Primary progressive multiple sclerosis: clinical and paraclinical characteristics with application of the new diagnostic criteria.

The aim of our study was to analyse clinical and paraclinical characteristics of patients with multiple sclerosis (MS) with previous diagnosis of primary-progressive (PP) MS according to the Poser's criteria and further investigate if they fulfil the McDonald's diagnostic criteria for this disorder. A total of 561 MS patients were registered in the database at the Institute of Neurology, Belgrade, from 1 January 1997 to 31 December 2000 and 63 of them (11.2%) with previous diagnosis of PPMS were analysed retrospectively. Male/female ratio was 1.3:1 and mean age at onset 33.2 years. Most frequent at onset were pyramidal (in 73% of patients) and sensory symptoms (in 41% of patients); 74.6% of patients had greater than or equal to nine brain magnetic resonance imaging (MRI) lesions. Intrathecal oligoclonal immunoglobulin G (IgG) was detected in 96.7% and prolonged visual evoked potentials (VEP) P100 latency in 82.4% of patients. Of the total study group of 561 patients, 10.2% fulfilled the recently recommended McDonald's diagnostic criteria for the diagnosis of PPMS. Our findings further support the significance of the brain/spinal cord MRI, cerebrospinal fluid and VEP findings for precise diagnostic assessment in patients with suspected PP form of MS.

Nitric oxide metabolites and interleukin-6 in cerebrospinal fluid from multiple sclerosis patients.

Interleukin-6 (IL-6) and nitric oxide (NO) are implicated in the pathology of multiple sclerosis (MS). We have investigated the levels of these mediators in the cerebrospinal fluid (CSF) from 50 patients with MS and 23 control subjects. Mean CSF IL-6 level was higher in the total MS group in comparison with controls, but not significantly, whilst the difference between patients with stable MS and controls reached the level of statistical significance. Mean CSF nitrite/nitrate level was significantly higher in the total MS group compared with the control group, as well as in active MS patients versus controls. There was significant difference neither in the mean CSF IL-6 nor in nitrite/nitrate levels between active and stable MS patients. Interestingly, we observed a significant negative correlation between IL-6 and nitrite/nitrate levels in the CSF in the total MS group. Such a trend existed in both subgroups with active and stable MS, but without reaching the level of statistical significance. Our data further support the involvement of IL-6 and NO in ongoing pathological processes in MS, suggesting their potential interplay within the central nervous system in this disease.

[Topographic distribution of multiple sclerosis in the Belgrade municipalities]. / Topografska distribucija multiple skleroze u opstinama Beograda.

The frequency of multiple sclerosis (MS) varies greatly in different parts of the world, but also within some countries. Many epidemiological surveys of geographical pattern of MS identified areas of different risk of MS in relation to the latitude. The study of disease clusters is a potentially powerful strategy in revealing the aetiology of a disease. A series of space and time clusters of MS has been described over the years, especially in islands' populations. The Belgrade region is located on 628 m height above the sea level, between latitudes 44 and 45 degrees north of the equator, and longitudes 20 and 21 degrees east of the Greenwich. The district of Belgrade is divided into 16 municipalities and extends over an area of 3,222 km2. According to the 1991 census, the area has a population of 1,602,226 inhabitants. All persons who were affected and/or died from MS with residence in Belgrade region had been registered from January 1,1985 to December 31,1996, on the basis of a retrospective analysis of hospital records. The main sources for this study were hospital case records in all 4 neurological hospitals in Belgrade (Institute of Neurology of the Serbian Clinical Centre, which is the primary referral national centre for suspected/diagnosed MS patients in Serbia, and three Departments of Neurology which belong to Zvezdara Clinical Centre, Dr. Dragisha Mishovitsh Clinical Centre, and Military Medical Academy). Only clearly diagnosed and fully documented cases with clinically and laboratory supported MS according to Poser's criteria were included. For each patient, two neurologists (JD and NS) and one epidemiologist (TP) reviewed all data collected in order to verify the validity of the diagnosis. The prevalence, as a number of persons with MS in the population at risk, was calculated on December 31, 1996, for each municipality and for the Belgrade population as a whole. The Belgrade municipalities were categorized according to MS prevalence level, as category 1 (> or = 50/100,000)--Stari Grad, Vrachar, Vozhdovac, Rakovica, Barajevo, Zvezdara; category 2 (30-49/100,000)--Savski Venac, Novi Beograd, Chukarica, Zemun; and category 3 (< 30/100,000)--Palilula, Sopot, Grocka, Obrenovac, Lazarevac and Mladenovac. The geographic distribution was estimated according to the prevalence of MS, and hypothetical cluster of the disease was tested by chi-square test. On December 31, 1996, the prevalence of MS in the Belgrade population was 44.87/100,000. The highest MS prevalence was registered in the municipality of Vrachar (74.63/100,000), and the lowest in the municipality of Mladenovac (17.73/100,000) (Table 1). Municipalities which are grouped within the 3 categories are not statistically significantly clustered (chi-square = 1.479, p > 0.05).

