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M Savoiardo

Publications and source records attributed to M Savoiardo.

At least 55 records · Page 3Linked to original sources

Magnetic resonance imaging in progressive supranuclear palsy and other parkinsonian disorders.

High field intensity MRI may demonstrate signal abnormalities consistent with deposits of iron or other paramagnetic substances in several extrapyramidal disorders. Hallervorden-Spatz disease was the only disorder widely known to have iron deposits in the pallidum, that are now easily demonstrated in vivo by MRI. However, lower field intensity MRI may also demonstrate characteristic findings. In progressive supranuclear palsy, definite atrophy of the midbrain and of the region around the third ventricle is seen in slightly more than half of the cases. Minimal signal abnormalities are sometimes seen in the periaqueductal region, but MRI studies remain of little help in establishing the diagnosis of the disease. Asymmetric atrophy in the parietal regions is seen in corticobasal degeneration, as expected from pathological studies. Minimal alterations may be seen in the substantia nigra in Parkinson's disease. The most interesting MRI findings are observed in multiple system atrophies. Variable abnormal signal intensities, depending on the field intensity, are visible in the putamen in striatonigral degeneration and in Shy-Drager syndrome; in this latter condition the abnormalities are due to its striatonigral degeneration component. Atrophy of the pons, middle cerebellar peduncles, and cerebellum, and signal abnormalities in a characteristic distribution are visible in olivopontocerebellar atrophy. A combination of these posterior fossa abnormalities and putaminal alterations may confirm the involvement of the cerebellar and extrapyramidal systems in multiple system atrophies.

Atrophy↗

Double cortex syndrome: electroclinical study of three cases.

We describe three female patients (aged 10, 11 and 21 years) with a Magnetic Resonance appearance of band heterotopia, a diffuse neuronal migration disorder, also known as double cortex syndrome. The clinical picture was characterized by the association of epilepsy and mental retardation in all three cases, as has been previously described in patients with double cortex syndrome. The epileptic syndrome (Lennox-Gastaut syndrome in one case, and symptomatic partial epilepsy in the other two), the response to medical treatment, and the severity of mental retardation were markedly different in the three patients. No clear-cut relationship was found between the clinical picture and the severity of the neuronal migration disorder, as revealed by magnetic resonance imaging. In the three cases, EEG shares some common features: multifocal epileptic activity with frequent bilateral diffusion, and high-amplitude anterior fast activity, intermingled in two patients with bursts of repetitive spikes.

Adult↗

Clinical and magnetic resonance features of the classic and akinetic-rigid variants of Huntington's disease.

We studied 32 patients with confirmed Huntington's disease (HD); six (mean age, 31.7 years) had the akinetic-rigid form and 26 (mean age, 46.1 years) had the classic hyperkinetic form. Clinical examination included a count of abnormal involuntary movements, motor self-sufficiency evaluation by the Physical Disability Rating Scale, cognitive function assessment by the Mini-Mental State examination, and a verbal fluency test. Magnetic resonance imaging permitted measurement of bicaudate diameter, a sensitive indicator of caudate atrophy in HD. Patients with the akinetic-rigid form of HD were younger and had earlier disease onset than those with the classic form of HD. All patients with akinetic-rigid HD (group 1) had striatal hyperintensity on T2-weighted magnetic resonance images; seven patients with classic HD (group 2) had a similar abnormality. Groups 1 and 2 were in fact similar in all other respects, except that the number of abnormal involuntary movements was greater in group 2. Groups 1 and 2 together had significantly younger age at onset, lower Mini-Mental State Examination score, more severe motor disability, worse verbal fluency test result, and greater bicaudate diameter than the 19 patients with classic HD without magnetic resonance signal abnormality (group 3) and appear to be a uniform population, distinct from group 3. The abnormalities on magnetic resonance images indicated greater striatal damage in groups 1 and 2, which could be the neuroanatomic substrate of their greater motor and cognitive compromise.

Adult↗

Multiple system atrophy. Clinical and MR observations on 42 cases.

