Progressive encephalomyelitis with rigidity: a case report.

BACKGROUND/OBJECTIVE: The most prominent clinical features of progressive encephalomyelitis with rigidity (PER) are painful spasms and rigidity accompanied by clinical signs of brainstem and spinal cord involvement. In initial reports, PER had fatal outcome. Later, clinical improvement related to corticosteroid therapy has been described in some cases. The objective of this study was to signify a reputed clinical significance of corticosteroid therapy in PER. METHODS: Case report. RESULTS: A 50-year-old man developed progressive syndrome of tonic extensor spasms. Magnetic resonance imaging (MRI) showed areas of signal changes in cervical spinal cord and lower brainstem, whereas cerebrospinal fluid analysis indicated subacute encephalomyelitis. His condition dramatically improved on oral corticosteroid therapy. Clinical improvement was accompanied by normalization of MRI findings. CONCLUSION: For this patient with PER, corticosteroid therapy was a dramatically effective and life-saving treatment, although initiated rather late in the course of the disease.

Age-related pattern of the antiepileptic drug utilization in active epilepsy: a population-based survey.

The aim of this study was to investigate the relationship between antiepileptic drug (AED) utilization and patient age in a population of patients treated by primary health care physicians. Data were collected by using questionnaires completed by family physicians and paediatricians working in primary health care. Only patients with active epilepsy confirmed previously by neurologists or neuropaediatricans were included. One hundred and twenty-three physicians provided the requested data for 966 patients (range 1-92 years). Most frequently prescribed AEDs were barbiturates (BARB) (37%) and carbamazepine (CBZ) (37%). Valproic acid derivates (VPA) were prescribed in 28%, but the rate was higher (51%) in children. By calculating the correlation between age and the prescription of single AEDs across the whole sample, linear correlations were found for BARB (r = 0.94; p < 0.01), VPA (r = -0.93; p < 0.01) and for topiramate (TPM) (r = -0.90; p < 0.01). Since our results showed significant correlations between age and the use of the majority of AEDs, we concluded that the age may be considered a methodological bias in the presentation of data. Therefore we calculated AED utilization as the age-adjusted prevalence rates (per/1000 inhabitants). For the most commonly prescribed AEDs they were: BARB 1.8 (95% CI 1.6-2.0), CBZ 1.9 (95% CI 1.7-2.1), VPA 1.3 (95% CI 1.1-1.5), lamotrigine (LTG) 0.7 (CI 95% 0.6-0.8), TPM 0.6 (CI 95% 0.5-0.7). In conclusion, the age of patients has a significant impact on the prescription patterns not only between children and adults, but at every age. Therefore we suggest that reporting of AED utilization pattern should also include age-standardized prevalence rates of individual AED utilization.

[Pathophysiologic basis of the treatment of neurogenic pain]. / Patofizioloske osnove lijecenja neurogene boli.

According to the International Association for the Study of Pain (IASP) neuropathic pain is "pain initiated or caused by a primary lesion or dysfunction or transitory perturbation in the peripheral or central nervous system". Neuropathic pain is usually classified according to the etiology, location of the lesion, and pain characteristics--individual symptoms and signs, but also according to the possible mechanisms involved. Identifying the underlying pain mechanisms during the diagnosis becomes essential for treatment strategies. The clinical picture of neuropathic pain is similar in many cases, and clinical features include: ongoing spontaneous or evoked pain in an area with sensory loss, positive sensory symptoms such as allodynia and hyperalgesia, wind-up pain following repetitive stimulation, referred pain and abnormal sympathetic activity. The understanding of the mechanisms underlying neuropathic pain has increased over the last decade. The primary pathophysiologic mechanisms that produce pain are: nociceptor sensitization, nerve trunk inflammation, sympathetic nervous system involvement, ectopic neuronal discharges, pathologic synaptic reorganization--neuroplasticity and central sensitization. In most clinical features, there is a complex interaction that involves peripheral and central nervous system rather than a single mechanism. Because numerous mechanisms are implicated, the traditional approach to pain control using single drug therapy may not be most effective, and therapeutic combinations are a better choice. Neuropathic pain is poorly responsive to conventional analgesics. In spite of a variety of drug classes used to treat neuropathic pain including antidepressants, anticonvulsants, antiarrhythmics, opioids, local anesthetic blockers, neuropathic pain remains difficult to treat. The possibility to select specific drugs and treatments for the individual patient lies in elucidating the relationships between clinical neuropathic states and underlying pathophysiologic changes. Progress in defining the mechanisms involved in neuropathic pain, based on further clinical studies and fundamental investigations, will improve therapeutic management of neuropathic pain.

Electrophysiological findings in early Guillain-Barré syndrome.

The aim of the study was to identify the most common electrophysiological abnormalities in early Guillain-Barré syndrome (GBS). Neurophysiological data on 51 GBS patients assessed within 12 days of symptom onset were reviewed. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) was present in 46 of 51 GBS patients. The following abnormalities were observed in our AIDP patients: absent H reflex in 90.7%, conduction block in the Erb-to-axilla segment in 78.6%, motor conduction velocity suggestive of demyelination in the Erb-to-axilla segment in 45.2%, prolonged F wave latency in 65.2%-73.8% of patients but only 20.0%-37.0% with prolonged F wave latency suggestive of demyelination, and reduced or absent sensory nerve action potential in 62% of patients. Abnormal values of terminal latencies, and motor and sensory conduction velocities in distal nerve segments suggestive of demyelination were recorded in less than 30% of patients. In conclusion, the most sensitive parameter in early GBS patients is conduction block in the most proximal segments of the peripheral nervous system, directly determined in the Erb-to-axilla segment or indirectly as absent H reflex. Motor conduction studies in the Erb-to-axilla segment are very informative in early GBS patients.

Social and medical care of preschool children with epilepsy in Croatia: population-based survey.

BACKGROUND: Early detection of mental retardation and other epilepsy-associated impairments is essential for successful medical and social care of children with epilepsy; the corresponding information for children in Croatia has not yet been known. AIMS OF THE STUDY: To obtain the basic information of epilepsy-associated disability in preschool children, and fundamentals of their medical and social care. METHODS: Data about mental retardation and other associated impairments (motor, speech, seeing, hearing), antiepileptic drug therapy and diurnal residence were collected by means of questionnaires completed by physicians working in primary health care (PHPs). Only children (0-7 years) with active epilepsy confirmed previously by neuropaediatricians were included. RESULTS: A total of 37 PHPs provided the required data for 116 children. One or more impairments were found in 56% children; most frequent were motor impairments (47%), speech impairments (42%) and mental retardation (40%). The regular kindergarten attendance rate of children without impairment (33%) was not different from the children without epilepsy, but high proportion (76%) of children with impairment stayed with their families during weekdays. In this subgroup monotherapy was more rarely used (64% vs. 90% in children without impairment (p<0.01)). Valproate was predominantly used (56%) in children with and without impairment; lamotrigine was more frequently used in the former subgroup (p<0.01). CONCLUSIONS: Existence of associated impairments has significant impact on medical and social care in preschool children with epilepsy. These children need an early diagnosis and consecutive multidisciplinary care of their intellectual and body impairments, as well as problems in social development.