Intravenous immunoglobulin is effective in patients with diabetes and with chronic inflammatory demyelinating polyneuropathy: long term follow-up.

BACKGROUND AND AIMS: Chronic inflammatory demyelinating polyneuropathy (CIDP) seems to be more common in patients with diabetes than in the general population. The long term outcome of these patients after receiving intravenous immunoglobulin is unclear and the precise optimal regimen needed has yet to be ascertained. Moreover, the influence of chronic hyperglycaemia on this neuropathy is not clear. METHODS: This prospective follow-up study included all consecutive patients with diabetes with a CIDP referred to our department during the 18 months of the study. RESULTS: 198 consecutive patients were referred to our neuromuscular unit and exhaustively screened. 16 patients with diabetes (8%) had a demyelinating polyneuropathy fulfilling the most restrictive diagnostic criteria for CIDP. They were treated with at least one course of intravenous immunoglobulin and, if responders, retreated in case of relapse. All patients were followed for at least 40 months. Patients with diabetes with CIDP significantly improved after immunotherapy and during follow-up. The Neuropathy Impairment Score changed from 38 at presentation to 16 at the end of the follow-up. Eight patients developed distal sensory disturbances during follow-up and four of these patients complained of distal paresthesias but no neuropathic pain. Sensory disturbances were detected after 30 months (mean time) from baseline. CONCLUSION: CIDP is not an unusual neuropathy in patients with diabetes. Our study underlines the importance of extensively investigating patients with diabetes with polyneuropathy to identify those with a treatment responsive demyelinating polyneuropathy.

Circulating levels of tumour necrosis factor-alpha and its soluble receptors are increased in the blood of patients with amyotrophic lateral sclerosis.

An immunologic pathogenesis for amyotrophic lateral sclerosis (ALS) has been recently proposed. We tested the whole tumour necrosis factor (TNF) system in the serum of 51 ALS patients at different stages of the disease and 36 healthy controls. Antigenic TNF-alpha and its soluble receptors (sTNF-Rs), measured by ELISA, were significantly higher in ALS patients than in healthy controls. However, biologically active TNF-alpha, corresponding to the sTNF-Rs-unbound trimeric TNFalpha molecule and assayed by its cytotoxic activity on a sensitive cell line, was similar between ALS patients and healthy controls. Neither antigenic TNF-alpha, bioactive TNF-alpha nor sTNF-Rs correlated with disease severity, disease duration, or weight loss. In conclusion, we reported an activation of the TNF system in ALS. The role of this activation in the pathogenesis of the disease remains elusive.

Quantitative electroencephalography in amyotrophic lateral sclerosis.

OBJECTIVES: The authors evaluated quantitative EEG (QEEG) in patients with amyotrophic lateral sclerosis (ALS), in order to see if the confined cortical degeneration found in anatomical and functional examinations of central (rolandic) regions could give rise to abnormalities of cortical electrical activity. MATERIAL AND METHODS: Eighteen patients with ALS were compared with 14 control subjects comparable for age and sex. The QEEG analysis was blind to the clinical data. Frequency analysis of 30 EEG epochs, each of 2.6 s duration, was performed. RESULTS: QEEG in ALS patients showed a significant well-localized decrease of alpha activity only in the central regions, while theta and delta relative power did not show any difference from controls. CONCLUSION: We suggest that QEEG is able to show evidence of subtle EEG changes probably due to loss of cells in the somatomotor cortex selectively affecting the generator sites.

Motor evoked potentials and disability in secondary progressive multiple sclerosis.

BACKGROUND: To investigate the mechanisms underlying disability in multiple sclerosis (MS), 40 patients with the relapsing-remitting form of the disease and 13 patients with secondary progressive MS underwent multimodal evoked potential (EP), motor evoked potential (MEP), and spinal motor conduction time evaluation. Clinical disability was evaluated by the expanded disability status scale (EDSS) and functional system scales. In secondary progressive MS patients, magnetic resonance imaging (MRI) was used to obtain a semiquantitATive estimate of the total lesion load of the brain. RESULTS: Spinal motor conduction time was significantly longer in secondary progressive MS patients than controls (p < 0.001) and relapsing-remitting MS patients (p < 0.05), but did not differ between relapsing-remitting patients and controls. Spinal motor conduction times also correlated directly with EDSS scores (p < 0.001) and pyramidal functional system scores (p < 0.001). Brain lesion load (4960.3 +/- 3719.0 mm2) and the total number of lesions (67.7 +/- 37.0) in secondary progressive MS did not correlate with disability scores. For the following EPs, the frequencies of abnormalities were significantly higher in secondary progressive MS patients than relapsing-remitting patients: visual evoked potentials (p < 0.05), somatosensory evoked potentials and upper limb motor evoked potentials (p < 0.01), and brainstem auditory evoked potentials, lower limb somatosensory evoked potentials and lower limb motor evoked potentials (p < 0.001). CONCLUSIONS: These findings suggest that disability in secondary progressive MS patients is mainly due to progressive involvement of corticospinal tract in the spinal cord.

Breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis.

