The sunflower syndrome.

Some photosensitive epileptic patients show absence attacks preceded by a "seeking of a source of light" and stereotyped movements of various kinds. The common explanation is an attempt to self-induce seizures. The case reported is of a young girl who had not shown evidence of photosensitivity for many years. Alternative interpretations of this phenomenon are discussed.

Successful treatment of subacute cerebellar degeneration in ovarian carcinoma with plasmapheresis. A case report.

Subacute cerebellar degeneration is a rare complication of some neoplasms, and is generally resistant to therapy. A case of subacute cerebellar degeneration in a 50-year-old woman with a Stage II grade 3 serous ovarian adenocarcinoma is reported. The onset of the neurologic symptoms preceded the diagnosis of cancer and progressively worsened during and after four cycles of chemotherapy. A quick, partial improvement of the neurologic syndrome was documented after three weekly treatments with plasmapheresis. The contribution of circulating factors in inducing subacute cerebellar degeneration can be postulated. A trial using this new type of treatment should be performed in patients who have this therapeutically refractory clinical condition.

Hypercalcemic encephalopathy in the course of hyperthyroidism.

A hypercalcemic condition can be observed in association with hyperthyroidism. The case of a patient suffering from hypercalcemia and hyperthyroidism is reported. A confusional state and EEG alterations, among which diffuse monomorphic delta rhythms were remarkable, are shown. As soon as normalization of calcemia was achieved, a rapid clinical and EEG improvement took place. A hypothetical interpretation is proposed, according to which a prolonged, though inconstant, and mild hypercalcemia in the course of hyperthyroidism could determine an encephalopathy, concealing in some way thyrotoxic symptoms.

Isolated benign cerebral vasculitis. Case report and review.

A case of isolated cerebral vasculitis showing benign outcome is presented. Review of pertinent literature suggests that isolated benign arteritis of the CNS should be considered as a distinct entity, in a heterogeneous group of vasculitides. The common features of the syndrome are as follows: 1(0) benign evolution; 2(0) sensitiveness to cortisone; 3(0) absence of CSF alterations; 4(0) constant angiographic pattern of arteritis. Early cortisone treatment is suggested in any case of cerebral arteritis.

Amyotrophic lateral sclerosis in the province of Parma, Italy: a clinical and epidemiological study in the period 1960-1980.

A clinical and epidemiological study on ALS was conducted in the province of Parma, Italy, in the period 1960-1980. A total of 78 cases was collected from hospital records. The average annual incidence was 0.93 per 100,000 with no difference between rural and urban areas. On prevalence day (October 24, 1971) a prevalence rate of 2.27 was found. Mean age at onset was 59 years, with no sex difference. In 70% of cases the onset was between 50 and 70 years, this datum being statistically significant (p less than 0.001). Mean duration of the disease was 30.4 +/- 20 months, with no difference for sex and age at onset. A comparison was then made between two decades (1961-70 vs 1971-80), in order to verify the clinical impression of a worsening of some ALS parameters in recent years. Although statistical evaluation did not show any significant data, a tendency to a worsening of some parameters of the disease may be postulated.

Pseudomyotonia of the striated urethral sphincter.

Pseudomyotonic discharges of the striated urethral sphincter are infrequent findings. We herein report on 9 patients with dissimilar anatomical-clinical conditions. In some patients a definite neurological lesion was ascertained, whereas in the remaining patients no neurological impairment could be determined clinically and instrumentally. The possibility of spontaneous resolution of the phenomenon is an interesting clinical feature.

Encephalopathy in the course of Wegener vasculitis.

A 44-year-old woman presenting first ear disease and then cranial nerve, lung and kidney lesions and ultimately central nervous system involvement was found on kidney biopsy to have Wegener granulomatosis (WG). It is unlikely that the vasculitic component of the illness was directly responsible for most of the neurological symptoms.

Polyneuropathy in progressive external ophthalmoplegia.

A case of progressive external ophthalmoplegia (PEO) in association with peripheral neuropathy and other neural and visceral manifestations is reported. Pathological studies of the sural nerve demonstrated loss of large myelinated fibers, segmental demyelination with remyelination, and excess of zebra bodies in the Schwann cells on electron microscopy. Peripheral neuropathy in the course of PEO is regarded as a further although rare aspect of a multisystem disease.

Meige syndrome: a clinical and EMG study.

