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Biomedical subjects

J T Caroscio

Publications and source records attributed to J T Caroscio.

14 recordsLinked to original sources
PubMed1988

Hexosaminidase A activity and amyotrophic lateral sclerosis.

Abnormalities of GM2 ganglioside metabolism owing to hexosaminidase A (Hex A) deficiency have been associated with ALS phenotypes. The clinical features described in these ALS patients with Hex A deficiency include early onset, positive family history, and/or long disease duration. In an attempt to determine prospectively the incidence of Hex A deficiency within an ALS population, the records of The Mount Sinai Medical Center ALS Clinic were reviewed to select those patients with "atypical" ALS (total N = 52), i.e. onset before age 35, positive family history, and/or disease duration greater than 90 months. The control group (total N = 50), "typical" ALS patients, did not fulfill any of these historical criteria. Hex A activity determined in isolated peripheral blood leukocytes was normal in all typical ALS patients (mean 67.3%). Hex A deficiency was not found in any atypical ALS patients. Thus, Hex A deficiency apparently is an unusual etiology of typical or atypical ALS but is of medical and genetic importance in individual families.

Age Factors↗
PubMed1987

Abnormal glutamate metabolism in amyotrophic lateral sclerosis.

Glutamate levels were determined in the fasting plasma of 22 patients with early-stage primary amyotrophic lateral sclerosis (ALS) and compared to those of healthy and diseased controls. There was a significant increase (by approximately 100%, p less than 0.0005) in the plasma glutamate of the ALS patients as compared with the controls. Oral glutamate loading (60 mg of monosodium glutamate per kilogram of body weight, taken orally after overnight fasting) resulted in significantly greater elevations in the plasma glutamate and aspartate levels in the ALS patients than in the controls. Glutamate, a potentially neuroexcitotoxic compound, is thought to be the transmitter of the corticospinal tracts and certain spinal cord interneurons. A systemic defect in the metabolism of this amino acid may underlie primary ALS.

Administration, Oral↗
PubMed1987

Amyotrophic lateral sclerosis. Its natural history.

ALS is the most common of the various MNDs, which also include the clinical entities of PBP, PMA, and PLS. Mean age of onset of ALS is 57 years, and there is a sex predilection for men in a ratio of 1.5:1. Area of first symptom is in the lower extremity is 36 per cent of cases, in the upper extremity in 32 per cent, and 25 per cent of patients have a bulbar onset. Motor cranial nerves in the lower brain stem from cranial nerve nuclei or corticobulbar tract degeneration are affected. This results in symptoms of speech and swallowing difficulty and emotional lability in up to 60 per cent of cases. One hundred per cent of cases have motor weakness, over 90 per cent have muscle atrophy and fasciculations, and 47 per cent have spasticity. There seems to be a pattern of progression of ALS signs and symptoms based on area of onset with LLE involvement tending to follow RLE weakness, LUE weakness following RUE onset, and RUE involvement following next in patients whose onset is bulbar. Significant numbers of ALS patients had sparing of involvement of parts of the body for follow-up times approaching 3 years. Although the majority of patients experience a deterioration that is significantly linear, seven of 28 patients or 25 per cent achieved a plateau lasting a minimum of 9 months. Survival in our series was 4.08 years for all forms of MND.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗
PubMed1986

Quantitative cerebrospinal fluid IgG measurements as a marker of disease activity in multiple sclerosis.

The value of various parameters of reporting quantitative cerebrospinal fluid (CSF) IgG levels to indicate disease activity in 34 patients with clinically definite multiple sclerosis was examined. IgG alone correlated significantly with increasing degree of disability and increasing number of clinical central nervous system lesions. There was also a trend toward higher mean IgG levels when the course was relapsing and progressive as opposed to progressive or relapsing. For the IgG index, the relationships were the inverse of that noted with IgG alone. IgG-albumin ratio and IgG synthetic rate did not correlate significantly with course, number of CNS lesions, or degree of disability, and there was no statistically significant relationship between any parameter of reporting quantitative CSF IgG and age, duration of disease, history of recent exacerbation, or area of first involvement in the nervous system. We conclude that although newer methods of reporting CSF IgG elevations in multiple sclerosis are more sensitive and some of them, more specific, in confirming a diagnosis than CSF IgG alone, this parameter remains the best marker of disease activity in individual patients.

Humans↗
PubMed1986

A double-blind, placebo-controlled trial of TRH in amyotrophic lateral sclerosis.

