Biomedical subjects

R J Caselli

Publications and source records attributed to R J Caselli.

45 records · Page 3Linked to original sources

PubMed1992

Asymmetric cortical degeneration syndromes. A proposed clinical classification.

Twenty-six patients presented with slowly progressive focal neurologic symptoms that conformed clinically to one of three categories: asphasia, perceptuomotor dysfunction, or neuropsychiatric dysfunction. Of 12 patients with progressive aphasia, seven were dysfluent and five were fluent. Nine patients had progressive perceptuomotor impairment due to bilateral parietal lobe atrophy, which also included frontal lobe signs in seven patients and occipital lobe signs in three patients. The right hemisphere was more severely involved in five patients and the left hemisphere in four. Five patients had a progressive neuropsychiatric syndrome, and there was also generalized spasticity in three patients due to frontal lobe atrophy. The clinically suspected anatomic localization of cortical atrophy or hypoperfusion in all three categories was confirmed with neuroimaging techniques. A brain biopsy specimen from one patient showed mild, nonspecific degenerative changes. A clinical classification scheme incorporating our observations as well as the observations of others is presented to aid in the recognition of these syndromes.

Aged↗

PubMed1992

Asymmetric cortical degenerative syndromes: clinical and radiologic correlations.

Eight patients presented with slowly progressive focal neurologic syndromes that conformed to one of three clinically defined categories: progressive nonfluent aphasia (three patients), progressive perceptual-motor impairment (four patients), and progressive frontal lobe syndrome (one patient). Planar MRI and MRI-based surface or volume renderings demonstrated focal areas of atrophy that correlated well with clinical deficits. Single-photon emission computed tomography (SPECT) showed areas of cortical hypoperfusion that corresponded to focally atrophic regions revealed by MRI, but abnormal areas with SPECT were larger than those suggested by MRI. MRI and SPECT are useful in defining the regional structural and functional cerebral abnormalities that underlie slowly progressive focal neurologic syndromes caused by asymmetric cortical degeneration.

Aged↗

PubMed1991

The treatable dementia of Sjögren's syndrome.

Progressive dementia developed during a 15-month period in a 56-year-old woman with serologically and clinically documented primary Sjögren's syndrome. Findings from magnetic resonance imaging and angiography were normal, but a brain biopsy disclosed perivascular lymphocytic inflammation in leptomeningeal and parenchymal vessels. Treatment with high-dose corticosteroids produced rapid and nearly complete resolution of the dementia.

Alzheimer Disease↗

PubMed1991

Rediscovering tactile agnosia.

Eighty-four patients with damage to various levels of the nervous system, ranging from the peripheral nerves to the cerebral cortex, underwent somesthetic assessment in order to determine the degree to which basic and complex perceptual and motor disorders affect tactile object recognition (TOR) and to determine whether TOR can be impaired in the absence of more basic sensorimotor imperception. The results suggest that (1) basic and intermediate disorders of somesthetic function impair TOR but are commensurately more severe for any given degree of TOR impairment in patients with peripheral lesions than in patients with cortical lesions; (2) neither hemiparesis nor hemianopia alone precludes normal TOR; (3) hemineglect contributes substantially to TOR impairment; (4) impairment of TOR can occur in the absence of more basic somesthetic dysfunction and constitutes tactile agnosia; (5) tactile agnosia is a subtle, nondisabling disorder that should be distinguished from the nonagnosic, severe and disabling disorder, astereognosis; and (6) tactile agnosia results from unilateral damage to parietotemporal cortices, possibly including the second somatosensory cortex, in either hemisphere.

Adolescent↗

PubMed1991

Bilateral impairment of somesthetically mediated object recognition in humans.

