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The paper describes the clinico-instrumental diagnostic iter in a 57-year-old patient with amyotrophy of the pelvic and pectoral girdles and of the roots of the limbs, with lively and symmetric R.O.T., and without fasciculation, feeling deficit, bulbar involvement and pathological reflexes; EMG was probative for the neurogenic involvement of all four limbs, whereas muscular enzymes were normal. Laboratory controls showed primary hyperparathyroidism caused by a single operated parathyroid adenoma; other test enabled multiple endocrine adenomatosis to be excluded. The paper discusses the involvement of the nervous system during hyperparathyroidism paying special attention to spinal amyotrophy.

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The paper describes the case of a 37-year woman, with no past history of measles infection, who initially showed behavioral disorders, interpretative and reference deliria with a persecutory content. Extrapyramidal rigidity, bradykinesia, myoclonia, dementia, hyperpyrexia and coma with decerebrate rigidity subsequently appeared. Instrumental tests showed a spinal fluid status with intrathecal IgG synthesis and oligoclonal pattern, aspecific signs of widespread electric anomalies in the EEG. Encephalic CT and NRM were altered showing multifocal damage to the white substance. Anti-measles antibody titres were high in both spinal fluid and serum. Post-mortem histological tests confirmed the clinical hypothesis of subacute sclerosing panencephalitis. The paper reviews existing reports and pays special attention to cases of SSPE in adults and those cases with a psychiatric outcome. Lastly, the role of neuroradiological methods in the diagnostic iter is assessed.

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The Miller Fisher syndrome (MFS) is generally considered to be a disease of the peripheral nervous system. In some cases contemporary involvement of the central nervous system has been described (CNS). We report a case in which it was possible to prove involvement of cranial nerves VII, VIII, IX and X and to exclude CNS involvement. We discuss the possible role of early plasmapheresis treatment on disease evolution.

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Vogt-Koyanagi-Harada syndrome is a rare disease, which probably has a cell-mediated autoimmune pathogenesis, marked by ocular (anterior and/or posterior uveitis), dermatological (poliosis, canities, vitiligo) and neurological (meningo-encephalitis) disorders of variable severity in variable combinations. The clinical pattern in the case reported here showed severe neurological involvement (headache, ataxia and confusional state) followed by anteroposterior uveitis. Instrumental investigations (cerebrospinal fluid, VEPs, BAEPs, EEG, CT and MRI brainscans) confirmed the diagnosis. The response to cortisone therapy was excellent. We emphasize the importance of the neuroradiological investigations, because of their peculiarities, and review the reports of cases with marked meningo-encephalitic impairment, given the dearth of reports in the neurological journals.

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A 19-year-old patient with acute idiopathic polyneuritis is described. Clinically, apart from ophthalmoplegia, areflexia and ataxia, the patient presented a serious bilateral and symmetrical deficiency of the VII-IX-X cranial nerve and hypanapallesthesia of trunk and extremities. Liquor examination showed albumino-cytological dissociation with an increase in liquor IgG; encephalic CT and encephalo-medullary NMR were normal; a neurophysiological study (EMG, PEV, BAER) was indicative of the PNS problems. Combined corticosteroid and plasmaferesis treatment produced complete clinical remission after about 3 months. The primary role of PNS involvement in this clinical entity is discussed.

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Two cases of spino-cerebellar heredoataxia are reported. The first patient, aged 18, presented the clinical peculiarities of Friedreich's disease; subjected to encephalic CT and encephalomedullary NMR the proved normal; EMG study and visual, acoustic and somatosensorial evoked potentials were not normal but there was nothing specifically wrong. The second patient, aged 30, followed up for more than 10 years, presented the clinical aspects of Pierre Marie disease; stress is laid on encephalic CT examinations carried out at the age of 20 and 30. These were pathological due to the marked dilatation of the IVth ventricle and the basal cisternae; evoked potential changes were aspecific. The nosography is discussed, especially as regards clinical diagnosis, in the absence of typical neuroradiological or other instrumental aspects and, obviously, in the absence of anatomopathological signs.

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A clinical of 37 patients suffering form Guillain-Baré syndrome has been carried out. Because of their unusual onset and clinical course, two of the cases are reported in full. The first case presented atypical onset modalities characterised by dysarthria, dysphonia and dysphagia with subsequent diplegia of the facial nerve and motor involvement of all four extremities; the second was difficult to diagnose and over time it turned out to be disimmune chronic-recurrent polyneuropathy. The clinic instrumental diagnostic and therapeutic course of this case, which was characterised by the poor response to corticosteroid therapy as a result of which use of immunosuppressor (azatioprine) and plasma-feresis were employed with a reaosable improvement, is described in detail. The differential diagnosis and nosologica classification are discussed.