Uric acid levels in sera from patients with multiple sclerosis.

The levels of uric acid (UA), a natural peroxynitrite scavenger, were measured in sera from 240 patients with multiple sclerosis (MS) and 104 sex- and age-matched control patients with other neurological diseases (OND). The mean serum UA concentration was lower in the MS than in the OND group, but the difference did not reach the level of statistical significance (P = 0.068). However, the mean serum UA level from patients with active MS (202.6 + 67.1 mumol/l) was significantly lower than that in inactive MS patients (226.5 + 78.6 mumol/l; P = 0.046) and OND controls (P = 0.007). We found a significant inverse correlation of serum UA concentration with female gender (P = 0.0001), disease activity (P = 0.012) and duration (P = 0.017), and a trend towards an inverse correlation with disability as assessed by EDSS score, which did not reach statistical significance (P = 0.067). Finally, multivariate linear regression analyses showed that UA concentration was independently correlated with gender (P = 0.0001), disease activity (P = 0.014) and duration of the disease (P = 0.043) in MS patients. These findings suggest that serum UA might serve as a possible marker of disease activity in MS. They also provide support to the potential beneficial therapeutic effect of radical-scavenging substances in MS.

Raised cerebrospinal fluid nitrite and nitrate levels in patients with multiple sclerosis: no correlation with disease activity.

A growing body of evidence implicates excessive generation of nitric oxide (NO) within the central nervous system (CNS) in multiple sclerosis (MS). The aim of our study is to analyse nitrite and nitrate as end products of NO in the cerebrospinal fluid (CSF) from MS patients and correlate the concentrations with clinicol characteristics of the disease. CSF nitrite and nitrate concentrations were measured after reduction of nitrate, by Griess reaction, in 105 MS potients, 27 patients with non-inflammatory neurological disorders (NIND) and 13 individuals without neurological disorder (Co). Mean CSF nitrite and nitrate concentrations were significantly higher in patients with MS and NIND compared with the Co patients (9.44 and 8.68, respectively, versus 6.85 microM; P=0.0001 and P=0.031, respectively). There was no significant correlation between CSF nitrite and nitrate concentrations and activity, phase, severity and duration of MS. Our data are in agreement with the results of previous studies which have demonstrated raised concentrations of CSF NO metabolites in MS patients, providing further evidence for NO involvement in MS. The lack of correlation between NO metabolites and disease activity speaks in favour of the possible dual role of NO, as both immunoregulatory and pro-inflammatory molecule, in the pathogenesis of MS.

Seizures in multiple sclerosis.

PURPOSE: In patients with multiple sclerosis (MS), epileptic seizures occur more frequently than in the general population. The aim of this study was to analyze clinical characteristics of epilepsy in patients with MS, potential correlation between the semiology of seizures, EEG and magnetic resonance imaging (MRI) findings in these patients, as well as to examine the response to anticonvulsant therapy. METHODS: In a series of 268 consecutive patients with definite MS hospitalized at the Institute of Neurology, Belgrade, we identified 20 (7.5%) patients with seizures or epilepsy. All patients with seizures or epilepsy were submitted to standard EEG and brain MRI with gadopentetate dimeglumine. RESULTS: In four patients, epilepsy occurred 1-5 years before other clinical manifestations of MS. Eight patients had seizures only during MS relapses (provoked seizures). In two of them, seizures were the only manifestations of relapse. In 12 patients, seizures occurred regardless of the phase of MS (chronic epilepsy). In the majority of patients, seizures were partial with secondary generalization. Five patients experienced episodes of status epilepticus, and they all had dementia. Abnormal EEG pattern was found in 11 patients. Brain MRI disclosed cortical-subcortical lesions in nine patients and focal cortical atrophy in one, whereas in the remaining patients, findings were inconclusive. Probable EEG-MRI-seizure type correlation existed in 10 patients. CONCLUSIONS: Our data suggest that epilepsy may represent an initial symptom of MS and a single clinical manifestation of a relapse, and further support the assumption of the existing correlation between the presence of cortical-subcortical lesions and epileptic seizures or epilepsy in patients with MS.