The aim of this study was to evaluate the time course of breathing pattern and respiratory mechanics in patients with amyotrophic lateral sclerosis (ALS). A study was conducted on 25 out of 38 eligible ALS patients. Neurological status, arterial blood gases (ABGs), spirometry, breathing pattern (minute ventilation (V'E), tidal volume (VT), respiratory frequency (fR), duty cycle (duration of inspiration/duration of total breathing cycle (tI/ttot)), respiratory drive (P0.1)), respiratory mechanics (oesophageal pressure (Ppl), dynamic compliance (CL,dyn), pressure time product (PTP) and index (PTI), work of breathing (WOB)), and respiratory muscle (RM) strength as assessed by maximal oesophageal pressure (Ppl,max) were evaluated at presentation (to) in all patients and after 6 months (t6) in 11 patients. At to, the mean values of the degree of neurological impairment were 60+/-20 and 103+/-30 as assessed by the Norris scale and Medical Research Council (MRC) score, respectively. From the time of the first neurological symptom, survival time ranged 7-50 months. Diurnal ABGs were normal. A mild restrictive pattern was observed, a forced vital capacity (FVC) <70% of predicted being present in 45% of patients, only FVC % pred (r=0.59; p<0.05), forced expiratory volume in one second (FEV1) % pred (r=0.53; p<0.05) and survival (r=0.64; p<0.05) showing a significant correlation with the Norris scale. A Ppl,max <30 cmH2O was associated with a significantly greater mortality, Ppl,max being correlated with survival (r=0.79, p<0.05). At t6, fR, fR/VT, P0.1/Ppl,max, were significantly increased in comparison to to, while FVC % pred, vital capacity (VC) % pred, FEV1 % pred, VT and Ppl,max were significantly reduced. These results suggest a progressive deterioration in breathing pattern and in respiratory muscle strength with progression of disease.

Conjugal amyotrophic lateral sclerosis: toxic clustering or change?

We describe a southern Italian couple, married for 28 years, who developed amyotrophic lateral sclerosis (ALS) within 30 months of each other. They had drunk water taken from an artesian well for nearly thirty years. Samples of the water were investigated in the search for heavy metals and pesticides using high performances liquid chromatography, gas chromatography and absorption spectrometry. No heavy metals, pesticides or known environmental toxic substances were found in significant concentration. This would seem to support the view that conjugal ALS in Caucasians is due to mere coincidence.

An unusual increase in haemodynamic values in a sleep apnoea syndrome patient with prolonged apnoea.

This case report shows the results of a right heart catheterisation in a patient with sleep apnoea syndrome (SAS). Arterial oxygen saturation (SaO2), heart rate, oronasal flow, and monitoring of electroencephalographic (EEG), sovrayoidal electromyographic (EMG) and thoracoabdominal movements were recorded simultaneously. A mixed apnoea of 180 s was registered, SaO2 fell to 54% and systolic pulmonary artery pressure (sPAP) increased to 130 mmHg. Slowly, haemodynamic values began to normalise with the reappearance of respiratory acts. We can hypothesize that this patient's nocturnal apnoeas may cause frequent and deep negative pleural pressure swings, with severe continuous action on pulmonary circulation. This case report has shown that a SAS patient is capable of developing an adaptative response to an unusual and remarkable systolic pulmonary artery pressure increase, which is mainly due to hypoxic vasoconstriction.

Respiratory muscle function and exercise capacity in multiple sclerosis.

Patients with multiple sclerosis (MS) show a poor exercise tolerance. A reduction in respiratory muscle strength has also been reported. The purpose of this study was to evaluate whether reduction in exercise tolerance was related to respiratory muscle dysfunction. Twenty four multiple sclerosis patients (mean +/- SD age: 48 +/- 9 yrs, duration of illness 12.2 +/- 6 yrs, severity of illness as assessed by Expanded Disability Scale Score (EDSS) 5.3 +/- 2), underwent detailed evaluation of lung function tests, arterial blood gas analysis, respiratory muscle strength and endurance, and exercise test on an arm ergometer. Sixteen of the 24 patients were able to perform the exercise test (Group I), whilst the other eight were not (group II). Arterial blood gases and lung function tests were normal for both groups. Respiratory muscle strength as assessed both by maximal inspiratory pressure (MIP) and maximal expiratory pressure (MEP) was significantly reduced (MIP 18-76 cmH2O; MEP 16-82 cmH2O) compared to predicted values. Inspiratory muscle endurance time was significantly reduced in Group II in comparison to Group I (247 +/- 148 vs 397 +/- 154 s, respectively). Both MIP and MEP were significantly related to inspiratory muscle endurance time. Endurance time, MIP and MEP were inversely significantly related to duration of illness, whilst only endurance time was significantly related to Expanded Disability Scale Score.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebral mapping in subjects suffering from migraine with aura.

Cerebral mapping of the spontaneous electroencephalographic activity was performed in 31 subjects suffering from migraine with aura and the results were compared with those of a matched control group. All the patients were examined during the interictal period. While traditional visual interpretation of EEG records was negative in all but five cases (16.1%) spectral analysis showed an asymmetry in alpha total power over the posterior regions in 13 cases (41.9%) and an asymmetry in alpha band peak frequency in 17 (54.8%). By means of cerebral mapping and statistical significance probability mapping we were able to demonstrate a significant increase in alpha total power in 13 cases (41.9%) and a regional increase in delta and theta total power in 20 cases (64.5%). In comparison with the control group, the migraine patients showed a widespread increase in slow activity (theta and delta) mostly over the temporal regions.