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The clinical case and instrumental diagnosis of a patient suffering from sporadic late cerebellar atrophy (Pierre Marie, Foix and Alajouanine's syndrome) is reported. Interesting was the long period of observation and the neurodiagnostic study rounded out by encephalo-medullary N.M.R. The differential diagnosis is discussed.

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The clinical and diagnostic procedures applied to a 53 year old woman admitted to hospital with left brachiocrural sensory and motor impairment subsequently extending to the right of the body are described. The complete clinical picture featured severe tetraparesis with pyramidal hypertonia and proprioceptive hyperreflexia as well as bilateral Babinski, marked atrophy of the scapulohumeral girdle, thermodolorific anesthesia and severe tactile hypoesthesia from C2 to C4. Magnetic resonance studies of the cervical marrow and spine revealed a meningioma on C1-C2 that was about 1.5 cm in diameter and situated anterior to the marrow with partial extension towards the left. Radical excision of the tumour totally cured the symptoms.

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After a definition of the condition and a discussion of its physiopathological significance, the need to examine the patient as a whole and provide total therapy is emphasised with reference to both the literature and personal cases. It is concluded: 1) that the mesodiencephalic structures, especially the hypothalamus, are of primary importance in human physiological and pathological processes. The hypothalamus is the real brain of the viscera, the functional bridge between the hypophysis, the limbic structures and the upper cortical centres; 2) that doctors should consider the role of the hypothalamus in the diagnosis and therapy of the total person: a) in evaluating the patient's real emotional and psychological situation; b) in realising that the simple administration of a drug to cure the organic symptom really means only partial treatment of the patient himself.

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Two children affected by cerebral hydatidosis are described. For preoperative diagnostic purposes computerised axial tomography appears currently to be the most suitable procedure. Complete surgical removal of the cyst, with methods preserving its integrity, seems to be the main factor for definitive recovery as the different outcomes in the two cases show.

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5 hemangiopericytomas have been studied together with 678 tumors of the CNS including 579 meningiomas, 13 fibrosarcomas, 4 malignant meningiomas, 3 unclassified malignant tumors and 79 angioblastic meningiomas. The main clinical and histological features of this series are discussed in order to identify the more useful criteria for biological and microscopical characterization of the hemangiopericytoma. Beside a pure type of growth, the presence of histological intermediate variants and mixed cell proliferations have been observed. The possibility of transitional features and mixed tumors is then outlined.

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We have reported a case of a racemose cyst of the fourth ventricle. The clinical and histopathological particularities are described. Among the clinical details, special emphasis is given on the intermittent attacks of obstructive hydrocephalus. Among the microscopical data, the demonstration of both scolex containing crystalline formations and parasite body in a racemose cyst is stressed as the most important finding for the diagnosis of this tapeworm species.

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A group of 5 selected malignant meningiomas was studied in relation to the incidence and morphology of the mitoses. Beside a high mitotic rate many structural chromosomal abnormalities were observed. In agreement with other malignant onco-types previously studied, the authors suggest that atypical mitoses together with a tissue and cytological undifferentiation may be assumed to be an important prognostic criterium for meningiomas. The problem of malignancy in meningiomas has not been, so far, satisfactorily resolved, so that, in our opinion, the definition of even minimal details may be of some interest. For example, as it clearly appears from reviewing the literature on this sugject, no particular attention has been paid to the mitotic features. Referring to previous studies on the mitotic abnormalities in different malignant cerebral onco-types, the authors aimed to examine the various mitotic aspects in a limited group of tumors selected on the basis of clinical and histological malignancy.

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A clinical and pathological description of five cases of granulomatous mycoses of the C.N.S. is reported. Four had an intracranial and one a spinal localization. In all patients the clinical pattern was of tumoral type, while the disease evolution was favourable only in one case. From a histipatholic point of view the preliminary diagnosis was of granulomatous process. The fungi organisms were identified only by means of combined stain methods. Beside the pathogenetic problems the Authors outline some histological methods useful for diagnostic purposes when a culture procedure for fungi is lacking.

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A rare case of primary cerebral actinomycosis is presented. Pathologically the most important finding is represented by two types of lesions occurring in the same granulomatous mass without abscess formation. The possibility of an associated infection by actinomyces israelii and actinobacillus actinomycetemcomitans or lignieresi is discussed.

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