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The authors report two computed tomographic observations of Fahr's disease without disturbances in calcium-phosphorus metabolism; the first case is a by chance discovery and the second one is characterized by a cerebellar tremor. From the literature data they discuss successively clinical, radiological and computed tomographic, neuropathological, aetiopathogenetic and therapeutic aspects actually presented by strio-pallido-dentate calcifications (SPD) or Fahr's disease.

Three children born of consanguineous parents had Lafora's disease and two of them had been studied clinically, pathologically and biochemically. The autosomal recessive transmission of the disease is confirmed by the familial study and the parents present no phenotypic anomaly of the disease. Electroclinical data are discussed with regard to the early differential diagnosis between this entity and idiopathic generalised epilepsy and myoclonic epilepsy without Lafora's bodies. Iodine spectrum of the cerebral and hepatic abnormal deposits suggest that Lafora's bodies mainly consist of long chains glycogen similar to amylopectin.

The clinical diagnosis of acute vascular disorders of the cerebellum (AVDC) is uneasy. Classical views emphasized poor prognosis and need for cranial decompression. These views were probably due to the fact that, in the past, a correct diagnosis was made in the cases with the most dramatic symptoms only. The authors report their findings in 13 cases of AVCD. The diagnosis was confirmed or established by CT scan, which showed cerebellar hemorrhage in 6 patients and cerebellar infarctus in 7 patients. CT scan lead to the recognition of AVCD cases with a more benign course than traditionally thought. In 12 out of 13 patients, there was no need for neurosurgical treatment. Finally, CT scans allowed the diagnosis of hemorrhage versus ischaemia and repeat scans demonstrated the evolution of the lesion.

Status epilepticus was a complicating feature of the last bout before the fatal outcome in a patient with a 11 years history of multiple sclerosis. The fits were seen as clonic movements of the eyes in a lateral direction, lasting for 30 to 90 seconds and repeated every 5 minutes on average. Electroencephalographic recordings demonstrated periodic slow spikes on the right hemisphere, starting in the anterior region. Neuropathological data eliminated an associated pathological condition and suggested that the development of acute cortico-subcortical demyelinating lesions was responsible of the epilepsy. The type of electrical activity observed, localized to one hemisphere, is found with recent ischemic lesions, and less frequently in cases of cerebral tumor, necrotizing encephalitis, metabolic encephalopathies or post traumatic disorders. A case with similar clinical and electroencephalographic features in a patient with multiple sclerosis has been reported in the literature.

The authors report clinical and scanographic findings in 26 cases of capsulo-thalamic hemorrhage. They stress the frequency of arterial hypertension as a pre-disposing factor. The clinical picture is represented by the association of disorders of consciousness (usually obnubilation), contralateral flaccid hemiplegia with hemianesthesia, oculomotor disorders (disturbance of vertical gaze, miosis) and disorders of linguistic and motor behaviour. The paraclinical examination of choice is scanography which enables the site, volume and extent of the hemorrhage to be estimated, together with the mass effect and intraventricular rupture. Clinical and scanographic follow-up indicates a favourable prognosis for capsulo-thalamic hemorrhage (77% survival). Follow-up findings point to several unfavourable prognostic factors: immediate coma, persistent and massive flaccid hemiplegia, hemorrhage size exceeding 3 cm, and severe intraventricular rupture. In the light of the data in the literature, the authors discuss the clinical and scanographic signs of capsulo-thalamic hemorrhage.

The authors report some observations about 228 cases of multiple sclerosis with secure diagnosis subdivided in severe (34,6 %), common (29,8 %) and benign after the tenth year of illness (35,6 %). These observations are in agreement with classical statistic reports concerning the natural disease history : higher female incidence (64 %), average age at onset about 29,5 years, 4 main first signs (ocular [42,5 %], motor [41,5 %], ataxia [36,3 %], sensory [35 %] and remitting course [82,5 %]). Follow up study of 81 "benign" forms after the tenth year of the disease allows us to look at the freqwuency of "secondarily worsening" forms (25,7 %) and "benign remaining" ones until the twentieth year (10,1 %).

The authors report 31 cases of "vascular epilepsy" among 280 cerebral strokes confirmed by cranial computerized tomography. A high incidence of ischemia (28 cases : 90%) is noted. Epileptic seizures are initial (14 cases) or sequellar (17 cases) manifestations of cerebral stroke. Partial seizures are the most frequent (58%), particularly "Jacksonian" motor fits, which, when initial, often lead to status epilepticus. Frequency and bad prognosis of initial status epilepticus are pointed out.

Onset of confusion with myoclonus occurred in a 55-year-old patient hospitalized for infectious endocarditis and under intravenous dopamine. The speculative responsibility of dopamine in the occurrence of this myoclonic encephalopathy is discussed. Indeed, although dopamine does not cross the blood-brain barrier, inflammation of the wall of the cerebral vessels (immunologic vasculitis) may have enabled dopamine to reach the central nervous system.

The authors report a case of tuberculous meningitis in which multiple bacteriological samples were negative during the eleven months of the clinical course. There were no other visceral localizations. The fatal outcome was due to vascular lesions (multiple cerebral infarcts), and to CSF flow disorders. The findings of cranial computerized axial tomography and of the pathological examination are reported.