PubMed Health⌕ Search

Biomedical subjects

R M Pascuzzi

Publications and source records attributed to R M Pascuzzi.

At least 19 recordsLinked to original sources
PubMed2003

A randomized, placebo-controlled trial of topiramate in amyotrophic lateral sclerosis.

OBJECTIVE: To determine if long-term topiramate therapy is safe and slows disease progression in patients with ALS. METHODS: A double-blind, placebo-controlled, multicenter randomized clinical trial was conducted. Participants with ALS (n = 296) were randomized (2:1) to receive topiramate (maximum tolerated dose up to 800 mg/day) or placebo for 12 months. The primary outcome measure was the rate of change in upper extremity motor function as measured by the maximum voluntary isometric contraction (MVIC) strength of eight arm muscle groups. Secondary endpoints included safety and the rate of decline of forced vital capacity (FVC), grip strength, ALS functional rating scale (ALSFRS), and survival. RESULTS: Patients treated with topiramate showed a faster decrease in arm strength (33.3%) during 12 months (0.0997 vs 0.0748 unit decline/month, p = 0.012). Topiramate did not significantly alter the decline in FVC and ALSFRS or affect survival. Topiramate was associated with an increased frequency of anorexia, depression, diarrhea, ecchymosis, nausea, kidney calculus, paresthesia, taste perversion, thinking abnormalities, weight loss, and abnormal blood clotting (pulmonary embolism and deep venous thrombosis). CONCLUSIONS: At the dose studied, topiramate did not have a beneficial effect for patients with ALS. High-dose topiramate treatment was associated with a faster rate of decline in muscle strength as measured by MVIC and with an increased risk for several adverse events in patients with ALS. Given the lack of efficacy and large number of adverse effects, further studies of topiramate at a dose of 800 mg or maximum tolerated dose up to 800 mg/day are not warranted.

Adult↗
PubMed2001

Pearls and pitfalls in the diagnosis and management of neuromuscular junction disorders.

Myasthenia gravis (MG) is a common autoimmune disorder characterized by the presence of pathogenic antibodies directed against the acetylcholine receptor. Patients present with variable degrees and distribution of fluctuating weakness, at times life-threatening. Clinical manifestations, establishment of diagnosis, the natural history of MG, and therapeutic options are herein reviewed with an emphasis on pearls and pitfalls of clinical relevance. Far less common is Lambert-Eaton syndrome (the myasthenic syndrome), another autoimmune disorder due to the presence of antibodies directed against the PQ-type voltage-gated calcium channels. Clinical features and treatment issues of these and other disorders of neuromuscular transmission are reviewed.

Botulism↗
PubMed2000

Neurology in the art museum: Andrew Wyeth's Christina's World.

Andrew Wyeth's painting of his friend and neighbor Christina Olson, Christina's World, arguably the best known picture by a living American painter, depicts a woman crawling across a field while gazing toward her house. Christina Olson had a lifelong slowly progressive paralytic disorder. Reflections on her life, clinical symptoms, medical evaluation, and her influence on Mr. Wyeth are presented herein.

Adult↗
PubMed1999

Early observations on muscular dystrophy: Gowers' textbook revisited.

Early clinical observations on Duchenne muscular dystrophy can be traced through the works of Meryon, Little, Duchenne, Gowers, and Erb. Gowers sites Sir Charles Bell with its earliest clinical description. Gowers' phenomenal textbook provides vivid descriptions of Duchenne dystrophy, clinical features which are herein revisited.

History, 19th Century↗
PubMed1999

Hors d'oeuvres for neurology.

From time to time, in the setting of lectures, rounds, or casual conversation, there is a need for hors d'oeuvres; small pieces, spices, and artifacts that generate a bit of thought and interest with a neurological twist. A potpourri of neurological trivia is herein presented for the purpose of stimulating the reader and serving as a brief reserve of questions and topics for use on rounds.

History, 18th Century↗
PubMed1999

Ravel's neurological illness.

In the last 10 years of his life, Maurice Ravel (1875-1937) experienced a gradually progressive decline in neurological function. Dr. Alajouanine examined Ravel, noting the presence of aphasia and apraxia with relative preservation of comprehension and memory. The exact diagnosis remains unclear, but the likelihood of a progressive degenerative disorder, such as frontotemporal dementia, is herein discussed.

Dementia↗
PubMed1999

Shostakovich and amyotrophic lateral sclerosis.

Dmitri Shostakovich (1906-1975) is one of the Twentieth Century's greatest composers. Beginning in the late 1950s, he experienced gradual progressive asymmetric weakness of the limbs, and was eventually diagnosed with motor neuron disease, which is the subject of this review.

Amyotrophic Lateral Sclerosis↗
PubMed1998

Blinded and seeing the light, (John Noseworthy), Lou Gehrig and other tales of enlightenment).

Patients having serious neurological diseases often wonder why clinical trials must use controls and double blinding in order to prove efficacy. Studies on the effect of examiner blinding in multiple sclerosis trials, as well as the published results of an unblinded uncontrolled clinical trial of Vitamin E therapy in patients with amyotrophic lateral sclerosis (including Lou Gehrig) provide clear illustrations of the impact of blinding and controls on outcome. These reports serve as a resource for physicians, patients and their families in discussing the rationale for controls and double blinding, and instill caution that should be used when judging results of studies which are unblinded or uncontrolled.

Amyotrophic Lateral Sclerosis↗
PubMed1998

Pearls and pitfalls in the horror cinema.

