Biomedical subjects
Publications and source records attributed to L S Adelman.
A 64-year-old woman had bilateral facial weakness, mild peripheral neuropathy, incoordination, and impaired balance. A corneal transplant had been performed for corneal lattice dystrophy. Sural nerve biopsy showed mild axonal neuropathy with deposits of amyloid in the perineurium. Histochemical studies showed amyloid protein AA in the nerve, but not in cornea or muscle.
In spinal cords from seven amyotrophic lateral sclerosis (ALS) patients and four controls, we found no difference in thyrotropin-releasing hormone (TRH) concentration relative to protein content, but there was a reduction per tissue wet weight in ALS. Immunohistochemical localization of TRH in ALS cord was unaltered. Histidyl proline diketopiperazine (HisPro-DKP), a possible metabolite of TRH, was significantly elevated per protein content in ALS. CSF levels of TRH and HisPro-DKP were unchanged. These findings suggest that TRH neurons are not primarily affected in ALS, but TRH and tissue protein are lost together as the disease progresses.
Four patients with inclusion body myositis were treated with 150 rad of total body irradiation given in 5 weeks. One patient responded subjectively and transiently, but no patient showed clear benefit. This treatment is not recommended for inclusion body myositis.
A 51-year-old woman with an inherited polyneuropathy had fatiguability and a dramatic historical response to prostigmine. Repetitive motor nerve stimulation produced a prominent decrement of the compound muscle action potential in distal muscles with marked facilitation after brief exercise. Defective neuromuscular transmission paralleled the polyneuropathy in distribution and severity. We hypothesize that deficient release of acetylcholine by regenerating or degenerating nerve terminals likely caused the defect of neuromuscular transmission in this patient.
Six patients had a syndrome of painful lumbosacral plexopathy and elevated erythrocyte sedimentation rate. Sural nerve biopsy in each case showed axonal degeneration and epineurial arterioles surrounded by mononuclear inflammatory cells. Differential fascicular involvement suggested an ischemic cause in three nerves, but no patient had a necrotizing vasculitis. None of the six patients had vasculitis or cancer. Three of the six were diabetic and were initially thought to have diabetic plexopathy, but deterioration continued despite control of the diabetes. These six patients appeared to have an ischemic neuropathy with an immunological basis. Five were treated with immunosuppressant drugs, and in four the plexopathy improved or was arrested. The pathogenesis is unclear, but postmortem findings in one case suggest that the syndrome does not stem from an underlying vasculitis.
Phrenic nerves of 11 patients with amyotrophic lateral sclerosis studied postmortem contained only 33% of the normal number of large myelinated fibers (9 controls; p less than 0.001). In the phrenic nerves of these patients, there were 18% fewer large myelinated fibers in the distal segment than in the proximal segment (p less than 0.025). The ratio of axonal circumference to myelin lamellae in large myelinated fibers in the distal segment was 34% greater than that in control fibers (p less than 0.002). The proportion of acute axonal degeneration was the same at all levels (48.0 +/- 13.7%). Sural nerves of 21 patients with amyotrophic lateral sclerosis had more acute axonal degeneration and 30% fewer myelinated fibers (p less than 0.05) than controls; evidence of degeneration also extended to unmyelinated fibers. The amount of axonal transport of acetylcholinesterase in 9 sural nerves determined in vitro was reduced by 24% (p less than 0.05) and the apparent transport rate was reduced by 44% (p less than 0.01) compared with 4 controls. These findings show that in amyotrophic lateral sclerosis a small degree of dying-back change and of distal axonal atrophy is superimposed on the degeneration of motor neuron cell bodies, and that the disease effects spread beyond the motor neurons.
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A patient developed conjugate horizontal eye deviation to the side opposite a large frontal-perisylvian hemorrhage from a ruptured aneurysm. Autopsy examination confirmed the hemispheric locus of the hemorrhage and failed to disclose any lesion of the brainstem or thalamus to explain the "wrong-way" eye deviation.
Thirty women with prolactin (PRL)-secreting adenomas underwent selective adenomectomy via a transsphenoidal route. All had abnormal sella polytomes or visual fields, amenorrhea with low basal serum gonadotropin levels despite decreased serum estradiol concentrations, and elevated basal serum PRL levels with blunted PRL response to neuroendocrine stimulation tests )thyrotropin-releasing hormone, levodopa, chlorpromazine, and hypoglycemia). Of 17 patients with microadenomas, 14 (82.4%) were cured and three (17.6%) improved. None were unchanged or worse. Three (60%) of five patients with larger, but still intrasellar tumors, had normalization of PRL levels, return of menses, and resolution of galactorrhea. The patients with tumors extending out of the sella did not fare as well. Overall, 21 (70%) were cured, six (20%) improved, two (6.7%) were unchanged, and the condition of one (3.3%) became worse. All preoperative neurologic deficits resolved. Postoperative complications were minimal with no neurologic morbidity. When tumors are small, surgical results are excellent with minimal risk.
Orange G-hematoxylin stains performed on frozen sections from the sella turcica had a diagnostic accuracy of over 90% when compared to permanent sections on the same tissue. This technique allows the pathologist to make an intra-operative contribution to the establishment of tumor margins in the selective removal of pituitary adenomas.
The authors present the case report of a patient in whom hypopituitarism, diplopia, and visual field defects were due to eosinophilic granuloma. Radiographic studies were suggestive of a pituitary tumor and subsequent histological evaluation showed replacement of the anterior pituitary by histiocytic cells. Eosinophilic granuloma should be included in the differential diagnosis of an intrasellar mass.
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A patient with dialysis cachexia became paraparetic within hours of therapeutic renal artery embolization. At autopsy, emboli of absorbable gelatin sponge filled spinal arteries, accompanied by spinal cord infarcts. The renal arteries of patients with end-stage renal disease are smaller than normal becaue of intimal fibrosis associated with prolonged dialysis. Embolic material can reflux more readily in these patients than in patients with renal tumors.
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While recovering from tuberculous meningitis, a 5-year-old boy developed a profound visual deficit because of optochiasmatic arachnoiditis. Following microneurosurgical decompression of the optic nerves and chiasm, prompt visual recovery occurred.
A patient with a small infarct located posteriorly in the internal capsule had 9 years of weakness of the contralateral face, arm, and leg. At necropsy, it was found that degeneration of the corticospinal tract was almost complete in the midbrain and medullary pyramid. This case supports the increasing evidence that the human pyramidal tract is located in the third quarter of the posterior limb of the internal capsule.