Prevalence of multiple sclerosis in Belgrade, Yugoslavia.

OBJECTIVES: To estimate the distribution of multiple sclerosis in the Belgrade population. METHODS: All persons who were affected and/or died from multiple sclerosis (Poser's criteria), with residence in the Belgrade region had been collected from January 1, 1985 to December 31, 1996. Prevalence was adjusted by direct method, using world population. RESULTS: From 1985 to 1996, 823 patients were suffering from multiple sclerosis. Sex ratio was 1:1.9. The mean age at onset was 32.2 +/- 9.8 years. A relapsing-remitting course of multiple sclerosis was reported in 50.7% patients, secondary progressive in 36.4%, patients, and primary progressive in 12.9% patients. On December 31, 1996, age-adjusted prevalence of multiple sclerosis in Belgrade was 41.5/100,000, 28.2/100,000 for males, and 54.1/100,000 for females. During the period studied, statistically highly significant increasing trend of multiple sclerosis prevalence was observed (P = 0.0001). CONCLUSIONS: According to findings presented in this study, Belgrade is an area with high prevalence of multiple sclerosis.

CNS tumours: oligoclonal immunoglobulin D in cerebrospinal fluid and serum.

Oligoclonal immunoglobulin D bands were detected by isoelectric focusing in 7 out of 25 unconcentrated cerebrospinal fluid (CSF) samples obtained from patients with tumours of the central nervous system (CNS). The tumours were confirmed by clinical and histological findings. Two patients with CNS malignancy had intrathecal synthesis of oligoclonal bands both IgD and IgG. Four patients with a variety of CNS tumours had a systemic IgD immune response but no oligoclonal IgG bands. One patient with the most malignant tumour histology had a systemic IgD response as well as local synthesis of IgG. The study reveals several new aspects regarding CNS tumours: they are immunologically active and are capable of invoking oligoclonal immunoglobulin production both within the CNS and systemically. Multiple immunoglobulin activation can be found in malignant CNS tumours, and systemic IgD production may occur independently from IgG synthesis and may represent an immune response to a neoantigen produced in the CNS compartment.

Prognostic factors for survival in multiple sclerosis.

In a hospital-based study of 119 patients with definite multiple sclerosis, demographic and clinical factors were analysed with respect to their validity in assessing the long-term prognosis. Over a mean follow-up of 21.7 years, the following factors negatively influenced the prognosis by the univariate analysis: male sex, age at onset over 25, pyramidal involvement or spasticity at onset, > or =3 functional systems affected at onset or after 5 years, incomplete first remission, length of the first remission < or =1 year, >5 attacks in the first 10 years, secondary or primary-progressive disease, time to reach secondary progression over 5 years and time to reach EDSS 6 over 7 years. The multivariate model showed that in patients with relapsing-remitting disease, 5 years after onset, pyramidal involvement at onset and shorter time to reach EDSS 6 predicted poor outcome, while after 10 years, higher age at onset and incomplete first remission indicated poor prognosis. Ten years after onset, the predictors of poor outcome in the secondary-progressive group were shorter time to reach EDSS 6 or secondary progression and higher EDSS, while in the primary-progressive group those variables were spasticity or higher number of functional systems affected at onset, and higher EDSS after 5 and 10 years.

Febrile convulsions in a Serbian region: a 10-year epidemiological study.

The first population-based study in the central region of the Republic of Serbia (total population 283,103) was carried out to assess some epidemiological features of febrile convulsions among children of between 6 months and 5 years of age. During the 10-year period, 1986 to 1995, there were 570 cases of the first febrile convulsions (287 males and 283 females). The average annual incidence rate was 3/1000 (2.9/1000 in males and 3.0/1000 in females), with the highest in 1995. During the study period, a significantly increased linear regression trend was observed. During the follow-up period of 5 years for children who had their first febrile convulsions in 1989 and 1990 (total 154 cases), 27 (17.5%) had a recurrence of the disorder, and ten (6.5%) had one or more afebrile seizures, of whom seven children (4.5% of total sample) developed epilepsy (recurrent afebrile seizures).

Urinary excretion of phenobarbital and its metabolite p-hydroxyphenobarbital in convulsing and non-convulsing patients.

