient with chronic liver disease: correlation of clinical symptoms with MRI findings.

Hyperintense symmetric pallidal lesions have been described in chronic hepatic failure. Similar lesions are reported in experimental models of manganese neurotoxicity. We describe an 8-year-old girl with chronic hepatic failure and dystonia in association with an elevated whole blood manganese level and symmetric hyperintense pallidal lesions on magnetic resonance imaging. After hepatic transplantation, her symptoms and signs resolved with normalization of magnetic resonance imaging and the whole blood manganese suggesting that in chronic hepatic failure, the pallidal lesions may be secondary to manganese deposition.

Alagille Syndrome

Long-term MRI changes in brain after pediatric open heart surgery.

We performed magnetic resonance imaging (MRI) on the brain and neurologic examinations on 23 children after open heart surgery for congenital heart disease. Twenty children also had psychometric assessments. Examinations were performed at a mean age of 66 months (range, 26 to 180 months). Age at operation was less than 1 month in 43% and more than 6 months in 45%. Abnormal scans were found in 17 (74%) and showed diffuse findings consistent with hypoxic-ischemic encephalopathy, with or without areas of cortical infarction; focal cortical infarction alone; and (in one patient) callosal agenesis and abnormal neuronal migration. Normal IQ and neurologic examinations were found in all six of those who had a normal MRI, and five of six children with changes consistent with focal cortical infarction without diffuse change had a normal neurologic examination. Cerebral palsy and mental retardation was common in the group with diffuse abnormality (in eight of nine children), and this was more likely to occur in those who underwent prolonged (> 45 minutes) hypothermic circulatory arrest and operation during early infancy (P = .004). Focal cortical findings without diffuse changes were more likely in those who underwent open heart surgery without hypothermic circulatory arrest and were older than 6 months at operation, and these children were less likely to have frank neurodevelopmental sequelae. Thus, in our population, focal cortical lesions were common after open heart surgery, and, in addition, diffuse brain abnormality on MRI plus neurologic sequelae were common after prolonged hypothermic circulatory arrest.

Adolescent

Neonatal pontomedullary disconnection with aplasia or destruction of the lower brain stem: a case of pontoneocerebellar hypoplasia?

We report a neonate who presented with marked hypotonia and absent suck reflex. MR demonstrated complete absence of the pons as well as absence of a basilar artery flow void. Our case exhibits features similar to those described in previous reports of pontoneocerebellar hypoplasia, but with a more severe degree of pontine involvement. The associated vascular findings suggest a vascular insult to the brain stem as the cause.

Basilar Artery

Handgun control.

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Firearms

Urea cycle defect: a case with MR and CT findings resembling infarct.

A 2 1/2 year old girl was admitted to the hospital because of recurrent vomitting, impaired consciousness, and hyperammonemia. MR and CT findings resembled an infarct, but she was found to have a defect in the urea cycle, partial ornithine transcarbamylase deficiency.

Amino Acid Metabolism, Inborn Errors