Observations on the neurologic signs and symptoms of Count Dracula, Wolfman, and Frankenstein's Monster are presented as viewed by a specialist in neuromuscular disease. Key clinical features of these horror movie figures illustrate a variety of pearls in the diagnosis of a variety of neurologic disorders, including porphyria, lead poisoning, osteosclerotic myeloma, and myasthenia gravis.

Humans↗
PubMed1998

American history 101: presidents, vice presidents, and paralytic illness.

Neurological disease has had a profound effect on history as with the fatal stroke of Franklin D. Roosevelt. Roosevelt and two of his vice presidents, Harry Truman, and Henry Wallace also suffered from acquired paralytic disorders. The sum total of the neurological problems of these three statesmen had a significant effect on their careers, and the course of history.

Amyotrophic Lateral Sclerosis↗
PubMed1998

Drugs and toxins associated with myopathies.

Drug-induced muscle dysfunction represents a significant and perhaps increasing subset of neuromuscular disorders that face the clinician. Whereas severe symptoms of proximal weakness and elevated muscle enzymes in an uncomplicated patient taking a single medication may lead to straightforward diagnosis, the tendency for patients with multisystem disease, on multiple medications, with multiple potential causes for weakness makes the diagnosis of toxic myopathy challenging. Furthermore, many toxic myopathies are characterized by nonspecific clinical and laboratory findings, ultimately requiring a trial of drug discontinuation in order to clarify the diagnosis. This review summarizes recent observations with regard to toxic effects on neuromuscular transmission and toxic myopathies.

Botulinum Toxins↗
PubMed1998

Primary intravascular lymphomatosis associated with Mycobacterium marinum.

Intravascular lymphomatosis (i.v.l.) is a rare condition in which neoplastic cells preferentially infiltrate blood vessels of the central nervous system. Nonspecific symptoms associated with i.v.l. include dementia, seizures, and multifocal cerebrovascular events. i.v.l. was discovered at autopsy of a patient whose neurological deficits were predated by a particularly aggressive form of Mycobacterium marinum soft-tissue infection. It is speculated that i.v.l. may have had an occult effect on the patient's cell-mediated immunity that predisposed him to this normally innocuous mycobacteria.

Blood Vessels↗
PubMed1997

Subacute sclerosing panencephalitis manifesting as viral retinitis: clinical and histopathologic findings.

PURPOSE AND METHODS: To describe the clinical and histopathologic features of a patient with viral retinitis secondary to subacute sclerosing panencephalitis. RESULTS: The patient was a human immunodeficiency virus-negative intravenous drug abuser with an acute retinitis that later progressed to encephalitis despite aggressive treatment for possible viral, protozoal, bacterial, and rickettsial infections. The patient had many of the characteristic findings of subacute sclerosing panencephalitis, including a history of measles in early childhood, myoclonus, periodic complexes on electroencephalographic testing, persistently elevated serum and cerebrospinal fluid antimeasles immunoglobulin G (IgG) titers, and a cerebrospinal fluid oligoclonal IgG gammopathy. Ultrastructural examination demonstrated numerous filamentous microtubular intranuclear viral inclusions in the nuclear layers of the retina consistent with the measles virus. This case is unusual in that our patient developed subacute sclerosing panencephalitis later in life and because there was an 8-year period between presumed viral infections in the two eyes. CONCLUSIONS: An acute retinitis in an intravenous drug abuser is not always caused by human immunodeficiency virus-related infections; not all viral retinitis responds to therapy; and mortality as well as the usual morbidity may be associated with viral retinitis. One might consider the diagnosis of subacute sclerosing panencephalitis in a young person with an acute retinitis with little or no vitreal inflammation and lack of response to anticytomegalovirus and antitoxoplasmosis therapy.

Adult↗
PubMed1997

Acute peripheral neuropathy in adults. Guillain-Barré syndrome and related disorders.

Acute paralysis in adults has an extensive assortment of etiologies. Guillian-Barré syndrome is the most common cause of acute neuropathy in adults. This review emphasizes pathophysiology, clinical features, differential diagnosis, and a practical approach to the laboratory work-up for patients with suspected Guillian-Barré syndrome. The current status of immunotherapy is reviewed.

Adrenal Cortex Hormones↗
PubMed1996

Sarcoid myelopathy.

A 45-year-old woman with history of iritis, uveitis, and sarcoidosis of the skin presented with a subacute cervical myelopathy. Magnetic resonance imaging (MRI) showed patchy, multifocal, gadolinium-enhancing intramedullary lesions of the spinal cord, and extramedullary lesions of the basal cisterns and fourth ventricle. Symptoms and MRI abnormalities were improved within 1 month of corticosteroid therapy.

Female↗
PubMed1996

The spectrum of neuroimaging abnormalities in solvent abuse and their clinical correlation.

The brain magnetic resonance (MR) images and medical records of 6 patients with a history of chronic toluene abuse were reviewed retrospectively. The imaging findings were correlated with clinical impairment. The major findings consisted of atrophy (6 patients), white matter T2 hyperintensity (6 patients), and T2 hypointensity involving the basal ganglia and thalami (5 patients). Also seen was focal enhancement, previously unreported for patients who abused toluene. This study showed a correlation between the degree of neurological impairment and extent of white matter disease. There was no correlation between the severity of white matter changes on MR images and the presence of T2 hypointensity or duration of toluene abuse. There was no definite clinical evidence of damage to the basal ganglia and thalami despite the MR imaging finding of T2 hypointensity. Temporal evidence against the theory that toluene accumulation causes the T2 hypointensity was found.

Adult↗