Amyotrophic lateral sclerosis in Sardinia (Italy): epidemiologic features from 1957 to 2000.

OBJECTIVES: To evaluate epidemiological variables of amyotrophic lateral sclerosis (ALS) in Sardinia (Italy) in the 1991-2000 periods and compare them with the preceding decades. MATERIAL AND METHODS: Survey, critical reappraisal or clinical re-evaluation of all ALS cases with onset in the decade 1991-2000; calculation of crude and age-adjusted incidence, duration of disease, survival rates and the latency between onset of symptoms and diagnosis. RESULTS: A significant increase in the mean annual incidence was observed in comparison with the values found in the two previous decades, 1971-1980 and 1981-1990. The distribution of the disease in various areas of the island was found to be not at all homogeneous. No significant modifications of the duration of the disease and survival rates were observed. CONCLUSION: The role of particular exogenous factors, albeit still unclear, can be invoked.

Is antegrade nailing a proper option in 2- and 3-part proximal humeral fractures?

PURPOSE: To report our experience with antegrade short locked intramedullary nail for treatment of proximal humeral fractures and to review the current literature. MATERIALS AND METHODS: From January 2012 to July 2013, 41 patients affected by two and three-part proximal humeral fractures were treated with surgical internal fixation with short locked intramedullary nails. Outcome analysis included standard clinical follow-up, Constant shoulder score and plain radiographs. The mean follow-up was 30 months (range 24-42). Moreover, a review of the literature was carried out. RESULTS: The mean Constant shoulder score was 81.5, excellent functional outcomes in 24/38 patients. All the fractures healed in an average time of 3.7 months. Five patients underwent additional operations, complications included hardware penetration into the joint (n = 2), backed out screw (n = 1), shoulder impingement due to protrusion of the nail (n = 2) and superficial infection (n = 1). The literature review showed 530 patients affected by proximal humeral fracture and treated with intramedullary nail with mean age of 65 years, mean follow-up of 22.2 months and a Constant shoulder score of 72.9 points; the major complications reported were backing out of the screws, shoulder impingement and joint protrusion of the screws. CONCLUSIONS: Antegrade short locked intramedullary nail allows stable fixation, minimal soft tissue dissection, early mobilization of the shoulder and good outcomes. It is an efficacious therapeutic solution for 2- and 3-part proximal humeral fractures.

Effects of tiapride on homovanillic acid levels in human cerebrospinal fluid drawn at pneumoencephalography.

The effect of tiapride on HVA and 5-HIAA levels in the CSF drawn at pneumoencephalography (PEG) was studied. Five consecutive 5 ml fractions of CSF were drawn from control and tiapride-treated subjects. In both groups, a linear increase in HVA concentrations was found between the first and subsequent fractions. On the contrary, no significant difference in 5-HIAA concentrations was found in sequential CSF samples. Tiapride increased the mean HVA concentrations and caused a steeper caudocranial gradient of this metabolite but failed to modify 5-HIAA concentrations. The results suggest that tiapride blocks dopamine (DA) receptors and increases DA synthesis.

Sensitivity and specificity of the EEG versus CT in acute cortical and subcortical stroke.

122 patients with acute stroke were examined in order to demonstrate the sensitivity and the specificity of EEG versus CT findings, in distinguishing cortical from subcortical lesions. To do this the electroencephalographic diagnosis performed by two different electroencephalographers was compared with the neuroimaging diagnosis. The results showed a sensitivity of 77% and a specificity of 75%. Furthermore, the EEG findings can also provide physiopathological data, in that the cortical lesions are likely to be due to embolism, while the subcortical lesions are more likely to be due to the pathological process of the intracerebral blood-vessels and the lesions of the watershed territories to hemodynamic phenomena.

Spontaneous mesencephalic hemorrhage: case report and physiopathological interpretation of its course.

We describe a case of spontaneous mesencephalic hemorrhage which evolved in 4 stages with complete clinical resolution. In the light of our findings and of published data we interpret the phenomenon in terms of the regional anatomy and vascularization.

