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R L Sufit

Publications and source records attributed to R L Sufit.

15 recordsLinked to original sources
PubMed1992

Acute myopathy with selective degeneration of myosin filaments following status asthmaticus treated with methylprednisolone and vecuronium.

We report two cases of severe, acute myopathy with selective degeneration of myosin filaments in asthmatics who developed respiratory failure with hypercapnia and acidosis requiring endotracheal intubation, administration of vecuronium and prolonged ventilatory support. Hypoxia was documented in one case and probably present in the other. Both patients received prolonged treatment with high doses of intravenous methylprednisolone. Flaccid quadriparesis was noted after discontinuation of vecuronium. Muscle biopsy showed nonspecific myopathic changes on light microscopy. Electron microscopy revealed selective loss of myosin filaments in many fibers. Recovery occurred within 2 months with supportive treatment. This entity is probably related to a combination of high doses of corticosteroids, vecuronium administration and metabolic abnormalities associated with respiratory failure.

Acute Disease↗
PubMed1992

Glycemic control and peripheral nerve conduction in children and young adults after 5-6 mo of IDDM. Wisconsin Diabetes Registry.

OBJECTIVE: A cohort of people (n = 86) was examined in the first few months after insulin-dependent diabetes mellitus (IDDM) diagnosis to evaluate the effect of hyperglycemia on nerve conduction velocities and latencies. RESEARCH DESIGN AND METHODS: Unselected cases with IDDM, who were 6-29 yr of age, were identified at diagnosis from a large, geographically defined area of southern Wisconsin. Peripheral nerve conduction was measured on a sample from this cohort. RESULTS: Peroneal nerve conduction velocity was significantly inversely related to glycosylated hemoglobin (P less than 0.05, age and height adjusted). All other nerve conduction velocities and latencies (median motor, median sensory, and sural) showed the same tendency, but the associations were not statistically significant. Twenty-four-hour urine C-peptide and duration of diabetes (3-11 mo) were not consistently related to nerve conduction parameters after controlling for age and height. CONCLUSIONS: These findings suggest that as early as 5-6 mo after diabetes diagnosis, and at a time frequently characterized by partial remission of IDDM, hyperglycemia has a role in the acute slowing of nerve conduction velocity. Other factors such as residual endogenous insulin production do not appear to influence these early changes.

Adolescent↗
PubMed1991

Speech deterioration in amyotrophic lateral sclerosis: a case study.

Few detailed reports have been published on the nature of speech and voice changes during the course of amyotrophic lateral sclerosis (ALS). The subject of this case study is a woman who was diagnosed as having ALS with bulbar signs at the age of 53. Speech intelligibility, pulmonary function, and selected speech and voice functions were tested during an approximately 2-year course of her disease. Over this period, her speech intelligibility, as measured by a multiple-choice word identification test, declined from 98% to 48%. Phonetic features that were most affected during the intelligibility decline included voicing contrast for syllable-initial and syllable-final consonants, place of articulation contrasts for lingual consonants, manner of articulation for lingual consonants, stop versus nasal manner of production, features related to the liquid consonants, and various features related to syllable shape. An acoustic measure, average slope of the second-formant frequency, declined in association with the intelligibility reduction and is thought to reflect the loss of lingual motoneurons. Her pulmonary function also declined over the observation interval, with particularly severe reduction in measures of air flow. Oral diadochokinesis and measures of vocal function (including jitter, shimmer, and signal-to-noise ratio) were highly variable across test sessions. These results are discussed in terms of the challenges they present to sensitive assessment of change and to management of the communication disability in ALS.

Amyotrophic Lateral Sclerosis↗
PubMed1990

Impairment of speech intelligibility in men with amyotrophic lateral sclerosis.

Speech intelligibility was studied in a group of 25 male patients with amyotrophic lateral sclerosis (ALS). The object of the study was to determine the phonetic impairments underlying the speech intelligibility deficits that frequently accompany ALS. Analyses with a word intelligibility test indicated that the most disrupted phonetic features involved phonatory (voicing contrast) function, velopharyngeal valving, place and manner of articulation for lingual consonants, and regulation of tongue height for vowels. The mean error proportion for the five most severely affected features correlated highly (0.97) with the intelligibility score (percent correct). The phonetic feature analyses are one index of bulbar muscle impairment in amyotrophic lateral sclerosis and also may help to direct the speech management in these individuals.