As part of an investigation of phenobarbital (PB) pharmacokinetics in patients with status epilepticus (SE), urinary excretion of PB and its main metabolite, hydroxyphenobarbital (HPB), was studied in patients who had an episode of SE, as well as in non-convulsing ones. Eleven in-patients were studied:(group 1) five patients (4 M + 1 F; 48 +/- 28 years old; 64 +/- 6 kg body weight; mean +/- SD) with convulsive status epilepticus, and (group 2) six patients (5 M + 1 F; 37 +/- 13 years old; 71 +/- 15 kg body weight) with epilepsy, seizure-free at the moment of PB administration and without established anti-epileptic therapy. All subjects received a single intravenous dose of PB (15 mg/kg) at a rate of 100 mg/min. PB and HPB concentrations were measured by high performance liquid chromatography with UV detection at 220 nm in urine samples collected throughout 24 h. The comparison of pharmacokinetic parameters of urinary excretion of PB and HPB showed a statistically significant difference in the values of recovery of HPB and total barbiturate (higher values in the patients with SE) in 24 h urine. Differences in the excretion of PB between the two groups of patients--higher values in the patients who had had an episode of SE, and in urine flow--slightly elevated volumes in the same group, failed to reach statistical significance, probably due to the small number of participants in the study.

[Juvenile myoclonic epilepsy]. / Juvenilna mioklonicka epilepsija.

CONCLUSION: We conclude that despite inevitable variability the clinical picture of JME is characteristic. It is easy to diagnose JME if one thinks of it while the history should be thoroughly analyzed. An EEG recording during sleep confirms the diagnosis. An early diagnosis of JME permits adequate prognosis of the subsequent course of epilepsy, and adequate therapy brings remission in most of the patients. If treatment starts following the large number of severe GTC seizures, the response to therapy is incomplete. The persistency of the illness throughout the life, the need for continuous medication and therapeutic unresponsiveness in cases with late diagnosis, do not justify the increasing misconception that JME is of benign nature. Diagnosis of JME is rare because of insufficient familiarily of physicians with the illness. BACKGROUND: Juvenile myoclonic epilepsy (JME) is an idiopathic generalized epileptic syndrome characterized with the combination of myoclonic, generalized tonic-clonic (GTC) and absence seizures that are readily provoked by sleep deprivation. PATIENTS: Forty-three patients, aged from 14 to 51 years, participated in a 5-year follow-up study. Diagnosis was made according to the criteria (Table 1) for diagnosis of JME set by Panayiotopoulos et al. (1994). Nineteen patients made their first contact with a neurologist at the Institute of Neurology and were diagnosed as JME, while the remaining 24 were referred to from other medical institutions with a diagnosis of therapy resistant to focal epilepsy. All patients underwent a somatic and neurological examination, "mini mental test," EEG in waking and CT scan of the brain. Some patients had EEG performed during sleep and some had MRI of the head. RESULTS: JME began between 9 and 26 (average 17) years. All patients had myoclonic seizures, 98% had GTC and 23% absence seizures. The first myoclonic seizure occurred between 9 and 24 years while the frst GTC seizure occurred between 10 and 32 years. Myoclonic seizures (83% of patients) and GTC seizures (70% of patients) occurred most often immediately after awaking. The most frequent provocative factors were insufficient sleep, alcohol abuse and tiredness. Epilepsy in the family was present in 39%, focal neurological deficiency in 9% and pathological findings on of CT and MRI in 7% of patients. Waking EEG was pathological in 77% of patients; it included generalized spike-wave discharges in 73%, multiple spike-wave complexes in 33% and focal discharges in 12% of patients, respectively. In all 26 patients tested, sleep EEG was pathological most often with multiple spike-wave complexes in 85% and 3-4 Hz spike-wave complexes in 57% of patients. The correct diagnosis of JME following a comprehensive examination was made in 24 (56%) patients after a delay of 1 to 35 years. In 24 patients with delayed diagnosis of JME the replacement of earlier medication with valproic acid (VPA) induced remission in 18 patients (75%) while 1 patient (4%) experienced a reduction in the number of seizures. Five patients (21%) did not respond to VPA medication: 2 due to a weak compliance, another 2 due to inefficient medication and 1 because of the preexistent malabsorption syndrome. In 19 patients (44%) with initial diagnosis of JME, VPA was introduced immediately upon diagnosis. Of them, 15 (79%) had excellent response to VPA, 1 refused therapy and for 3 patients there is no information. In 2 patients VPA was substituted due to side effects (hepatotoxicity and alopetia) with lamotrigine (low doses), which brought about decrease in frequency and mitigation in myoclonic seizures.

