L. Bettoni

STEADINESS OF AMYOTROPHIC LATERAL SCLEROSIS IN THE PROVINCE OF PARMA, ITALY, 1960-1990

A clinical and epidemiological study on amyotrophic lateral sclerosis (ALS) was conducted in the province of Parma, Italy, from 1960–1990. A total of 121 cases were collected from hospital records. The average annual incidence was 0.98 per 100000 inhabitants, with a male/female ratio of 1.1. Age‐specific incidence was maximal in the age group 60–69 years. No difference between rural and urban areas was found. Prevalence on October 26th, 1981 was 2.5 per 100000. Mean age at onset was 60 years, with no significant sex difference. Mean duration of the disease was 30 (sd 21.4) months. Bulbar forms were significantly (p<0.05) shorter than conventional forms, with a mean duration of 23.4 (sd 21.4) months. Age at onset did not influence prognosis. A comparison of three decades was made, to verify whether possible variations of the disease had occurred with time. From our data a definite stability was found in such epidemiological parameters as incidence, prevalence, mean duration and mortality of ALS in the period.

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.

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.

Unilateral trismus caused by vertebrobasilar dolichoectasia.

Vertebrobasilar dolichoectasia is a relatively uncommon pathological entity and generally asymptomatic. We report a quite unusual case of unilateral motor trigeminal involvement with trismus, due to VD. Apart from tetanus or local morbid conditions of the mouth, trismus is often attributed to disturbed programming and co-ordination of the masticatory muscles within the mesencephalic nucleus. The possibility of truncal compression of the trigeminal motor root by vertebrobasilar dolichoectasia being responsible for masseter muscle spasm, in analogy with the pathogenetic mechanisms proposed in hemifacial spasm, is proposed as an alternative explanation in the present case.

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.

MR findings in Meige syndrome.

A 65-year-old woman was diagnosed clinically to have Meige syndrome in the oromandibular dystonic clinical variant of Marsden. On magnetic resonance imaging a reduced signal intensity in the head of the caudate nuclei, more prominent on the right, and in both putamina was shown.

Myokymia in the Course of Peripheral Idiopathic Facial Palsy

5 cases of myokymia from a total of 31 patients showing peripheral ‘idiopathic’ facial palsy are reported. Myokymia began during the acute phase of palsy and abruptly disappeared during reinnervation. These findings lead us to consider damage of the facial nerve axon along its bony channel as a possible source for myokymia.