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Biomedical subjects

M T Iba-Zizen

Publications and source records attributed to M T Iba-Zizen.

At least 19 recordsLinked to original sources
PubMed1995

Clinical spectrum of CADASIL: a study of 7 families. Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an inherited arterial disease of the brain recently mapped to chromosome 19. We studied 148 subjects belonging to seven families by magnetic resonance imaging and genetic linkage analysis. 45 family members (23 males and 22 females) were clinically affected. Frequent signs were recurrent subcortical ischaemic events (84%), progressive or stepwise subcortical dementia with pseudobulbar palsy (31%), migraine with aura (22%), and mood disorders with severe depressive episodes (20%). All symptomatic subjects had prominent signal abnormalities on MRI with hyperintense lesions on T2-weighted images in the subcortical white-matter and basal ganglia which were also present in 19 asymptomatic subjects. The age at onset of symptoms was mean 45 (SD [10-6]) years, with attacks of migraine with aura occurring earlier in life (38.1 [8.03] years) than ischaemic events (49.3 [10.7] years). The mean age at death was 64.5 (10.6) years. On the basis of MRI data, the penetrance of the disease appears complete between 30 and 40 years of age. Genetic analysis showed strong linkage to the CADASIL locus for all seven families, suggesting genetic homogeneity. CADASIL is a hereditary cause of stroke, migraine with aura, mood disorders and dementia. The diagnosis should be considered not only in patients with recurrent small subcortical infarcts leading to dementia, but also in patients with transient ischaemic attacks, migraine with aura or severe mood disturbances, whenever MRI reveals prominent signal abnormalities in the subcortical white-matter and basal ganglia. Clinical and MRI investigations of family members are then crucial for the diagnosis which can be confirmed by genetic linkage analysis. The disease is probably largely undiagnosed.

Adult↗
PubMed1995

Autosomal dominant migraine with MRI white-matter abnormalities mapping to the CADASIL locus.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant cerebral arteriopathy mapped to chromosome 19 and characterized mainly by recurrent subcortical ischemic strokes and extensive white-matter signal abnormalities (WMAs) on magnetic resonance imaging. Other clinical features include migraine attacks and progressive subcortical dementia. Herein, we describe several members of the same family who suffered migraine attacks, mostly with aura, associated with WMAs, segregating with an autosomal dominant pattern of inheritance. One individual had a progressive subcortical dementia with similar WMAs. Although ischemic stroke, one of the hallmarks of CADASIL, was not present in this family, we hypothesized that the present disorder resulted from an alteration of the CADASIL gene. Genetic linkage analysis, using four chromosome 19 markers spanning the CADASIL locus, supports this hypothesis.

Adult↗
PubMed1994

Pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroid alone: first case report.

We report the first case of pseudotumoral lymphocytic hypophysitis successfully treated by corticosteroids without surgery. A 27-year-old woman had been monitored for chronic headache 13 months after giving birth, associated with amenorrhea and galactorrhea. Cranial magnetic resonance imaging revealed a markedly enlarged pituitary gland with a suprasellar extension; the only biochemical abnormality was a mild hyperprolactinemia. Because of a putative diagnosis of prolactinoma, bromocriptine was prescribed at a dose of 5 mg daily, soon followed by the transitory appearance of menstruation. Two years later, panhypopituitarism was present and was revealed by acute adrenal insufficiency. Magnetic resonance imaging revealed that the pituitary mass was the same as previously described, but hormonal investigation showed evidence of complete hypopituitarism and no hyperprolactinemia. Nuclear antibodies were negative as well as other autoantibodies. Human leukocyte antigen serological Class II typing was DR3/DR4. Lymphocytic hypophysitis was then suspected; in the absence of visual complication and because this patient refused surgery, corticosteroids were attempted at a daily dose of 60 mg of prednisone for 3 months, progressively decreased for the next 6 months. Under this treatment, a gradual recovery of all pituitary hormones was observed and magnetic resonance imaging showed a reduction of two-thirds in pituitary mass. Five months after the end of corticoid treatment, our patient relapsed with panhypopituitarism and an increase of pituitary volume. She underwent steroid treatment, and a biopsy was performed and confirmed the diagnosis of autoimmune hypophysitis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1991

The commissuro-mamillary plane in MRI of the brain (preliminary communication).