Serum interleukin-12 levels in patients with multiple sclerosis.

Increasing body of evidence which suggests a crucial role for interleukin (IL)-12 in modulating immune responses in multiple sclerosis (MS) prompted us to analyze IL-12 in serum from MS patients. We measured the sera concentrations of IL-12, tumor necrosis factor (TNF)-alpha and transforming growth factor-beta1, in 21 MS patients and 13 patients with non-inflammatory nervous diseases. In clinically active MS, serum levels of IL-12 were detectable in 53% and TNF-alpha in 40% of patients. None of the patients with clinically inactive MS had detectable IL-12 and TNF-alpha sera levels. Analysis of serum concentrations of all three cytokines revealed no significant differences between MS patients and controls. These findings provide further evidence that both IL-12 and TNF-alpha might have an active role in immunopathogenesis of MS.

Unusual association of multiple sclerosis and tomaculous neuropathy.

We describe two cases in which multiple sclerosis (MS) occurred in association with tomaculous neuropathy, presenting as chronic, distal sensorimotor polyneuropathy. In Case 1, monoclonal gammopathy of undetermined significance with monoclonal IgG lambda reactive against GM1 ganglioside, was also detected. The diagnosis of tomaculous neuropathy was established after sural nerve biopsy. Teased fibers examination revealed focal 'sausage-like' thickenings of the myelin sheaths in intact fibers and in fibers with segmental demyelination. Electron microscopy showed them to be due, mostly, to multiple windings of redundant myelin and concentric apposition of numerous lamellae, in contact with an intact myelin sheath. These are the first reported cases of tomaculous neuropathy in patients with MS. Whether the combination of the two conditions is purely coincidental or suggests the possible causal relation between MS and tomaculous neuropathy, is not certain.

Seizure disorders in preschool children in a Serbian district.

All children born in 1989 and 1990 in the district of Krusevac in Central Serbia (population 140,000) were followed to the end of 1995. The total number of children was 3,102. The cumulative incidence of febrile seizures was 25.1/1,000, that of single febrile seizures 3.9/1,000 and that of epilepsy (i.e. recurrent afebrile seizures) 6.5/1,000. The peak age-specific rate of the first febrile and the first afebrile seizure was in the second year of life (11.6/1,000 and 2.9/1,000, respectively). Among 20 cases of epileptic children, the most common type of seizure was the generalized type--14 cases (70%), while 6 children (30%) suffered from partial seizures. Of these epileptic children, there were 7 children (35%) with associated neurological abnormalities and/or mental retardation, including one with tuberous sclerosis. Thirteen of the total number of epilepsy cases were considered as having active epilepsy (i.e. still having had seizures and/or being on antiepileptic therapy). The calculated point prevalence rate was 4.2/1,000 on December 31, 1995.

A 44-month clinical-brain MRI follow-up in a patient with B12 deficiency.

We report a 51-year-old woman with vitamin B12 deficiency who presented with slight megaloblastic anemia and severe neurologic deficits associated with multiple focal and confluent T2-weighted white matter hyperintensities on brain MRI. Forty-four months after initiation of hydroxocobalamin therapy, there was clinical improvement and striking reduction in the MRI abnormalities. B12 deficiency should be considered in the differential diagnosis of neurologic disorders associated with multiple areas of white matter hyperintensities on T2-weighted brain MRI.

Interleukin-12 and tumor necrosis factor-alpha levels in cerebrospinal fluid of multiple sclerosis patients.

Concentrations of interleukin (IL)-12 and tumor necrosis factor-alpha (TNF-alpha) in cerebrospinal fluid (CSF) were measured in patients with multiple sclerosis (MS) and control patients with non-inflammatory neurological diseases (NIND) by an enzyme-linked immunosorbent assay. TNF-alpha was detectable in the CSF of 60% of the patients with active MS, none of those with inactive MS and 29% of patients with NIND. CSF concentrations of TNF-alpha correlated with the degree of disability in MS patients (P < 0.05). Detectable levels of IL-12 were found in 10% of the MS CSF samples and 18% of NIND CSF samples. There was a significant relationship between CSF concentrations of IL-12 and those of TNF-alpha in MS patients (P < 0.05); no relationship was observed between the presence of IL-12 and disease activity or severity. These findings further stress the involvement of T helper 1 type-response within the central nervous system in MS.