Thirty adult patients (six in each of five groups--neurologically normal, lacunar infarct-related hemiparesis, unilateral thalamic lacunar infarction, right cortical infarction with mild left hemineglect, and extensive right cortical infarction with severe left hemineglect) were asked to perform various tasks that encompassed basic and intermediate somatosensory functions and tactile and visual object recognition. Patients with thalamic and cortical infarctions had severe impairment of contralateral hand-mediated somatosensory functions in all three categories of somesthetic tasks, although patients with cortical infarction were more impaired on the object recognition task than were patients with thalamic infarction. Patients with extensive damage to the right hemisphere and severe left hemineglect also had impairment of somesthetically mediated object recognition in the ipsilateral hand despite normal basic and intermediate somatosensory function and visually mediated object recognition analogous to unilateral tactile agnosia. All other groups had normal ipsilateral tactile object recognition.

Adolescent↗

PubMed1991

Associative anomia: dissociating words and their definitions.

A 60-year-old woman, in whom magnetic resonance imaging and single photon emission computed tomography confirmed the presence of chronic damage to the left temporal lobe, had pure anomia with sparing of other language and cognitive functions. She could independently access lexical knowledge (words) and semantic knowledge (word definitions). We postulate that her anomia was due to a failure to associate lexical and semantic knowledge.

Anomia↗

PubMed1990

Giant cell (temporal) arteritis: a treatable cause of multi-infarct dementia.

Dementia occurs infrequently in patients with giant cell (temporal) arteritis (GCA). Three elderly women with biopsy-proven GCA showed abrupt cognitive decline during periods of clinically active GCA, 1 to 6 months after diagnostic temporal artery biopsy, during periods of corticosteroid taper. One patient had additional clinical signs of cerebral infarction and other ischemic phenomena. Reinstitution of higher oral doses of corticosteroids successfully prevented further cognitive losses and permitted gradual but incomplete improvement of cognitive function in 1 patient. Neuropsychologic data from 2 patients 7 to 10 months after temporal artery biopsy suggested multifocal cognitive impairment, and the 3rd patient appeared clinically to be globally, severely demented. Neuroimaging studies revealed multiple areas of infarction, predominantly in the posterior circulation territory. One patient had bilateral vertebral artery occlusions (digital subtraction angiography) and bilaterally reduced carotid system perfusion pressures (oculoplethysmography). There were no associated cardiovascular risk factors or family history of dementia in these patients.

Aged↗

PubMed1988

Neurologic disease in biopsy-proven giant cell (temporal) arteritis.

Neurologic findings were studied in 166 consecutive patients with biopsy-proven giant cell (temporal) arteritis. Neurologic problems occurred in 51 patients (31%): neuropathies (23), TIA/strokes (12), neuro-otologic syndromes (11), tremor (6), neuropsychiatric syndromes (5), tongue numbness (3), and myelopathy (1). Neuro-ophthalmologic problems occurred in 35 patients (21%): amaurosis fugax (AF) (17), permanent vision loss (PVL) (14), scintillating scotoma (8), and diplopia (3). Abnormalities in large arteries in 52 patients (31%) included bruits and diminished pulses. The carotid artery was involved in 31 patients (bilateral in 58%). Overall, 35% of patients with carotid disease had TIA/stroke, AF, or PVL.

Aged↗

PubMed1988

Peripheral neuropathic syndromes in giant cell (temporal) arteritis.

Of 166 consecutive patients with histologically confirmed giant cell (temporal) arteritis (GCA) seen during a 3-year period, 23 (14%) had clinically diagnosed peripheral neuropathic syndromes temporally coincident with clinically active GCA. Electromyography and nerve conduction studies were performed in 16, confirming abnormalities in all. Of the 23 patients, 11 had a generalized peripheral neuropathy, nine had multiple mononeuropathies, and three had a mononeuropathy. The nerves affected as mononeuropathies were the median, ulnar, peroneal, tibial, and sural nerves, and the C-5 and L-5 nerve roots. Angiography, performed in two patients, demonstrated widespread arteritis involving the lower limbs and, after 3 months of oral corticosteroid treatment in one of these patients, an amputation specimen showed chronic arteritis.

Aged↗