Headache is a common Emergency Department (ED) problem accounting for 1.7%–4.5% of patients every year. The main aim in the ED is to differentiate between primary and secondary headache. Although secondary headache represents the lowest percentage (20%) of ED patients presenting with headache, it can be life threatening. Patients with primary headache require a follow-up programme on discharge from the ED with a specialist headache clinic taking responsibility for these patients. This procedure was applied at Ravenna Hospital ED for 2 years and recorded (unpublished data) a reduction in the number of repeat visits to the ED for headache and the number of inappropriate admissions for headache (DRG 25).

Headache is a common Emergency Department (ED) problem accounting for 1.7%-4.5% of patients every year. The main aim in the ED is to differentiate between pri- mary and secondary headache. Although secondary headache represents the lowest percentage (20%) of ED patients presenting with headache, it can be life threatening. Patients with primary headache require a follow-up pro- gramme on discharge from the ED with a specialist headache clinic taking responsibility for these patients. This procedure was applied at Ravenna Hospital ED for 2 years and record- ed (unpublished data) a reduction in the number of repeat visits to the ED for headache and the number of inappropri- ate admissions for headache (DRG 25).

OBJECTIVES To estimate the incidence rate of Guillain-Barré syndrome variants in an unselected population and to describe their clinical features and prognosis. METHODS A two year prospective multicentre study on the incidence and prognosis of Guillain-Barré syndrome was performed in Emilia-Romagna, northern Italy (3 909 512 inhabitants). A surveillance system was instituted within the study area, which comprised all the neurological departments, private and public general hospitals, and practising neurologists. The international classification of diseases (ICD) codes 357.XX (any peripheral neuropathy) of hospital discharges were also reviewed. RESULTS Data were separately analysed for Miller Fisher syndrome and other Guillain-Barré syndrome variants. During the study period 18 patients with Guillain-Barré syndrome variants including seven with Miller Fisher syndrome were recruited; the incidence rates were 0.14/100 000/year (95% confidence interval (95% CI) 0.07–0.25) for Guillain-Barré syndrome variants (excluding Miller Fisher syndrome) and 0.09/100 000/year (95% CI 0.04–0.18) for Miller Fisher syndrome. Guillain-Barré syndrome variants alone (excluding Miller Fisher syndrome) accounted for 10.5% of total cases. Death and relapses were not found. Details of clinical, electrophysiological, and CSF findings of Guillain-Barré syndrome variants are provided. CONCLUSIONS Guillain-Barré syndrome variants other than Miller Fisher syndrome, as obtained through a population based study, account for about 10% of total cases of Guillain-Barré syndrome and, as a whole, have a good prognosis. Their clinical features are heterogeneous; bifacial weakness (associated with other signs, mainly sensory disturbances) represents the most frequent finding.

We performed a multicenter prospective study on the incidence and prognosis of Guillain-Barre syndrome (GBS) in the Emilia-Romagna region (3,909,512 inhabitants), Italy, over a 2-year period (1992-1993). The case finding method was based on a surveillance system including neurologic departments, all private and public general hospitals, and all practicing neurologists. We also reviewed all the ICD codes of hospital discharges. Ninety-four patients with GBS were identified, giving a mean incidence rate of 1.20 per 100,000 per year. Men/women ratio was 1.94. Incidence rate increased with age for both sexes. There were no significant incidence variations among seasons or months. Antecedent infections were reported in 59% of the patients. There was an overall good prognosis. After 6 months, 64% of patients had recovered and only 4% was still severely disabled. Acute mortality was 3%. Older age was the strongest predictor of poor outcome. NEUROLOGY 1997;48: 214-221

We report the effects of treatment with plasma-exchange (PE) and intravenous immune globulins (IVIg) in 36 out of 50 patients with Guillain-Barré syndrome (GBS) recruited by an incidence study in the Emilia-Romagna region of Italy. Comparison of the patients treated with PE and IVIg showed no significant differences in terms of effectiveness in improving the clinical course of GBS: at one month, respectively 11.1% and 25% had recovered, and 55.5% and 58.3% had improved by at least one grade. These results are in agreement with those of the Dutch GBS trial. No relapses were observed in either group. Moreover, our results showed no difference in clinical outcome at 1 and 3 months between the patients receiving only one therapy and those receiving two; a second cycle of therapy did not seem to improve the clinical course of the disease significantly. We conclude that PE and IVIg are both safe and effective therapies for GBS.

The relationship between polyneuropathies and monoclonal gammopathies is well known even though the pathogenetic hypotheses are controversial. The role of autoantibodies against neural antigens has been recently underlined. 45 patients (29 M and 16 F), affected by multiple myeloma (MM) non-Hodgkin lymphoma (NHL) with paraproteinemia and monoclonal gammopathies of undetermined significance (MGUS)4, underwent an EMG study including SCV, MCV and late responses of several nerves, and a search for serum antibodies against neural antigens by immunoblotting assay. 19 out of 45 pts. tested positive to EMG and 15 out of 45 (10 MM and 5 NHL) showed a serological positivity. Among them 11 were positive to EMG too. The results confirm the hypothesis of a possible pathogenetic role of high-titer autoantibodies against neural antigens in cases of polyneuropathy.

The relationship between monoclonal gammapathies and peripheral nerve involvement is well known. The pathogenesis of neuropathy has been ascribed to infiltrative, compressive, ischemic or haemorrhagic phenomena, to an amyloid storage or to antibody autoaggression toward neural antigens. Forty-five patients suffering from monoclonal gammapathy of uncertain significance (MGUS), multiple myeloma (MM), MG IgM, were submitted to a neurophysiological examination. 10 pts. were excluded because of neurotoxic drugs, diabetes or alcohol abuse. The investigation included SCV of median, ulnar, and sural n., MCV and F wave of peroneal n., H reflex, and computerized analysis of recruitment pattern. A subclinical neuropathy was found in 11 of 35 pts. (30%) without any neurological symptom. A CTS was found in 4 patients. The high rate of subclinical neuropathy in asymptomatic pts. is underlined by the Authors, while the presence of subclinical neuropathy in cases of MGUS needs further studies.

Three familial cases of idiopathic hypertrophy of the masticatory muscles are reported. The muscular enlargement was noted in late childhood or early adult life, was bilateral, insidious, painless and non-progressive. Electrophysiological and laboratory findings were normal in one case. CT examination, performed in two cases, showed homogeneous bilateral enlargement of masseter muscle bulk consistent with true hypertrophy. Two patients also showed congenital malformations. A review of the literature showed that this disorder is very rare.

In four patients, calf muscular hypertrophy developed after the onset of sciatica. Hypertrophic muscles were weak and showed electromyographic signs of denervation. In all cases, calf muscle biopsy showed striking hypertrophy of type 1 and, especially, type 2 muscle fibers. This hypertrophy was associated with other signs indicating a neurogenic lesion. Muscle hypertrophy is a rare finding in neurogenic lesions. Stretch and exercise of muscle are probably the causative factors.

Five cases of Shy-Drager Syndrome (SDS) are reported. All patients showed marked muscular wasting often with fasciculation and without sensory loss. Clinical, electromyographic and in one case, pathological findings in the spinal cord indicated a lesion at the level of the anterior born cell. An extensive review of the literature disclosed a significant number of cases of SDS displaying amyotrophy referable to a spinal lesions.