The coexistence of Atrial Natriuretic Factor (ANF) and Angiotensin II (Ang II) receptors in the subfornical organ suggested that ANF may influence the action of Ang II at this level. To test this hypothesis the effect of ANF pre injected into subfornical organ (SFO) on Ang II-induced vasopressin release was investigated in rats. ANF blunted vasopressin release elicited by circulating Ang II. This study indicates SFO as one of the main sites at which ANF-Ang II interactions occur.
Hyaluronan (HYA) is a large molecular weight polysaccharide which functions in various roles throughout the body. Little is known regarding HYA in human cerebrospinal fluid (CSF). We measured CSF and serum HYA concentrations in eleven patients with varying degrees of head injury. In these patients, CSF HYA ranged from 46 to 772 micrograms/l and serum HYA from 16 to 573 micrograms/l. We did not find any relationship between HYA values in CSF or serum and underlying disease or Glasgow Coma Scale (GCS). The role of CSF HYA in the injured human brain has yet to be elucidated.
To evaluate effect of the calcium-channel blocker nicardipine in essential tremor (ET), 14 patients with ET affecting upper limbs underwent a cross-over study with nicardipine (1 mg/Kg/day) and propranolol (160 mg/day). Evaluation (baseline and 1 month after each drug) was done by using the Fahn-Tolosa-Marin scale. Two patients withdrawed nicardipine (pretibial edema and palpitations) and other 2 propranolol (dyspnea and hypotension). Both drugs improved ET, with a non-significantly higher efficacy of propranolol. These results suggest that nicardipine might be efficacious for ET.
The case of a 32-year-old man with a giant, partially thrombosed, basilar artery aneurysm associated with moya-moya disease is described. The patient exhibited symptoms due to compression of the aneurysm on the right cerebral peduncle. He was discharged after conservative treatment for 10 days. Pathogenesis and management of this rare case are discussed in the light of data reported from the literature.
Two cases of primary antiphospholipid antibody syndrome are reported. One patient presented multiple abortions and epilepsy. The second patient was affected by a brain vascular accident, with a residual hemiparesis. Both cases showed livedo reticularis in arms, NMR evidence of diffuse lesions of the white matter, high serum levels of anticardiolipin antibodies and cardiopathy. Lupus anticoagulant was also found in the serum of the first patient, and cortisone and antiaggregants enabled her to reach term in a fifth pregnancy after four miscarriages. In the other case histological examination of specimens of skin, peripheral nerve and skeletal muscle revealed occlusive, non arteriosclerotic vasculopathy and an absence of inflammatory lesions. Histological study has rarely been performed in primary antiphospholipid syndrome but suggests that the mechanism of thrombosis is not vascular; in our subjects it revealed findings similar to those in Sneddon syndrome.
Literature suggests that not only homozygotes but also heterozygotes for ataxia-telangiectasia have a high incidence of cancer, probably due to an impairment in DNA repair. The most frequent associations are with breast, lung, bladder, prostate and stomach tumors, while no correlation with colorectal cancer has been demonstrated. The affected family reported in this paper seems to have a high incidence of gastrointestinal tract tumors, including the colorectal ones.
The Authors report on 150 cases of patients suffering from Parkinson's disease. The patients represent 8.3% of total parkinsonian patients and are selected on the basis of clinical and laboratory criteria that allow the diagnosis of "angiopathic parkinsonism" as an autonomous entity. Cerebral blood flow alterations in Parkinson's disease and associated dementing symptoms are also discussed. In conclusion, the Authors emphasize the usefulness of recognizing the "angiopathic parkinsonism" in view of therapeutical implications.