2 patients showing oral-facial asymptomatic dyskinesia are reported. Clinically observed involuntary movements are the same as those described by Henry Meige in 1910 for the first time. An outstanding EMG feature is the recording of spontaneous potential bursts, both short and long in duration, that show synchronism when activity is registered from different facial muscles at the same time. Clinical and EMG data allow close comparison between involuntary movements in the course of Meige syndrome and idiopathic hemifacial spasm.

Facial myokymia in the course of a pontine tumor.

A patient with facial myokymia, suffering from an infiltrating grade II astrocytoma originating within the pons with vegetations invading the cerebellopontine angle, was studied clinically and electromyographically from 1973 to 1979. The myokymias started in the muscles of the right side of the face and later spread to the left side. The latter point is worth noting because the myokymias of the left side were detectable by EMG but not by inspection. They preceded clinical and instrumental evidence of a contralateral spread of tumoral damage to the brainstem. From the physiopathogenetic angle they point up the importance of a mechanism of hyperexcitability and release of the facial motoneuronal pool. The results of the EMG study suggest that the persistence of myokymia, its association with an ingravescent neurogenic impairment of the facial musculature and its polymorphism in the course of pontine tumors are more reliable features than their discontinuity and rhythmicity.

[Late myopathy with mitochondrial changes in the muscle]. / Miopatia tardiva con alterazioni mitocondriali del muscolo.

The ultrastructural finding of abnormal muscle mitochondria has been reported in various conditions, but mostly in association with the clinical picture of ophthalmoplegia, and in cases of "floppy infant" syndrome. In the case herein reported, the mitochondrial abnormalities were found in the muscle biopsy of a 49-year-old man suffering from a late onset proximal myopathy; he was affected also by polyneuropathy, subclinical thyroid dysfunction, disturbances of heart conduction, and unilateral gynaecomastia. The association of abnormal muscle mitochondria and late onset myopathy without involvement of the extraocular muscles has been reported in a very few cases. It is not possible, at present, to state that these cases represent a nosological entity; the existence of an underlying biochemical defect, accounting for the mitochondrial abnormalities, could be suspected, but it seems more probable that these changes are non-specific features of muscular damage, possibly related to the stage and the degree of the process. In this view, the coexistence of neurogenic damage, gastrointestinal malabsorption, and thyroid dysfunction, could play an additional role in the case herein described. Finally, the coexisting findings of cardiac, endocrine, and neuropathic damage are discussed with regard to the Kearns-Sayre syndrome, which also associates mitochondrial myopathy and multisystemic involvement.

[Subclavian arteriography in Pancoast syndrome. Case report (author's transl)]. / L'arteriografia succlavia nella sindrome di Pancoast. Studio clinico di un caso.

A case of Pancoast's syndrome is reported, where subclavian arteriography was the only useful diagnostic procedure to make a diagnosis possible. Utilization and meaning of such a diagnostic technique is discussed, since the typical radiological picture of the syndrome may be lacking, especially in the early stage of the disease. Subclavian arteriography explores th costal-pleuro-vertebral space selectively, being aimed to one of the anatomical structure, the mechanical engagement of which is responsible of the syndrome. Such a technique is advisable even in cases where the Pancoast's syndrome is sustained by an infrequent aetiology, such as metastases.

Studies on the etiology of the experimental neuropathy from industrial adhesive (glues).

Chickens treated by paintbrushing with glue distillate (used in shoe industries), hydrocarbon mixture and TOCP in hexane, developed paralysis (4/5); in these, we have demonstrated a diffuse degeneration of the myelinic sheath of the peripheral and central neurites. Besides TOCP, cyclohexane (because of its higher concentration in the glue distillate and in the hydrocarbon mixture) may be indicated as responsible for occupational neuropathy.

[Petit mal status]. / Petit mal status.

The historical evolution of P.M.S. nosography is pointed out, and his clinical features are exposed, mainly on the ground of the study performed by Lob and Coll., for the Tenth Marseilled Colloquium. Stress is laid upon the onset age of P.M.S., making reference to a review of 133 cases previously reported in the literature. Two paradigmatic cases are reported, selected between six P.M.S. observations we collected, and electroclinically investigated, from 1972 to 1975: a woman aged 34 who was suffering from P.M. and G.M. seizures since she was 12-years old, and also had some other P.M.S. episodes during past years; a woman aged 45 who suffered from a sudden P.M.S. attack, during a febrile illness: it seemed that it was the first occurrence, but an accurate catamnestic search suggested that previous P.M.S. manifestations had probably occurred during the infancy. Finally P.M.S. nosography is discussed for what concerns the variable features emphasized by our observations, the correlation between P.M. and P.M.S., and the problem of P.M.S. as the sole epileptic phenomenon.