Probable or possible multiple system atrophy (MSA) was diagnosed on strict clinical criteria in 42 patients: 20 with combined parkinsonism and cerebellar ataxia, 9 with striatonigral degeneration (SND) and 13 with olivopontocerebellar atrophy (OPCA). All patients were then studied with 0.5 and/or 1.5 Tesla magnetic resonance (MR) units. MR imaged putaminal abnormalities in all 9 patients with SND and posterior fossa abnormalities consistent with OPCA in all 13 patients with this diagnosis. Of the 20 patients with parkinsonism and cerebellar involvement, classified as probable MSA, 7 presented putaminal abnormalities only, 3 abnormalities consistent with OPCA only and 10 a combination of both. These findings show strong MRI support for the clinical diagnosis of MSA.

Adult↗

Combined central and peripheral acute demyelination.

We describe a patient with multiple sclerosis who had a bout of central demyelination associated with an acute inflammatory demyelinating polyneuropathy. The contemporary involvement of central and peripheral nervous system due to a demyelinating disease has been reported anecdotically in humans, and can be induced experimentally in animals. It may be sustained by a common pathogenetic factor.

Adult↗

MRI in ataxias.

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Ataxia↗

The effects of wallerian degeneration of the optic radiations demonstrated by MRI.

The effects of wallerian degeneration can be demonstrated by MRI as abnormal signal along the course of the degenerate fibres; they have previously been reported in the corticospinal tract. We report two cases of wallerian degeneration of the right optic radiations due to lesions of the right lateral geniculate body. The anatomy and the MRI visibility of the normal optic radiations are briefly discussed.

Brain Ischemia↗

Hallervorden-Spatz disease: clinical and MRI study of 11 cases diagnosed in life.

The diagnosis of Hallervorden-Spatz disease (HSD) has usually been made post mortem, although the recent description of characteristic abnormalities in the globus pallidus has suggested the possibility of an in vivo diagnosis. We present the clinical histories, neurological features and MRI findings of 11 patients, diagnosed as having HSD. Generalized dystonia with predominance of oromandibular involvement, behavioural changes followed by dementia and retinal degeneration were present in all the patients. MRI pallidal abnormalities consisted of decreased signal intensity in T2-weighted images, compatible with iron deposits, and of a small area of hyperintensity in its internal segment ("eye of the tiger" sign). We propose that the combination of these neurological signs with these MRI findings could be considered as highly suggestive of a diagnosis of HSD in living patients.

Adolescent↗

Abnormal MRI signal in the rigid form of Huntington's disease.

Eighteen patients with Huntington's disease (HD) were examined with MRI. Eleven had the common hyperkinetic form, seven had the rigid variant. All seven patients with rigid HD had increased signal intensity in the neostriatum in intermediate and T2-weighted images. Only one hyperkinetic HD had similar findings. In all the other cases, signal abnormalities were questionable or absent. Histological differences that account for differences in signal intensity may therefore lie in the caudate nucleus and putamen. Age of onset may be important, since the rigid patients were younger. Follow up studies may help in understanding these signal differences.

Adolescent↗

Double blind study of intrathecal beta-interferon in multiple sclerosis: clinical and laboratory results.

Sixteen patients with clinically definite MS admitted to a double blind randomised controlled trial of intrathecal natural beta-IFN were followed for a mean of 22 months including the six month treatment period. Clinical response, evaluated in terms of relapse frequency and of progression rate, showed an increase in relapse rate in treated patients during the six month treatment period and, overall, no benefit in treated versus placebo patients. Serial evaluations were made of cerebrospinal fluid (CSF) cells, IgG, myelin basic protein and CSF and blood T-cell subsets. A rise in CSF IgG Index, MBP and DR+ cells in IFN-treated patients suggested an activation of intrathecal immune response in treated patients.

Adolescent↗

Olivopontocerebellar atrophy: MR diagnosis and relationship to multisystem atrophy.