A double-blind, placebo-controlled trial of single doses of thyrotropin releasing hormone (TRH) was performed on 12 patients with amyotrophic lateral sclerosis. Each patient was given subcutaneous injections of TRH 150 mg or placebo, and IV infusions of TRH 500 mg or placebo at 72- to 96-hour intervals. Eight motor and functional ratings were scored at regular intervals after each injection. Side effects were seen in all patients and were obvious to patients and examiners, making true blinding impossible. Nevertheless, statistically significant improvement was seen only in dynametric strength 1 hour after subcutaneous injection (p less than 0.05). Significant improvement occurred, in one patient only, on subjective speech testing during IV infusion of TRH. In none of six other ratings was there a significant difference between TRH and placebo. Subjective improvement was noted by 11 of 12 patients.

Adult↗
PubMed1985

ALS and pet exposure.

To examine animal exposure in ALS patients, a case-control study was undertaken on 40 ALS patients and 40 closely matched controls. Exposure to pets of any kind and to small dogs in the period from birth until 10 years before onset of ALS symptoms was significantly increased. Affected men showed a trend toward increased exposure to neurologically ill pets, but there was no significant difference when male and female cases were grouped. No differences between ALS cases and controls were observed in exposure to any other single animal species. There may be a link between ALS and exposure to house pets.

Adult↗
PubMed1983

Quantitative CSF IgG measurements in multiple sclerosis and other neurologic diseases. An update.

To compare four ways of measuring CSF IgG levels in diagnosing multiple sclerosis (MS), we analyzed CSF samples of 106 patients with clinically definite, probable, or possible MS and 127 patients with other diseases. The IgG synthetic rate and IgG index were the most sensitive tests at 0.88 and 0.94, respectively; IgG alone and IgG-albumin ratio, at 0.53 and 0.59, were less valuable. The IgG synthetic rate (0.87) was more specific than the IgG index (0.73), making it the quantitative measure that best correlated with a clinical diagnosis of definite MS. However, combining these four methods showed an even higher correlation. Quantitative CSF IgG elevations occurred much less frequently in patients with clinically definite MS receiving immunosuppressives and in those with clinically probable and possible MS. We did not perform qualitative CSF IgG measurements, but our methods' sensitivity and specificity were comparable with those attributed to oligoclonal IgG bands by others. We also found numerous other diseases where elevations of CSF IgG occurred by all four methods.

Central Nervous System Diseases↗
PubMed1983

Amyotrophic lateral sclerosis: a comprehensive rehabilitation approach.

A clinic was established at The Mount Sinai Medical Center and staffed by a multidisciplinary team to assess the feasibility of caring for the patient with amyotrophic lateral sclerosis (ALS) through an outpatient facility. Three hundred ALS patients were evaluated and followed in a 3-year period. Each patient was viewed as an individual, and the establishment of four clinical neurologic scales (or stages) proved helpful in tracking declining functional performance and in the establishment of suggestions for care. A rehabilitation program can be effective in helping the ALS patient maximize functional abilities while minimizing disabilities and complications at every stage of the disease. Such a program can be implemented through an outpatient clinic with regularly scheduled follow-up visits.

Aged↗
PubMed1980

Subarachnoid hemorrhage secondary to spinal arteriovenous malformation and aneurysm. Report of a case and review of the literature.

A patient with recurrent subarachnoid hemorrhage was seen initially with intermittent signs and symptoms of intracranial and spinal cord dysfunction. Myelography and spinal angiography revealed an arteriovenous malformation (AVM) and aneurysm of the spinal cord. Extensive investigation failed to reveal any intracranial lesion. The relationship of subarachnoid hemorrhage at a spinal level to the development of remote neurological abnormalities is discussed, and previous reports of aneurysms associated with spinal AVM are reviewed.

Adult↗
PubMed1980

Progressive myelopathy due to sarcoid.

A patient developed a progressive paraparesis after a bout of uveitis and parotitis; and lung biopsy was diagnostic of sarcoidosis. A myelogram was normal on two occasions. Spinal tap revealed an elevated protein, a pleocytosis and a low glucose, and a diagnosis of sarcoidosis of the spinal cord made. Previous reports of sarcoidosis of the spinal cord are reviewed. Of interest in this case was an elevated CSF IgG. Elevation of CSF globulin or gamma globulin may be a common finding in this condition as it was noted in 14 of 15 cases of CNS sarcoid previously reported.

Humans↗