The frequent negativity of neuroradiological changes after neck injury despite the constant complaints (neck pain and stiffness, limited neck movements, arm pains) experienced after a motor vehicle crash has led us to verify the diagnostic and prognostic validity of different neurophysiological tests in these patients. To this aim 120 young patients (67 females and 53 males) referred for a whiplash syndrome aged 18-31 years without neuroradiological abnormalities have been submitted to EMG and BAEP studies immediately after the traffic accident and after six months. Constant slowing of median MNCV and SNCV and ulnar SNCV without changes in morphology, amplitude and duration of MAP and SAP have been observed in 92 patients, with persistent abnormalities in 64 cases after six months. Increase in I-III or I-V interpeak interval of BAEP have been observed unilaterally in 45 patients and bilaterally in 32 ones without changes in absolute latencies and V/I amplitude ratio, with persistence of such abnormalities in 31 and 16 patients respectively after six months. Simultaneous abnormalities of all neurophysiological tests have been observed in 31 patients, with unmodified recordings after six months in 24 patients. The above findings could be accounted for subtle cerebral lesions and a possible damage to brain stem structures as claimed by Ettlin et al. (1992). The usefulness of the above proposed neurophysiological tests in assessment of asymptomatic dysfunction of central motor and sensory pathways in whiplash injuries is discussed.
A case of idiopathic superficial hemosiderosis (SH) of the central nervous system and a review of the literature are presented. The patient suffered from progressive cerebellar ataxia, hearing loss, anosmia, spastic paraparesis, but no mental deterioration. The diagnosis was made with brain and spinal MRI, that showed in T2 weighted images superficial hypointensity of spinal cord, medulla oblungata, pons, mesencephalon, cerebellum and cerebral hemispheres, images that are considered pathognomonic of SH. Repeated spinal fluid examinations were negative, suggesting that evidence of overt subarachnoidal bleeding is not essential in the diagnosis. In patients with SH of unknown etiology no valid therapy is yet available.
A 21-year-old female patient with psychotic symptoms developed hyperthermia, muscular rigidity and hypertension after administration of haloperidol. A muscle biopsy showed some atrophic and necrotic fibers, and a great number of fibers with central cores in the oxidative enzyme preparations. A related syndrome, Malignant Hyperthermia (MH), is sometimes associated with central core disease. The present case shows an association of a hyperthermic syndrome related to haloperidol with central core disease.
In a retrospective case-control study of multiple sclerosis (MS), set up with the aim of verifying the role of environmental factors in pathogenesis, we included a section on dietary habits. We compared the frequency and pattern of food consumption by cases and controls. We found an association between. MS and high consumption of bread and "pasta", butter and lard, legume soup, horse flesh, coffee and tea in the period from infancy to adolescence. A different pattern of consumption of eggs, wine and mineral water between cases and controls was found during adulthood. Our results support, at least partially, the data already reported in the international literature on an association between certain dietary factors and MS. Some foods consumed at certain "critical" ages could play a causal role in the onset of MS.
Multiple sclerosis presenting at onset with clinical signs and CT picture suggesting a cerebral neoplasm or a slowly evolving stroke, is uncommon. We report one patient in whom cerebral tumour was suspected not only clinically but also from CT features. M.R.I., demonstrating multifocal, high intensity, unsuspected periventricular and white-matter lesions, permitted to diagnose what seemed an intracranial left parietal neoplasm as an acute plaque of demyelination. In fact, in the follow-up, this large low-intensity left parietal area acquired structural and volumetrical homogeneity compared to all other present hyperintense lesions. Instead multimodal EPs, though disclosing subclinical dysfunction in the early stage of the disease, did not correlate with changes in neurological examination to follow-up. So they, unlike the M.R.I., seem of no particular usefulness to serially monitor the clinical modification of MS.
A case of congenital lipodystrophy complicated by complex-partial epilepsy is reported in a nine-year-old girl. The peculiarity of this rare case is represented by partial complex epilepsy with diffuse electroencephalographic alterations represented by a continuous seizure-like pattern that persisted unmodified despite the successful antiepileptic treatment. Although the etiopathology of lipodystrophy is, at present, still elusive, we hypothesize that the primitive dysfunction of lipidic metabolism plays a critical role in both determining central nervous system (CNS) alterations and the findings that characterized this extremely rare disease.