MRI sections of the brain in the coronal plane through the line joining the anterior commissure and the mamillary bodies display the constituent parts of the basal forebrain. The visualisation of the septal nuclei and the anterior columns of the fornix show the importance of this plane in the study of behaviour disorders and amnesic syndromes.

Corpus Striatum↗
PubMed1991

Cognitive function in adult adrenoleukodystrophy: comparison with leukoaraiosis and multiple sclerosis.

Cognitive evaluation of 6 cases of adult adrenoleukodystrophy (ALD) included in a brain magnetic resonance (MR) study are reported: 2 males with adrenomyeloneuropathy and 4 women heterozygous for ALD. Cognition was normal in 4 and MR scan in 2 of them. In the 2 others, there were mild modifications of the white matter. One patient suffered of visual retention disturbances with abnormalities of the white matter in MR scan. In the last, cognitive decline was observed; MR scan showed atrophy of cortex and corpus callosum and periventricular high signal areas. Comparison with leukoaraiosis in healthy adults and with multiple sclerosis suggests that there is probably a relationship between cognition and extension of brain MR abnormalities. Time of appearance and frequency of cognitive dysfunction might be explained by the natural history of each of these diseases.

Adrenoleukodystrophy↗
PubMed1991

Autosomal dominant syndrome with strokelike episodes and leukoencephalopathy.

BACKGROUND AND PURPOSE: We conducted a prospective survey of a family presenting a new syndrome characterized mainly by recurrent strokelike episodes and neuroimaging evidence of leukoencephalopathy. SUMMARY OF REPORT: Forty-five members of a single family were studied clinically and with magnetic resonance imaging. Nine had strokelike episodes, including transient ischemic attacks, and minor or major strokes starting between the fourth and sixth decades, with neuroimaging evidence of small, deep infarcts and a widespread white matter disorder. Other symptoms included migraine (three), dementia (two), epilepsy (one), and hearing loss (one). In some patients, we found various immunologic anomalies and muscular lipidosis without ragged-red fibers. Eight other family members were clinically normal, but had identical neuroimaging signs of leukoencephalopathy. No abnormality was detected in the 28 other members of the family examined. Extensive investigations failed to reveal any known cause of cerebral ischemia. CONCLUSIONS: There appears to be a new syndrome in this family that is characterized by recurrent subcortical strokelike episodes, leukoencephalopathy, immunologic anomalies, muscular lipidosis, and an autosomal dominant pattern of transmission.

Adult↗
PubMed1990

The commissuro-mamillary plane in MRI of the brain (preliminary communication)

MRI sections of the brain in the coronal plane through the line joining the anterior commissure and the mamillary bodies display the constituent parts of the basal forebrain. The visualisation of the septal nuclei and the anterior columns of the fornix show the importance of this plane in the study of behaviour disorders and amnesic syndromes.

Brain↗
PubMed1990

[The dynamic study of the human body using MRI].

This work deals with dynamic anatomy of human body explored by magnetic resonance imaging (MRI). Recent development of MRI pulse sequences allow very fast data acquisition. Using an 1,5 Tesla system (General Electric--CGR, Signa), different body segments have been explored, in two different ways. The first approach consisted in a cinematographic display of anatomical sections of an articulation obtained in different positions. The result shows the movement of the articulation. The second approach consisted in the examination of a single slice several times, with cardiac synchronization ("gating"). the result is the evaluation of phase desynchronization, measuring the flow directions and the velocity of circulating fluids. The results are shown as a video tape with commentary. It starts with the joints: the ankle, the knee, the wrist, the hand, the cervical spine, de cervico-occipital junction, the temporo protrusion during deglutition and the ocular movements (normal and pathological), according to different planes, are successively proposed. Finally, flow kinetics are shown: cardiac movements and supra-aortic arterial flow, the intracranial cerebro-spinal fluid circulation, in normal and pathologic (intra-cranial hypertension) cases. The exposition is concluded by showing a work in progress on urine flow during micturition.

Cerebrospinal Fluid↗
PubMed1990

[Eye movements: initial dynamic approach in high resolution magnetic resonance imaging].

