Biomedical subjects
Publications and source records attributed to F Barbieri.
We report clinical features of two patients suffering from Locked-In Syndrome (LIS) who made a moderate recovery, some years after the onset: a 29-year-old man, with mitral valve stenosis, and a 41-year-old diabetic woman. CT scan findings showing cerebellar and brain stem atrophy, as well as hypodensity areas in the ventral pons, are described.
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The bioptical morphometric and ultrastructural study of sural nerve in a 17-year-old boy with ataxia-telangiectasia (AT) is reported. Our findings include a loss of fibers, particularly of large ones, axonal degenerative changes, Schwann cell inclusions of various type, and rare signs of primary demyelination. Teased-fiber study showed paranodal myelin enlargements, segmental demyelination, shortening, and/or variability of internodal length. This picture is similar to that in Friedreich's ataxia (FA), although they differ in degree and time of onset. A correct neuropathologic diagnosis of AT cannot be made on the basis of sural nerve biopsy alone.
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We report a further case of human placental lactogen (hPL) deficiency associated with a normal pregnancy. To our knowledge this is the first report in which the examination of placenta by means of the immunoperoxidase technique confirmed that hPL was absent from the trophoblast.
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The clinical hematologic and pathologic findings of 30 patients with hairy cell leukemia observed between 1966 and 1979 were studied. Twelve patients had long-lasting course of the disease. Seven of them displayed a survival greater than or equal to 120 months, whereas 18 patients died within 36 months of the diagnosis. Their clinical and laboratory characteristics (age of onset, sex, ESR, hemoglobin, WBC, neutrophils, monocytes, platelets, spleen and liver size) were analyzed to ascertain possible prognostic features. Multivariate discriminant analysis, performed both with a direct method and with a stepwise method (Wilks' method), provided a discriminant function able to correctly predict the prognosis of the disease in 83.3% of the examined cases. Spleen size, neutrophil count, age of onset, ESR and liver size turned out to be the most important prognostic factors; in contrast, splenectomy did not significantly affect the prognosis in our cases.
This paper deals with the histopathology of the levator muscle of the upper eyelid obtained in three cases of ptosis due to 3rd nerve palsy. Light- and electron-microscopy show muscle fibers decreased in number and altered, showing vacuolation, mitochondrial degeneration, large glycogen amounts, disorientation of myofibrils and Z-line changes. The findings are discussed and compared to the data reported by others.
We report data on 3 members of a family affected by a dominantly inherited disorder closely resembling Roussy-Levy syndrome (RLS). Electrophysiological findings showed a marked decrease of motor and sensory conduction velocities and EMG signs of mild neurogenic damage. Light and electron microscopy of sural nerve biopsy showed a hypertrophic neuropathy with diffuse onion-bulb formations and marked decrease of large size fibers. Teased fiber preparations evidenced reduced internodal lengths and segmental demyelination. Other data from the literature on RLS are reviewed and discussed. The hypothesis that RLS is not a disease entity but a hypertrophic-type of Charcot-Marie-Tooth disease with essential tremor (HMSN type 1) is strongly supported.
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Electromyography was performed, and motor and sensory nerve conduction velocities were measured in 19 patients definitely affected by Friedreich's ataxia. Biopsy of the sural nerve was also performed in 9 patients. Most patients presented a moderate to severe loss of motor units, a significant increase in mean duration of motor unit potentials, and in the incidence of polyphasic potentials. Short-lasting spontaneous activity was rarely seen. Conduction velocity along the motor and sensory fibres of the median and tibial nerves was moderately slowed, while distal conduction time to muscle was significantly increased and the sensory orthodromically-evoked response markedly reduced. Intraoperative electrophysiological recordings obtained during biopsy of the sural nerve in 4 patients were consistent with the changes conventionally observed in the median, tibial and sural (6 patients) nerves. Quantitative histology revealed a reduced number of total myelinated fibres with a severe loss of large fibres, and a moderate loss of fibres of less than 7 microns in diameter. In teased nerve fibre preparations, the most evident abnormality consisted of fibres with uniformly short internodal length, while signs of remyelination were less prominent. Signs of active axonal degeneration were rarely observed in electron microscopy. Electrophysiological and histological findings were uniformly distributed, and the changes were neither related to the duration nor to the severity of the clinical condition.
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