Subarachnoid hemorrhage of unknown origin. A 44 cases study.

44 cases (20 men and 24 women) of subarachnoid hemorrhage (SAH) of unknown origin were studied: the mean age was 54.1 yrs. Highest frequency of the disease was observed in 51-70 years old patients. There was only a slight involvement of consciousness (I-II grade of HESS and HUNT). Vascular hypertension was noticed in 45.5% of patients, but outcome did not differ in hypertensive and normotensive subjects. The CT findings showed a typical picture of SAH. 4 cases of perimesencephalic and peripontine hemorrhage were noticed. 24 patients underwent lumbar puncture. The angiographic study was normal in all patients. 14 subjects repeated four-vessel angiography. Transient hydrocephalus was found in 22.7% of patients. Approximately 70% of patients received antifibrinolytic therapy, either with or without calcium entry-blockers (nimodipine). Neither ischemic complications nor rebleeding were observed. Follow-up showed that all patients had survived 7-88 months after SAH. All subjects made a complete recovery and have since been able to resume work.

Epidemiologic and clinical features of optic neuritis in the 20th and 21st sanitary districts of the Sardinia (Italy).

An epidemiologic and clinical study on optic neuritis in two sanitary districts of the Sardinia was carried out. The results were partially in agreement, partially in contrast with the literature data. The most important datum is that the annual incidence rate of optic neuritis in Sardinia is similar to the rates observed in medium-high risk for MS geographical areas, according to observation which showed that frequency of MS in Italy, and in Sardinia particularly, is much higher than that indicated by previous studies.

Epidemiologic and clinical features of the Guillain-Barre' syndrome in Sardinia in the 1961-1980 period.

Epidemiologic and clinical features of the Guillain-Barrè syndrome in Sardinia, in a twenty years period, were studied. 120 subjects (62 males and 58 females) with Guillain-Barrè syndrome were identified. An annual incidence rate of 0.4/100,000 population was found. The results were compared both with national and international data of the literature. The peculiarity of some aspects was pointed out.

Epidemiological and clinical features of primary intrarachidian tumours in the island of Sardinia, during the 1977-1986 period.

The cases of primary intrarachidian tumours observed in the 1977-1986 period in the neurological, neurosurgical, oncological and radiological Departments in Sardinia, were collected. Only histologically diagnosed tumours of spinal cord, root-nerve and their envelops in patients resident in the island for at least one year were included in the investigation. The annual crude incidence rate was 0.5 per 100,000 population (0.63 in the females and 0.37 in the males). M/F ratio was 1.7. Mean age was 45 years (35.5 in males; 50.7 in females). Age and sex distribution showed higher frequence of intrarachidian tumours in females over 40 years of age and in males younger than 40 years. Meningiomas were more frequent in females aged between 47 and 74 years. The dorsal localization of the tumours was more frequent. Subjective sensory complaints (85.3%) and motor impairment (76%) were the main symptoms at onset; at the time of diagnosis motor impairment (92%) and objective sensory disturbances (82.7%) prevailed. A twelve months interval elapsed between onset of first symptoms and diagnosis in 64% of patients; within 24 months 81.3% of tumours were diagnosed. Surgery induced improvement in 74.6% of patients, no improvement in 17.3%; a worsening was noticed in 8%. Seven patients died after surgery (3 owing to the tumour). These data are partly consonant and partly in contrast with those present in literature.

Rigidity and painful muscle spasms in a patient with probable myelitis.

We describe a case of continuous motor unit potential (MUP) activity of central origin (unlike stiff man syndrome and progressive encephalomyelitis) characterized clinically by rigidity, painful muscle spasms, abnormal postures and spinal myoclonus. The topography of the manifestations, the subacute and benign course, the presence of stable sequels 2 years after onset and a searching process of differential diagnosis lead us to attribute the condition to an inflammation of the cord, which makes the case of particular clinical interest.

Primary optic neuritis evolved in multiple sclerosis: an epidemiological study.

