Biomedical subjects
Publications and source records attributed to G Bogliun.
We report the case of a patient affected by contralateral hemiplegia during herpes zoster ophthalmicus (HZO) evaluated both with serial CT scans and with MRI. We suggest that MRI examination of patients affected by HZO could be useful for the detection of early signs of cerebral arterial damage which are not yet clinically and radiologically apparent.
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Adrenoleukodystrophy (ALD) is a sex-linked disorder characterized by very long chain fatty acid accumulation in different tissues, but mainly in the adrenal cortex, gonads and nervous system. The typical symptoms are hypoadrenalism, hypogonadism and central and peripheral nervous system impairment due to demyelination. On neurological grounds visual and hearing loss associated with quadriparesis are the most common symptoms, onset in childhood and a rapidly progressive course leading to a vegetative state and death. We report the case of a 31-year-old man affected by ALD, whose neurological symptoms started at the age of 12 and showed a markedly slow progression during the following years.
We describe a case of aphasia associated with ideomotor apraxia due to a CT-proven ischemic lesion strictly confined to the dominant hemisphere white substance at the junctional zone of Donnan. The aphasia did not fit any of the known patterns. At first severe, it cleared in 5 days, while the apraxia remained unchanged 25 days after onset.
A radiological diagnosis of hemorrhagic infarction (HI) was made in 41 of 2726 cases with cerebrovascular lesions (1.9%). The clinical records of the cases and those of 82 age- and gender-matched subjects with ischemic infarction were examined, and notes of the principal risk factors of cerebrovascular disorders, the clinico-radiologic features and the outcome of the disease were taken for comparison. Cardiac sources of emboli (atrial fibrillation, native or prosthetic valve disorders, recent myocardial infarction) were present in 44% of cases and in 24% of controls. Diabetes mellitus was recorded in 31% and 18% respectively. Thirteen percent of cases and 35% of controls gave a history of transient ischemic attacks. Stupor or coma during the acute phase and a more severe course were more common among cases. In general, HIs were significantly larger than ischemic infarcts, with mass-effect, although the size of the lesion did not seem to be related to the presence of cardiogenic embolism.
The prevalence and characteristics of polyneuropathy were assessed using standard clinical and electrophysiological criteria in 39 consecutive outpatients with primary hypothyroidism, 15 of whom were previously untreated. Subjective complaints, mainly paraesthesiae, were recorded from 25 cases (64%) and objective findings supporting a clinical diagnosis of polyneuropathy were present in 13 (33%). Using standard electrophysiological criteria, a definite diagnosis of polyneuropathy was made in 28 cases (72%). The commonest sites of abnormal nerve conduction were the sensory nerves, especially the sural nerve. Polyneuropathy was generally mild. None of the clinical and biochemical indicators of hypothyroidism were significantly correlated with the electrophysiological signs of peripheral nerve impairment or the diagnosis of polyneuropathy.
26 patients with ataxic hemiparesis syndrome (AHS), due to acute ischemic cerebrovascular disease, have been submitted to clinical and electrophysiological evaluation, in order to assess the frequency of sensory disturbances in this condition. Sensory impairment were present in 78% and SEP abnormalities in 54% of the patients, while they were entirely absent in 23% of them. Lesions responsible for AHS, detected by CT scan, were mainly located in the thalamus, capsula interna, subcortical white matter, centro parietal cortex; sensory and SEP changes were more frequent in gross infarct involving the cortex and in smaller infarcts involving the thalamus, less frequent in the lacunar infarcts of the capsula interna and subcortical white matter, relatively rare in patients with CT scan without hypodense lesions. Although a statokinesthesic defect and/or major SEP abnormalities were often present (38% of patients), our findings do not support the view that they are involved in the pathogenesis of the ataxia, which may rather be attributed to a derangement of cerebro-cerebellar and cerebello-cerebral connections.
