Biomedical subjects
Publications and source records attributed to A Fiaschi.
A man who had had an episode of localized encephalitis, diagnosed as viral cerebellitis in childhood came to observation at the age of 23 for attacks of segmental myoclonus of the cervical axial musculature. BAEP changes at brainstem level in this case point to involvement of the Guillain-Mollaret triangle in the pathogenesis of this syndrome.
In a patient with AIDS presenting partial epilepsy cerebral toxoplasmosis was diagnosed on the serological and CT evidence. The diagnosis was confirmed by the immediate response to sulfonamide therapy.
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The short latency auditory electrical response (BSER) was evaluated in 15 patients suffering from spino-cerebellar atrophy (SCA). All patients had normal hearing and presented with various degrees of neurological involvement. BSER findings along with clinical symptoms furnished the following grouping of results: 1 Patients with recent and mild symptomatology showed excellent intratest stability, normal III-V and prolonged I-V and I-III interpeak values. 2 Patients with severe symptoms dating back 10-15 y showed low intratest stability, prolonged latency of waves I, III and V, along with remarkably lengthened I-III and III-V interpeak intervals. 3 Patients with moderate to severe symptoms, dating back 10-15 yr showed good stability, absence of wave II and IV, abnormally long I-III intervals and reduced III-V intervals.
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4 patients with prosopagnosia are described. They presented associated neuropsychological deficits (achromatopsia, topographical disorientation) and visual field defects. In 1 case, gross pathological examination revealed bilateral symmetrical infero-medial occipito-temporal infarcts. In 2 other cases, computer tomography (CT) demonstrated bilateral inferior temporo-occipital infarction. In 1 case CT showed only occipital infarctions in the occipital lobes. This brings to 16 the number of anatomically studied cases of prosopagnosia (8 by necropsy, 8 by CT). In contrast to clinical localization, which often indicates only a right cerebral lesion, all anatomically verified cases of prosopagnosia exhibit bilateral lesions in the posterior cerebral artery distribution.
Sural or superficial peroneal nerve biopsies of patients with clinical diagnosis of Friedreich's ataxia were studied. Patients were divided in two groups, typical and abortive forms: loss of fibers accompanied by axonal atrophy and segmental demyelination are the basic changes in both groups, although the decrease in number of myelinated fibers was most severe in typical FA. In the cases with slower progression there is a tendency to form onion bulb complexes.
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Severe weakness of lower cranial and girdle muscles has been found in 4 subjects among 20 dialysis patients treated with carnitine in order to correct their high plasma triglyceride level. Neurophysiological investigation showed an impairment of neuromuscular transmission with short-term reduction of evoked muscle responses to repetitive stimulation and the presence of "post-activation exhaustion phenomenon" 3 min after the train. Both the weakness and the neurophysiological findings were less pronounced after edrophonium. These findings suggest a hemicholinium activity of carnitine at synaptic level.
In 3 patients with sequelae of brachial plexus birth injury we observed associated movements and also synchronous motor unit potentials and motor axo-axonic reflexes between different muscles, sometimes with antagonist function. To explain these synkineses is necessary to assume that there is simultaneous innervation by the same motoneurone of 2 or more "motor subunits" in different muscles. Therefore we suggest that the motoneurone branches originate proximally from the primary trunk of the brachial plexus. Careful clinical and neurophysiological study can show these complex simultaneous innervations and also suggest correct kinesiotherapy.
The authors describe one case of lipoid granuloma of the lung in a patient with PARKINSON'S disease. The cause of this pulmonary sequel is probably to be found in the alteration of the mechanism of deglutition, typical of PARKINSON'S disease, with aspiration bronchopneumonia and subsequent evolution to granuloma formation. In the case presented (a man of 64) the X-ray picture resembled the irregular mass opacities and bronchial amputations characteristically associated with cancer of the lung. As the actual anatomical extent of the process exceeded the limits indicated by radiological examination, the whole left lung was removed at surgery; histological studies, however, revealed the essentially benign nature of the lesion. The patient was discharged in due course of time as surgically cured. There was no major deterioration of his neurological status either at discharge time or at later rechecks.
Radicular paralysis is the most frequent, though usually less serious, complication of Herpes Zoster, being observed in rather less than 5% of patients. The site affected--leaving aside exceptional cases--it that of the gangliar and radicular district where the eruption occurs. A clinical and neurophysiological study was made of 5 patients with loss of strength and trophism on the part of muscles in the eruption site within 2 to 5 weeks after the onset of Herpes. Routine blood chemistry tests were run, together with spinal puncture in three cases. The EMG data pointed to variously severe neurogenic distress referable to myeloradicular involvement. Subsequent clinical and EMG controls demonstrated gradual, though slow improvement. This finding is in line with the literature data.
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A man had a stroke 27 years after radiation therapy to the neck for treatment of laryngeal papillomas. There were premonitory symptoms suggesting cerebral ischemia. In contrast to many previously reported cases, in our patient there is a strong relationship between radiation therapy and stroke. Pathoanatomic study of the surgical vascular specimen strongly suggests that radiation is a potentially modifiable cause of delayed stroke.
The neurophysiological findings obtained with standard electromyography (EMG) and single fiber EMG (SFEMG) in a case of hypokalemic periodic paralysis (HoPP) are reported. During the period between paralytic attacks the only abnormalities consisted of scanty fibrillation potentials and, with SFEMG, a fiber density increase. In the first stage of an induced paralytic attack the most striking feature was decrease in fiber density, slight increase in jitter with several blocks. These results indicate a failure of the membrane surface to propagate an action potential. In some fibers the block is likely to be permanent, thus explaining the decrease in fiber density. The jitter increase is due to a slight abnormality at the synaptic site or to a variation in the propagation velocity of the muscle fiber.
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