Biomedical subjects
Publications and source records attributed to P A Hanson.
This article is a review of the current technique and materials used in the restoration of endodontically treated teeth with post and core materials and procedures. It is intended to aid clinicians in making selections which are appropriate to their individual practices through the evaluation of relevant literature about the restoration of endodontically treated teeth.
Thyrotropin-releasing hormone has been reported to increase strength in patients with amyotrophic lateral sclerosis (ALS). DN-1417 is an analog of thyrotropin-releasing hormone, which has less endocrinologic activity, but more anterior horn cell stimulating effect (with no "autorefractory state"). However, 2 mg DN-1417, IM twice a day for 1 month in an open-label trial, produced no objective improvement of strength in nine patients with ALS. No patient entered the double-blind, placebo-controlled phase of the trial.
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From a kinship with a dominantly inherited motor-sensory neuropathy, we studied 2 brothers with brainstem auditory evoked potentials and behavioral audiometric tests. They had abnormal prolongation of I-III interpeak intervals. Wave V was poorly developed. Conventional audiometric tests did not reveal a peripheral hearing loss. It is probable that their auditory nerves and spiral ganglia are undergoing a pathophysiological process analogous to that of their peripheral nerves.
Sixteen patients with Reye syndrome had diminished concentration of serum complement proteins and/or hemolytic activity in the earliest blood sample. All 12 studied with hemolytic methods had significantly reduced C1 activity; total hemolytic complement activity was reduced in only three. Low Cl activity was accompanied by equivalent reduction of Cls in 11 of 12 patients; Clq was less than normal in only two of 12. Decreased levels of at least one other classical pathway complement hemolytic activity or protein concentration were found in 13 patients, whereas factor B or the alternate complement pathway was normal or elevated in the ten patients studied. The consistent reduction of Cls protein concentration in Reye syndrome suggests that early metabolic abnormalities regularly affect the production or catabolism of this protein. Although normal serum Clq concentration in the majority of these patients does not support an immune pathogenesis, decreased Clq, C4, and C2 in three patients does suggest that immune mechanisms may be responsible for the serum complement abnormalities in this latter group of patients.
In three patients hemiparesis was a manifestation of focal seizures. In all, there were electroencephalographic abnormalities and radionucleotide uptake in the contralateral hemisphere. Treatment of the seizures resulted in clearing of both paralysis and abnormal brain scan. These patients fit the diagnostic category described by Higier as a "paralytic equivalent of epilepsy without disturbance of consciousness in the form of status hemiparalyticus," and described by others as inhibitory seizures or partial seizures with subsequent hemigeneralization. The etiology of the paralysis and its relation to Todd paralysis are not clear, but it may result from excessive inhibitory discharge.
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Thirteen sequential percutaneous skeletal muscle biopsy specimens from 4 patients with Reye's syndrome were studied ultrastructurally and by direct immunofluorescence. Prominent generalized intermyofibrillar edema, mitochondrial disruption, and swelling of vascular endothelium were demonstrated in 2 patients. In all 4 patients there were granular deposits of immunoglobulins G and M in intramuscular vessels, presumably representing antigen-antibody complexes. We postulate that these changes may be pathogenetically important in this syndrome and suggest that muscle biopsy tissue be utilized for further investigation of Reye's syndrome.
An eight-year-old girl presented with a bizarre posture and gait and was diagnosed as having hysterical astasia-abasia. She walked with marked lordosis and head hyper-extended, and would walk backwards across the room to a bed or chair for support. She had a midline astrocytoma and the disorder was relieved with removal of the tumour. The unusual symptomatology accompanying many midline lesions is re-emphasized.
An infant born with marked hypotonia showed prompt regression of skeletal muscle weakness, but by 7 weeks of age had total external ophthalmoplegia. Biopsy of the gluteus muscle at 14 days showed marked variation in fiber size with a large proportion of very small fibers (less than 3 mu). By 10 months of age, biopsy of the vastus was virtually normal. The inferior oblique muscle was replaced by fibrous tissue containing a few remaining degenerating fibers. The child was normal at 2 years of age except for mild facial weakness and ophthalmoplegia. This syndrome may be the result of a reversible intrauterine process.
Herpes simplex virus was isolated from the tracheal aspirate of a 10-year-old boy presenting with acute onset of multiple cranial nerve palsies and a mild right hemiparesis. There was also an elevated herpes complement-fixation titer with decrease in the following weeks infection by herpes virus have been debated, we propose that this represents a case of brain-stem encephalitis due to herpes simplex infection. The importance of early diagnosis and evaluation of therapy are emphasized by this case in which the patient recovered completely.
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A 23-year-old ex-bicycle racer with vague gastrointestinal symptoms had an acute weight loss of 13.6 kg (30 lb). During the period of maximal weight loss, he experienced muscle weakness, dysphagia, bradycardia, and T wave changes on the electrocardiogram. His skeletal muscle biopsy showed a severe vacuolar myopathy devastating the atrophic type II fibers. Without treatment, he recovered completely and has remained well. This may be an exaggerated or acute form of type II atrophy not previously reported, or it may represent an acute muscular degeneration.
Two adolescent brothers were studied because of cramps in the legs following strenuous exercise. The symptoms were maximal at adolescence and later subsided. Serum creatine phosphokinase elevations were significant. Examinations of muscle biopsy material revealed changes compatible with a myopathy. The family consists of seven children. All have eventually developed elevations of serum creatine phosphokinase, which appear to reach the highest point during adolescence. This familial disorder may represent a new myopathy with maximal penetrance at adolescence.
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