A case of HMN, distal type transmitted as autosomal dominant is described. Clinical findings appear to be consistent with a peroneal muscular atrophy, indistinguishable from HMSN types I and II. The ...electrophysiological data reveal a pathological involvement of the anterior horns, whereas sensory and motor conduction are normal. A muscle biopsy showed neurogenic atrophy, while the morphology of the sural nerve was normal.
An 18 year old man with fibrous dysplasia of bone and several endocrine abnormalities was investigated. Laboratory tests showed high levels of prolactin, TSH and GH and low levels of testosterone and ...LH. TRH and GnRH showed normal responsiveness of the pituitary in spite of the high hormonal levels. The circadian rhythms disclosed no abnormalities of hypothalamic control. The neuroradiological study revealed pathological tissue but no positive sign of a pituitary adenoma, although the sella turcica polytomograms did suggest erosion of the floor. Our data are compatible with the presence of a mixed adenoma or adenomas of the pituitary. We emphasize the importance of a correct study, including neuroradiological and neuroendocrine investigations, in patients with fibrous dysplasia of bone.
We have tested the feasibility of local intraarterial thrombolytic therapy in ischemic stroke in the carotid artery territories observed within 5 h of the onset of symptoms. Only 5 of 615 consecutive ...patients were enrolled. They were 1 man and 4 women aged 50 to 75 years. Angiography disclosed occlusion of the M2 tract in one, of the M3 tract in one, of the carotid siphon in one of M4 tract in two. Intraarterial urokinase was infused at a mean dosage of 560,000 units close to the site of occlusion. Four of them had partial or complete recanalisation at early angiographic control and were independent at 6th month control. The one who did not demonstrate recanalisation was confined to a wheelchair. One patient, who had recanalisation, sustained a hemorrhagic transformation of the brain ischemia not interfering with outcome. Our experience, at the light of the low rate of enrollment, despite the results, suggest that intraarterial thrombolysis may be a therapeutic tool for selected patients with stroke in the carotid artery territories and not a routinary treatment for them.
To evaluate the safety and possible efficacy of heparin sodium anticoagulation within 5 hours of the onset of first-ever nonlacunar ischemic strokes in the internal carotid artery territories.
Pilot ...study, prospective and open.
Inpatient stroke unit.
Of 360 stroke patients observed during 13 months, 45 (12.5%) were included in the study.
Heparin sodium was administered intravenously, starting with a bolus of 10,000 U, followed by continuous infusion over 4 days at a rate adjusted to maintain an activated partial thromboplastin time ratio between 2 and 2.5. The mean interval from stroke to treatment was 197 minutes.
Two patients had cerebral hemorrhage, one of which was fatal. None had extracranial major bleeding, while six had minor bleeding. The conditions of 23 patients improved, 16 patients were stable, and six patients worsened by day 1, while 29 patients improved, eight patients were stable, and eight patients worsened by day 7. Six patients died by the first month and five more by the sixth month. Twenty-one patients were self-sufficient, both at 1 and 6 months. Hemorrhagic complications were unrelated to any investigated factor. Multivariate analysis indicated that short-term outcome was predicted only by infarct size (P < .0001) and long-term outcome by infarct size (P = .002) and large vessel status (P = .0235).
Our study suggests that immediate heparin treatment for ischemic carotid stroke is feasible and generally safe and that patients whose conditions improve are those with smaller infarct size and no evidence of large vessel obstruction.
The motor and sensory conduction velocities were measured, and the somatosensory evoked potentials were recorded on median nerve stimulation in 20 epileptics on long-term carbamazepine therapy. The ...plasma drug levels were monitored and found to be consistently within therapeutic range. The electrophysiological study showed that the motor and sensory conduction velocities in the lower limbs were significantly lower than in age-and sex-matched controls, while no significant variation was found in the somato-sensory evoked potentials. There was no correlation between the neurophysiological data and any of the pharmacological data, such as duration of treatment, plasma levels and dose of drug per Kg of bodyweight per day.
The case of a "young stroke" affecting a 22 years old man is reported. Essential clinical features were drowsiness, headache, motor aphasia, right hemiparesis and Claude-Bernard-Horner in the left ...eye. An extensive investigative protocol was carried out which revealed an occlusion of the intracranial left ICA with subsequent recanalization. The possible causes of the stroke (migraine, dissection or combination of both) are discussed and the importance of a complete evaluation of patients of this kind is stressed.
OBJECTIVE To investigate the frequency of cancer developing in patients with peripheral sensory neuropathy of unexplained cause. DESIGN Prospective study. SETTING A neurologic unit in a general ...hospital. METHODS Following the diagnosis of neuropathy, we searched for occult malignancy. This search was repeated together with neurologic evaluations every 6 months thereafter. Patient recruitment began January 1, 1988, and ended December 31, 1995. The end point of the study was December 31, 1996. RESULTS In the study period, we observed 363 patients with peripheral sensory neuropathy. Of these, 53 patients without any identified cause of neuropathy were invited to participate in the study. Of the 53, 2 patients refused. Thus, we examined and followed up 51 patients, 42 men and 9 women, with a mean age of 64.5 years (range, 19-80 years). The range between the onset of neurologic symptoms and the diagnosis of neuropathy was 2 to 72 months (mean, 13.9 months). The follow-up period ranged from 14 to 94 months (mean, 51.4 months). In 18 patients (35.3%) (16 men and 2 women)whose mean age at diagnosis of neuropathy was 66.5 years. malignant growths were found 3 to 72 months (mean, 27.4 months) after the onset of the neuropathy. The cancer was in the liver in 4 patients (all had a primary hepatoma), the bladder in 3, the lymph nodes in 3 (all with non–Hodgkin lymphoma), the prostate gland in 2, the lungs in 2 (small cell lung cancer in both), the breast in 1, the pancreas in 1, the sublingual gland in 1, and the bone in 1 (a metastatic sarcoma). CONCLUSIONS More than one third of the patients with peripheral sensory neuropathy of unexplained cause developed cancer without any predominating type of malignancy.Arch Neurol. 1998;55:981-984-->
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