A preliminary trial with fluoxetine, a 5-HT reuptake inhibitor, was carried out on two young male patients (21 and 32 years old) affected by Gilles de la Tourette syndrome. They both underwent a complete neurological evaluation also including neuroradiological, neurophysiological and neuropsychological assessment. Both patients had already been treated with benzodiazepines and amitriptyline; the older one was also given haloperidol and chlorimipramine with definite, but short-lasting improvement. During hospitalization a therapeutic trial with fluoxetine (20 mg/day in the younger patient and 40 mg/day in the older) in association with chlorimipramine (75 mg/day) was initiated, leading to a significant reduction (at least 50%) of abnormal movements and obsessive-compulsive behaviour. The older patient had no side effects while the 21 year old subject complained of insomnia, urinary retention and anorexia; despite the objective improvement, these side effects led us to modify the therapy after the first month. The favourable action of serotoninergic agents on TS symptoms supports the hypothesis that the multiple tics of the syndrome are motor compulsions.
From 1974 to 1988, 15 adult patients (aged over 16 years) with cerebellar medulloblastomas were observed in our Neurosurgical Department. All were treated by total (8 patients) or subtotal (7 patients) resection of the tumor, followed by radiation therapy to the posterior cranial fossa, spine and whole brain. A 5-year survival was achieved in 7 patients (46.6%). Local tumor recurrence occurred in 4 patients. The best treatment and the factors that influence the prognosis and survival of adult medulloblastomas are discussed also from the analysis of the pertinent literature.
In 22 migraine patients and 5 healthy controls an investigation has been performed on lymphocyte subsets in basal conditions and 90 minutes after sublingual isosorbide dinitrate (IDN) administration. In all patients the drug assumption induced a typical migraine attack with increase in all examined lymphocyte subsets with except of NK cells. A statistically significant increase (p < 0.05) in T lymphocyte subsets we found during the crisis, whereas in healthy controls neither a migraine attack nor any modification in lymphocyte subsets could be observed. These results seem to confirm the immune alteration we have previously found in migraineous patients and suggest the possible role of IDN-stimulated endothelium not only in vasodilatation but also in production of substances responsible of the observed immune modifications.
The hypothesis that alpha-adrenergic receptor blockade accounts for the ability of ergotamine to stop migraine attacks was tested, in migraine patients, in an experimental migraine model based on nitroderivative- induced attacks. In a preliminary single blind, placebo controlled study, thymoxamine, a prevalently post-synaptic alpha adrenergic receptor antagonist, was able to abort migraine attack in 9 out of 10 patients, as opposed to 2 out of 10 by placebo (p < 0.005 Fisher's exact test). In a subsequent randomized, crossover, placebo controlled double blind study, the ability of a selective alpha-1 adrenergic receptor agonist, methoxamine, to block ergotamine antimigraine effect was studied. In 26 patients migraine was induced in two separate tests and then ergotamine was administered once after methoxamine pretreatment and once after placebo; methoxamine was significantly more effective than placebo in blocking antimigraine effect of ergotamine (p = 0.0055 Fisher's exact test). These results support the hypothesis that ergotamine alpha-1 adrenolytic properties may account for its antimigraine effect suggesting that this action takes place outside the blood-brain barrier, since methoxamine can cross it very poorly. Ergotamine target structure could be the trigeminal innervation of the extracranial and/or dural vessels.
As far as we know, only 20 patients with association of aneurysm and arteriovenous malformation (AVM) on the posterior inferior cerebellar artery (PICA) have been reported so far. Further three personal cases are presented here and the pertinent literature is reviewed, the coexistence of aneurysm(s) and AVM on the PICA district increases the risk of intracranial bleeding compared with patients harbouring this association in the cerebral hemispheres. The direct approach of both lesions (excision of the AVM and clipping of the aneurysm) in one-stage operation is the treatment of choice.
Hand deformities cause severe discomfort and functional limitations to patients with Parkinson disease (PD) and related disorders. In clinical practice the problem is often overlooked or misdiagnosed. This paper reports on four cases whose characteristics are discussed.