Clinical diagnosis of olivopontocerebellar atrophy (OPCA) must be confirmed by radiologic demonstration of atrophy in an appropriate distribution. OPCA may be associated with degeneration of other systems in multisystem atrophy (MSA). The authors report 23 cases of OPCA, eight of which were associated with MSA. Atrophy involved the cerebellum, pons, and middle cerebellar peduncles in all cases. On intermediate and T2-weighted magnetic resonance (MR) images, abnormal signal intensity was always observed in the transverse pontine fibers, middle cerebellar peduncles, and cerebellum, structures known from pathologic study to degenerate in OPCA. Pyramidal tracts and superior cerebellar peduncles stood out because of their normal signal intensity. Of the eight patients with MSA, four also had variable abnormal signal intensities in the putamen. The authors believe that the combination of atrophy and abnormal signal intensity in the appropriate distribution strongly supports the diagnosis of OPCA. In some cases, MR imaging may demonstrate involvement of different systems, thus confirming the diagnosis of MSA.

Adult↗

Iatrogenic intraspinal epidermoid tumor: myelo-CT and MRI diagnosis.

An 11-year-old boy, treated for acute lymphatic leukemia at the age of 2 with intrathecal injections of Methotrexate, presented with a two year history of pain and signs of lumbo-sacral lesion. MRI, myelography and myelo-CT demonstrated an intradural L4-L5 epidermoid tumor which was removed. Iatrogenic implantation of epithelial cells at the age of two with lumbar punctures is most likely. Decline in incidence of lumbar iatrogenic epidermoid cysts, now an exceedingly rare event, is probably related to improved needles for lumbar punctures.

Child↗

MRI in internal carotid artery dissection.

We report two cases of internal carotid artery dissection in which MRI unequivocally demonstrated the intramural hematoma. We propose that, whenever the clinical presentation or the angiogram suggest arterial dissection, MRI be done to confirm the diagnosis.

Carotid Artery Diseases↗

Brainstem auditory evoked potential abnormalities and magnetic resonance imaging in posterior fossa lesions.

Brainstem auditory evoked potentials (BAEPs) were evaluated in a series of 15 patients with extra-axial cerebello-pontine angle tumors (3 cases), demyelinating plaques (11 cases), and intra-axial tumor (1 case), verified by magnetic resonance imaging (MRI). A satisfactory correlation between the location of the lesions and the type of BAEP abnormalities was found in 11 cases. In 2 other cases, definite MRI brainstem lesions located outside the acoustic pathways were associated with normal BAEPs. In the opposite situation (2 cases of BAEP abnormalities with normal MRI), the inability of MRI to detect demyelinating lesions may be due to the temporal evolution of the lesions themselves. The results of this BAEP-MRI comparative study confirm that BAEP is a sensitive diagnostic tool in revealing brainstem dysfunction, although its localizing power appears to be debatable. The most satisfactory MRI/BAEP topodiagnostic correlation was found with lesions involving the acoustic nerve in its intracranial tract or the caudal pons and bulbopontine junctions.

Adolescent↗

Magnetic resonance imaging in multiple sclerosis: diagnostic value and clinical correlations.

101 patients with multiple sclerosis (MS) (53 definite, 22 probable, 8 possible and 18 suspected according to the McDonald and Halliday criteria) were investigated by magnetic resonance imaging (MRI), to evaluate its diagnostic sensitivity compared to other diagnostic parameters such as cerebrospinal fluid (CSF) findings and auditory brainstem responses (ABR). In addition, correlations between severity of lesions on MRI and clinical data (disability duration of the disease and progression rate) and CSF immunoglobulin abnormalities were evaluated. MRI detected multiple demyelinating lesions in 96% of definite, 77% of probable, 50% of possible and 56% of suspected MS. In the same groups CSF was positive for oligoclonal banding in 85%, 68%, 50% and 37% and ABR in 82%, 50%, 80% and 28% respectively. Severity of MRI was found to correlate with disability and duration of the disease, but not with progression rate. The presence of CSF oligoclonal bands and a raised and IgG index were usually associated with more extensive white matter involvement as evaluated by MRI.

Brain↗

CT and MRI in maple syrup urine disease.

We describe a patient with a variant form of maple syrup urine disease who had unusual CT and MRI features that raised the suspicion of a metabolic disease. There were low density and abnormal signal in the white matter and pallida. Sponginess in these areas is the likely explanation for these findings.

Amino Acids, Branched-Chain↗