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20 new cases of ataxic hemiparesis syndrome (AHS) are reported and the findings compared with those of published cases. AHS may be due to lesions either of the brainstem or of supratentorial structures, where motor fibers run together with the cerebro-cerebellar pathways. Specific syndromes related to the lesion site cannot be identified from the clinical signs. Ischemic infarct is the most frequent cause of the syndrome, but hemorrhagic, neoplastic and demyelinating lesions have also been reported.
The influence of chronic antiepileptic treatment on bone metabolism has been investigated in 52 adult epileptics, who had normal dietary intake, sunlight exposure and daily living activities. None of the patients had symptoms or signs suggestive of osteomalacia. Serum phosphate levels were significantly decreased and serum alkaline phosphatase levels were significantly increased in the patients compared with matched controls. Calcitonin values and bone mineral content, measured by single photon absorptiometry, were significantly lower among anticonvulsant users. Calcium metabolism impairment grossly correlated to the number of drugs concurrently used by the patient but not to the types, to the relative plasma concentrations or to the overall duration of the treatment. Our findings indicate that in ambulatory patients with adequate diet and outdoor activities in Italy present with clinically irrelevant impairment of bone metabolism.
The distribution of the motor impairment was correlated to the site and extension of the lesion of 18 patients with restricted lesions of the internal capsule, detectable with computerised tomography (CT). In three cases the lesion was located in the anterior limb of the internal capsule and the patients did not suffer any motor defect. Among the 15 patients with lesions in the posterior limb, 13 presented a distribution of the motor weakness consistent with a location of the corticobulbar fibres in the genu and corticospinal fibres in the anterior half of the posterior limb. Our CT study confirms the classical view on the location of the pyramidal tract in the internal capsule of man, but also evidences the possibility of individual variations.
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One hundred forty-one adult patients treated for no less than 6 months with standard daily doses of the commonest antiepileptic drugs (AEDs) were recruited in five Italian centers and submitted to intensive clinical and electrophysiologic investigation to assess the effects of AEDs on peripheral nerves. Eighty percent of the patients were receiving monotherapy. Carbamazepine (CBZ) was the most common AED (51 cases), followed by phenytoin (PHT) (46), phenobarbital (PB) (42), and valproate (VPA) (25). Fifty-three percent of the patients had one or more symptoms of polyneuropathy (paresthesias being the most common complaint). The neurologic examination was abnormal in 32%. Electrophysiologic findings in two or more separate nerves were abnormal in 77 patients (54.6%); of these, 27 (19.1%) had abnormal neurologic findings and 21 (14.9%) also had symptoms of polyneuropathy. Sensory functions were most frequently impaired. Sural nerve biopsy was performed in 4 patients receiving monotherapy with CBZ, PHT, PB, and VPA. Except in patients receiving VPA (in whom no morphologic abnormalities were detected), mild predominantly axonal damage with secondary myelin changes was noted. A correlation was noted between polyneuropathy, age of the patient and, to a lesser extent, receipt of two or more AEDs.
The classical thalamic syndrome of Dejerine and Roussy is very rare. Sensory disorder are almost constant but the thalamic hyperpathia had not been reported. Clinical findings are reported in 14 patients with restricted lesions of the thalamus visible with the CT scan. Occasionally aphasia was found in patients with left sided lesion (with Thalamic Neglect, apraxia, mainly of ideomotor type and the so-called "Central Dazzle"). Thalamic lesions can produce a great variability of signs and symptoms.
We evaluated clinically and neurophysiologically the immediate and long-term involvement of the peripheral nervous system in 22 selected patients with epithelial ovarian cancer successfully treated with DDP alone or in combination with non-neurotoxic drugs. While the motor nerves were unaffected, generally the involvement of sensory nerves was more severe at the examination performed several months after DDP discontinuation than at the evaluation performed after the "induction phase". We conclude that up to now the importance of long-term DDP-induced peripheral nerve damage has probably been underestimated. DDP-induced long-term damage is at least as severe as the immediate toxicity and, moreover, it is likely that complete recovery can occur, if ever, only years after DDP discontinuation.