Adult↗
PubMed1990

Doxacurium and mivacurium do not trigger malignant hyperthermia in susceptible swine.

The role of succinylcholine in the precipitation of malignant hyperthermia (MH) necessitates the testing of new neuromuscular relaxants for their ability to trigger MH in MH-susceptible swine before general human use. We tested doxacurium and mivacurium, two new nondepolarizing bis-benzylisoquinolinium neuromuscular relaxants, at ED95 and at four times ED95 doses in swine previously documented to be MH-susceptible. In none of the 16 animals was MH triggered after administration of these relaxants, whereas all animals developed fatal MH after administration of halothane or halothane plus succinylcholine. Muscle biopsy specimens taken before administration of the relaxant confirmed that all animals had increased sensitivity to halothane, caffeine, or both. Thus, we conclude that doxacurium and mivacurium are not triggering agents of malignant hyperthermia in MH-susceptible swine.

Animals↗
PubMed1988

Thyrotropin releasing hormone uptake into serum and cerebrospinal fluid following intravenous or subcutaneous administration.

Intravenous thyrotropin releasing hormone (TRH) was administered to 6 amyotrophic lateral sclerosis (ALS) patients at the dose rate of 10 mg/kg. Blood samples were obtained prior to and at 10, 20, 40, 60, and 120 min during the TRH infusions. Lumbar punctures were performed at 90 min following the start of infusion. The mean serum TRH concentration rose from 0.03 +/- 0.02 (SD) to 17 +/- 2 ng/ml by 60 min and remained constant to 120 min. The mean CSF TRH concentration rose 10-fold from 0.02 +/- 0.01 to 0.2 +/- 0.02 ng/ml at 90 min and increased further to 0.5 +/- 0.2 ng/ml at 120 min. Subcutaneous TRH was administered to 4 ALS patients at 2.5 mg/kg and to 5 ALS patients at 5.0 mg/kg. The mean serum TRH concentration increased to 1.4 +/- 0.6 ng/ml (2.5 mg/kg) and 3.2 +/- 1.1 ng/ml (5.0 mg/kg) by 60 min. The mean CSF TRH concentration at 60 min increased to 0.3 +/- 0.08 ng/ml following 2.5 mg/kg TRH and 0.8 +/- 0.04 ng/ml following 5.0 mg/kg TRH. TRH entry into the CSF is comparable following subcutaneous or intravenous administration.

Adult↗
PubMed1987

Drug-induced peripheral neuropathy in a patient with psoriatic arthritis.

A patient with psoriatic arthritis developed a peripheral neuropathy while taking hydroxychloroquine and naproxen. Although it was initially suspected that hydroxychloroquine was responsible for the neuropathy, subsequent rechallenge with naproxen demonstrated that clinical and electrophysiologic findings were related to routine pharmacologic doses of naproxen.

Adult↗
PubMed1987

Visual evoked potential changes following renal transplantation.

We have followed a group of 18 uremic patients through living-related donor renal transplantation (RTX) using pattern-reversal VEPs. Recordings were made prior to and 10 weeks after surgery at high, medium and low spatial frequencies. Prior to RTX, mean latency of the P100 component of the VEP was 107 msec. Individual values did not correlate with blood urea nitrogen or creatinine. Patients requiring hemodialysis did not differ from non-dialyzed patients. Ten weeks after RTX P100 latencies were significantly shortened while N75 latencies were unchanged. Several diabetic patients exhibited the appearance of previously unrecorded wave forms. P100 latency increased significantly with increasing spatial frequency before and after transplantation. Diabetic patients demonstrated a consistent increase in P100 amplitude while non-diabetic patients demonstrated a consistent decrease in P100 amplitude after RTX. The data indicate that renal transplantation has beneficial effects on the central nervous system of uremic patients not seen with chronic hemodialysis and that these effects may be quantitatively measured using the VEP. The data further suggest that electrophysiological effects of uremia and diabetes may be additive, but reversible after RTX. Alterations in the uremic and diabetic VEP may be related to retinal or more proximal central nervous system structures.

Adolescent↗
PubMed1987

Intermittent negative pressure ventilation in the treatment of respiratory failure in progressive neuromuscular disease.