New MR softwares allow a dynamic display of different fonctionnal anatomical regions. This work is a first attempt to visualize the ocular movements. A video tape constitute the support of our results. Technically, the work is performed on a Signa 1,5 T GE CGR System. During each MR acquisition, the subject is asked to fix his gaze successively on each spot of a scale set inside the bore of the magnet. Twenty four images are performed to show the entire lateral course of the globes. The examination time is about half an hour. The video display show the movements of agonist and antagonist muscles, the eyeballs and the optic nerves displacements, in the axial neuro-ocular plane (NOP). Different clinical applications of this new method are discussed (muscular surgery...).

Eye Movements↗
PubMed1989

Dynamics of the junction between the medulla and the cervical spinal cord: an in vivo study in the sagittal plane by magnetic resonance imaging.

Sagittal sections of the brain-stem made by MRI reveal differences in the angle formed by the medulla and the cord. In order to study the normal mobility of this region of the CNS during flexion and extension of the head, sagittal MRI studies were made in the sagittal plane in 18 young volunteers. The volunteers were in dorsal decubitus with the cervical spine first flexed and then extended, with the movement localized to the cranio-cervical junction as far as possible. T1-weighted sequences were used, with body coils in 16 cases and surface coils in two. Measurements were related to global cranio-cervical range of movement, movement at the cranio-cervical junction and spino-medullary movement. Variations in the depth of the free space in front of the medulla, pons and spinal cord during movement were also noted. We also checked for downward shift of the lower part of the 4th ventricle and modification of the shape of the ventricle during flexion-extension. The global range of cranio-cervical movement was between 31 and 100 degrees (average 63 degrees). The range between the cranium and C1C2 was 4 to 39 degrees (average 19 degrees) and the spino-medullary range was from 1 to 32 degrees (average 14 degrees). During flexion, the free space narrowed in front of the pons 11 times, in front of the medulla 14 times and in front of the cervical cord 11 times. There was a downward shift of the lower part of the 4th ventricle during flexion in 4 cases but no change in shape was noted. Though this study is open to criticism from several aspects, it may be concluded that variations of the spino-medullary angle in the sagittal plane during flexion-extension do occur, that they are closely correlated with movements at the cranio-cervical junction, moves forward during flexion.

Adult↗
PubMed1989

[Diffuse retinoblastoma--apropos of a case].

We report a case of diffuse retinoblastoma in a 6 years old boy. The diagnosis should be suspected in front of the very evocative clinical picture itself. The aspect of the tyndall phenomenon, and of the convex pseudo-hypopyon, with iris pearls and nodules, together with a peripheral, imprecisely limited, retinal mass, non calcified on ultrasonographic examination, are typical. Aqueous paracentesis shows increased levels of Lactic Dehydrogenase (LDH), but most of all the cytologic examination confirms the diagnosis. The only treatment is enucleation. The clinical picture which may simulate uveitis, especially as this particular type of retinoblastoma has a late onset.

Child↗
PubMed1989

[Retrochiasmatic lesions in multiple sclerosis. Demonstration by visual evoked potentials. Correlation with magnetic resonance imaging].

Monocular stimulation of each visual hemifield can show an interhemispheric asymmetry of VEP. Validity of this test needs a reproducibility of responses and exclusion of stimulation induced by eye movements. In a prospective study of 22 MS cases, it appeared that interhemispheric asymmetry was a criterion of dissemination is space and had a good diagnostic value: MS became clinically definite in 10/12 cases; in 10 other cases in which a correlative MRI-VEP study was possible, there were disseminated high signal areas in T2 weighted sequences on hemispheric MRI. In 7/10 cases, these areas were located on retrochiasmatic visual pathways. With MRI, VEP are the most performant tests for early diagnosis in MS. Technical progress will improve its fiability. Prospective correlative clinical, electrophysiological and MRI studies are necessary on a larger number of MS patients.

Adult↗
PubMed1988

Fetal curarization for prenatal magnetic resonance imaging.

Fetal magnetic resonance (MR) imaging was performed at 33 weeks of gestation for investigation of a posterior fossa abnormality found at ultrasound screening. Fetal movements were abolished by vecuronium injected under ultrasound guidance into the umbilical vein. MR images showed atrophy of the left cerebellar lobe with cisternal dilatation. These were confirmed postnatally by CT scan.

Adult↗