[Sensitivity of criteria for MRI interpretation in patients with multiple sclerosis]. / Senzitivnost kriterijuma za interpretaciju nalaza magnetine rezonancije kod bolesnika od multiple skleroze.

UNLABELLED: Magnetic resonance imaging (MRI) has become the preferred imaging technique in multiple sclerosis (MS). Areas of increased signal (AIS) are detected on T2-weighted (T2W) pulse sequencies in 70-100% of patients with clinically definite MS. However, AIS similar to those seen in MS have also been described in healthy elderly subjects and patients with various neurological disorders including several vasculitises, migraine, and trauma. The aim of this study was to test and compare the sensitivity of different currently used MRI criteria for the diagnosis of MS. METHOD: The study comprised 49 patients wiht clinically definite MS diagnosed according to McAlpine's criteria. Cranial MRI was performed on a 1.5 T Magnetom with spin-echo pulse technique defined by relaxation times: T1W, T2W and proton density. Gadolinium-DTPA was not administred. We interpreted only spin-echo T2W images because they are the most sensitive for MS. The presence, number, size and location of AIS were recorded for all scans. We calculated the sensitivity for the four different sets of criteria (Paty's A and B criteria, Fazekas' criteria and Barkhof's criteria). The presence of four AIS greater than 3 mm was designated as Paty's A criteria, and presence of three AIS, one periventricular, greater than 3 mm was designated as Paty's B criteria. Fazekas' criteria require the presence of at last three AIS and two of the following three features: abutting body of lateral ventricles, infratentorial lesion location, and size = 6 mm. Barkhof's criteria were considered fulfilled if at least eight AIS were present, one infratentorial. RESULTS AND DISCUSSION: Brain MRI revealed normal finding in four (8%) of 49 studied patients with clinically definite MS. Number, size and location of AIS detected in the remaining patients is presented in Table. Characteristic patterns of MRI lesions on T2W images fulfilling different sets of criteria for the diagnosis of MS are presented in Figures 1-3. Using Paty's B criteria, sensitivity of MRI in patients with clinically definite MS was the highest, reaching 92%. Applying Paty's A criteria sensitivity slightly decreased to 88%. Using Fazeka's and Barkhof's criteria led to a further, significant decrease in sensitivity (71% and 57%, respectively; p = 0.045). In 1993, Offenbacher et al. have reviewed 1500 consecutive brain MRI scans for the presence, number, size and location of AIS, and calculated the sensitivity and specificity of Paty's and Fazekas' criteria with respect to clinical evidence for MS. In this study, similar to our results, using Paty's criteria resulted in higher sensitivity (87% for Paty A and 90% for Paty B) than Fazekas' criteria which led to decrease in sensitivity (81%). However, when Fazekas' criteria were used, decrease in sensitivity was associated with highly significant improvement of specificity. In conclusion, according to our and previously published results, Paty's criteria based on the presence of three AIS, one periventricular, or on the presence of four AIS, greater than 3 mm, are the most sensitive for MRI interpretation in MS. Other criteria (greater number of AIS, greater AIS diameter, infratentorial location and gadolinium enhancement) should be used, in order to improve the specificity, only to the MRIs of elderly patients (> 50 years) with suspected MS or patients suspected of MS in whom alternative explanations seem equally similar.

[Treatment of diabetic amyotrophy with steroids]. / Lecenje dijabeticke amiotrofije steroidnim lekovima.

The cause of diabetic neuropathies has not yet been fully elucidated. In severe forms of proximal diabetic neuropathy, the role of ischaemia caused by inflammatory microvasculopathy has been recently implicated, suggesting the use of anti-inflammatory and immunosuppressive therapeutic agents. We present a 70-year-old man with non-insulin-dependent diabetes mellitus, who developed diabetic amyotrophy responsive to steroids. Pain preceded the development of severe, asymmetric, lower extremities weakness, with amyotrophy, areflexia and fasciculations. Weakness gradually worsened over a 15-month period. The electrophysiologic study revealed predominant motor and axonal polyneuropathies. Laboratory analyses showed elevated erythrocyte sedimentation rate (130/h), moderately elevated cerebrospinal fluid (CSF) protein level and mildly elevated CSF cell content. Therapy with methylprednisolone (120 mg dailu, i.m., with slow tapering) was administered with beneficial effect. Pain subsided immediately after starting the therapy, and strength gradually improved. In conclusion, immunosuppressive treatment should be considered in selected patients with diabetic amyotrophy who develop severe and disabling weakness of the lower extremities.