Five men with degenerative neuromuscular diseases (three with amyotrophic lateral sclerosis [ALS] and two with Duchenne's muscular dystrophy [DMD]) who had respiratory failure were treated with intermittent negative pressure ventilation (NPV). One patient with ALS in severe acute respiratory failure was successfully treated with NPV alone. This patient and two other ALS patients in chronic respiratory failure with PaCO2 elevation stabilized or improved their vital capacity (VC) and lowered their PaCO2 after 5 to 11 weeks of therapy. Finally, intermittent NPV was used to replace 24-hour positive pressure ventilation in two patients with DMD. It is concluded that intermittent NPV may stabilize or temporarily improve the respiratory status in patients with progressive neuromuscular diseases.

Adult↗
PubMed1987

Intravenous thyrotropin-releasing hormone in patients with amyotrophic lateral sclerosis. Dose-response and randomized concurrent placebo-controlled pilot studies.

Focal, small-to-moderate and transient improvement occurred in the muscle strength and function of patients with ALS who received TRH in dose-response and screening studies. In a small pilot study of 12 patients, 3 months administration of TRH at 10 mg per kg on alternate days resulted in localized increased strength of jaw muscles as well as significant improvement in lower extremity function. Aerobic exercise capacity was particularly improved in patients with ALS following administration of TRH. Autonomic effects of TRH on heart rate, respiration, and blood pressure were not serious and attenuated slightly over the course of the study.

Amyotrophic Lateral Sclerosis↗
PubMed1979

Sudden monocular visual loss in pseudotumor cerebri.

We describe a patient with papilledema due to benign intracranial hypertension who had sudden, painless monocular visual loss. Fear of further visual deterioration prompted vigorous therapeutic efforts to reduce intracranial pressure. Subsequent neuro-ophthalmologic evaluation disclosed that the visual loss was due to a subretinal neovascular membrane. Vision continued to improve after medical therapy was discontinued, and was accompanied by complete resolution of papilledema and headache. In any patient with minimal papilledema, sudden visual deterioration should initiate a search for other causes rather than zealous therapeutic efforts to reduce intracranial pressure.

Blindness↗
PubMedDate not supplied

Chronic compartment syndrome: diagnosis, management, and outcomes.

A consecutive operative series of 100 patients with chronic compartment syndrome involving 233 compartments is reported. Seven of every eight were athletes, and runners predominated. Exercise-induced symptoms of consistently recurring tightness, aching (in some, sharp pains) in anatomically defined compartments were pathognomonic. Mean months of symptoms prior to operation was 22; median age was 26 years. Bilaterality occurred in 82. The distribution of compartments was: anterior, 39%; lateral, 12%; and posterior, 48%. Incidental compartment pressures were elevated (mean = 23 mmHg). Fasciotomy using local anesthesia was performed on 70 outpatients. At a median of 4.5 months, over 90% were cured or significantly improved in symptoms and/or function. Median time to walking unassisted was 2 days, and to resumption of conditioned running 21 days. Fasciotomy can be a safe, effective, and economical treatment for chronic compartment syndrome.

Adult↗
PubMedDate not supplied

Infantile olivopontocerebellar atrophy with spinal muscular atrophy (infantile OPCA + SMA).

We report three siblings (two boys and girl) with familial (autosomal recessive) infantile olivopontocerebellar atrophy (OPCA) associated with lower motoneuron involvement. Brain autopsy findings in two of the children revealed a multisystem degeneration characterized by marked hypoplasia of phylogenetically new parts of the brain stem (basis pontis and inferior olivary nuclei) associated with hypoplasia of the neocerebellum, both cerebellar and cerebral peduncle. All three infants died before six months of age. The clinical features are characterized by severe hypotonia, areflexia, failure to thrive, respiratory insufficiency in all cases, cardiomyopathy and dislocated hips at birth in two of the three siblings. Extensive serum, urinary and leukocyte enzyme assays in the second infant failed to disclose a specific metabolic abnormality. The diagnosis of OPCA was established prior to death by Magnetic Resonance Imaging (MRI) in the youngest infant. Since OPCA represents a heterogeneous group of diseases, correlation of neuropathologic, clinical, genetic and MRI findings at early stages of evolution becomes crucial in the understanding of the nosology of OPCA and its variants.

Female↗