Sixty-nine cases of Optic Neuritis were studied in order to evaluate the percentage of evolution into multiple sclerosis. We observed an incidence rate of 53.6% which is somewhat high respect to data present in literature. The various findings obtained in the present study were compared with those of the literature and the similarities and discrepancies underlined.

Myelographic and MRI appearances of a thoracic spinal extradural arachnoid cyst of the spine with extra- and intraspinal extension.

An unusual arachnoid cyst of the thoracic spine is reported. The clinical and neuroradiological features are described.

Thalamic hemorrhage. 30 cases studied: clinico-tomodensitometric correlations.

The clinico-tomographic correlations in 30 patients hospitalized for primary thalamic hemorrhage were studied. Arterial hypertension, observed in 90% of patients, represented the most important risk factor. Twenty-six subjects showed a sensory-motor hemisyndrome contralaterally to the lesion, nineteen showed alteration in level of consciousness from confusion to stupor and coma. Twelve subjects had poorly reactive pupils and eleven speech disturbances with involvement of the left thalamus. Seven patients died following hemorrhage; all subjects presented ventricular bleeding, severe disturbance of consciousness and arterial hypertension. On admission to hospital impairment of consciousness was the most significant unfavourable prognostic factor.

Low doses of TRH in amyotrophic lateral sclerosis and in other neurological diseases.

30 subjects--23 with amyotrophic lateral sclerosis (ALS), 4 with Charcot-Marie Tooth atrophy, 2 with progressive spinal muscle atrophy and 1 with radiation myelopathy--were given chronic low-dose TRH therapy. The effects of treatment were assessed on the scale of Norris et al. (1974). The outcome of the study, in agreement with some and at variance with other studies, was that TRH induced a statistically significant neurological improvement in 17 of the 23 ALS patients but little or none in the other ALS patients and in patients with other neurological diseases.

Effect of ethanol on the metabolism of regional brain dopamine.

Acute oral administration of ethanol (3.2g/kg) to rats increased (DOPAC) levels in the caudate nucleus, but had no effect on DOPAC levels in the substantia nigra and frontal cortex and failed to modify dopamine content in any of the above areas. On the other hand, the administration of the same dose of ethanol to rats which had been chronically treated with ethanol (3.2g/kg daily for 60 days), produced a decrease of DA content and a parallel increase of DOPAC levels in all areas studied. In chronically treated rats, 24 hrs after last ethanol administration dopamine levels in the frontal cortex were 60% higher than in controls. The results suggest that ethanol administration causes dopamine release in different brain areas.

Low androgenization index in elderly women and elderly men with Alzheimer's disease.

The authors investigated whether testosterone levels and testosterone availability differ between older lean subjects with and without Alzheimer's disease (AD). Sex hormone binding globulin (SHBG) and estradiol levels were higher, whereas the free androgenization index (FAI) was lower, in lean subjects with AD than in lean subjects without AD. Factors involved in the increase of SHBG secretion could have an important role in the lower testosterone availability of subjects with AD.

Bipolar affective disorder and Parkinson's disease: a rare, insidious and often unrecognized association.

Five patients (4 women) with Parkinson's disease (PD) and primary major psychiatric disorder (PMPD) meeting DSM-IV criteria for the diagnosis of bipolar affective disorder (BAD) were studied. Four patients had early onset PD. Four developed a severe psychiatric disorder a few years after starting dopaminergic therapy in presence of a mild motor disability and a mild cognitive impairment, with no evidence of cerebral atrophy at CT or MRI. Two patients developed a clear manic episode; the other three presented a severe depressive episode (in one case featuring a Cotard syndrome). None showed previous signs of long term L-dopa treatment syndrome (LTS), hallucinosis or other minor psychiatric disorders. The two manic episodes occurred shortly after an increase of dopaminergic therapy and in one case rapid cyclic mood fluctuations were observed. At the onset of psychiatric symptoms, all patients had an unspecific diagnosis of chronic delusional hallucinatory psychosis (CDHP).