[New evidence of the prion nature of amyotrophic leukospongiosis].

We described 2 cases and presented new experimental data on the study of the etiopathogenesis of amyotrophic leukospongiosis (AL) using Western blot, electronic and atomic force microscopy. AL is characterized by the gradual and steady progression of flaccid paralysis of extremities and muscles of the trunk without the expressed atrophy with an inevitable fatal outcome resulting in breathing disorders of spinal type, degenerative changes of the central nervous system and, first of all, spongiosis of the white matter of the brain. Several lines of evidence suggest that the accumulation of pathological prion protein (PrPAL) in the human brain is a critical event for the development of the disease. The new findings add knowledge of the AL etiopathogenesis and support the evidence that the disease is caused by prion infection and refers to the group of transmissible spongiform encephalopathies.

[Surveillance of acute flaccid paralysis in Belarus].

The ten-years experience of acute flaccid paralysis (AFP) surveillance in Belarus has been summarized. Among 456 AFP cases reported from 1996 to 2005, 11 were classified as vaccine-associated paralytic poliomyelitis (VAPP), 445--as non-polio AFP. The risk of VAPP for the period 1996-2001 was 1 case per 745,000 used doses of oral poliovaccine (OPV). For the recipients of OPV the risk was 1 case per 911,700 doses and for the first-dose recipients--1 case per 96,000 doses. The high incidence of VAPP was a reason for implementation of sequential polio vaccination schedule in 2000. Guillain-Barre syndrome dominated among non-polio AFP (39.3% of cases); more rare were traumatic neuritis (27.9% of cases), transient monoparalysis (12.1%), myelitis (7.6%). Non-polio AFP differed from VAPP by following epidemiological and virological characteristics: predominance of previously repeatedly vaccinated against poliomyelitis; development of paralysis in long-term period after vaccination; isolation of non-polio viruses belonged to three serotypes of Coxsackie B viruses (B1, B4, B6) and six serotypes of Echo viruses (6, 7, 11, 14, 24, 25) in 8.1% of cases; absence of typical for polio residual paralyses in patients who excreted vaccine polioviruses.

[Epidemiology and clinical aspects of central European (western) tick-borne encephalitis in the Brest region of the Republic of Belarus]. / Otdel'nye voprosy épidemiologii, kliniki tsentral'no-evropeiskogo (zapadnogo) kleshchevogo éntsefalita v Brestskoi oblasti Respubliki Belarus'.

The paper presents the results of epidemiological and clinical studies of the peaks of tick-borne encephalitis (TBE) in the Belovezhskaya Pushcha region in 1993-2001. The findings confirm the concept worked out by Byelorussian researchers on nosological differences in the western and eastern subtypes of TBE.

Vaccine-associated paralytic poliomyelitis and other diseases with acute flaccid paralysis syndrome in Belarus.

According to the WHO global polio eradication initiative acute flaccid paralysis (AFP) surveillance has been conducted in Belarus since 1996. For the period 1996-2002, 295AFP cases were reported. The main indices ofAFP surveillance in Belarus met the WHO criteria. A11 AFP cases, with the exception of one, were virologically examined. Polioviruses (PV) were isolated from 28 (9.5%) of them. Results of intratypic differentiation (a neutralization test with type-specific monoclonal antibodies and a restriction fragment length polymorphism assay) proved vaccine origin of all isolated PV. According to the final classification, 11 AFP cases were classified as vaccine-associated paralytic poliomyelitis (VAPP). Nine VAPP cases were recipient [six of them developed after the first, two--after the third and one--after the fourth oral poliovirus vaccine (OPV) dose] and two cases in non-vaccinated children were classified as contact VAPP cases. PV of all three serotypes were isolated with an equal frequency from the recipient cases and only PV2--from contact ones. Immunological investigations of children with VAPP showed that the majority of them had disorders in B-cell immunity. A risk of one VAPP case per 96,000 first OPV doses and per 745,000 distributed ones was estimated. The other 284 AFP cases were classified as AFP of non-polio etiology (non-polio AFP). Among them Guillain-Barré syndrome (118 cases, 41.5% of all non-polio AFP cases), traumatic neuritis (63 cases, 22.2%), transient monoparesis of limb (35 cases, 12.3%), myelitis (26 cases, 9.2%) were registered most frequently. Vaccine PV were isolated from 19 (6.7%) children with non-polio AFP, 28 (9.9%) children excreted non-polio enteric viruses. In contrast to VAPP, other AFP with PV isolation had no clinical picture typical of poliomyelitis, and had no any residual paralysis 60 days after the onset of paralysis. PV isolation from them seemed to be not related to the etiology of the disease, but was a mere coincidence of paralysis with the recent vaccination. Results of AFP surveillance supported the previous data on the absence of classical poliomyelitis cases caused by wild PV in Belarus for more than 35 years.

[The clinical characteristics of progressive focal epilepsy with a herpetic etiology]. / Klinicheskie osobennosti progressiruiushchei fokal'noi épilepsiei gerpeticheskoi étiologii.

Cases are described of chronic zoster encephalitis presenting with the leading progressive focal epilepsy syndrome. The clinical presentation of the trouble can include both general cerebral signs and those of the focal affection of the central nervous system only. The progressive course and duration of remission suggest to us a persisting infection manifesting against the background of immune deficiency.

Progressive muscular atrophy variant of familial amyotrophic lateral sclerosis (PMA/ALS).

Twelve cases of adult-onset progressive muscular atrophy variant of amyotrophic lateral sclerosis (PMA/ALS) were studied in a small rural population of 1500 in the Republic of Belarus (former Soviet Union). The patients were members of three apparently related kindreds, each showing autosomal dominant pattern of disease inheritance. The average age at clinical onset ranged from 26 to 57 years (mean, 40 years). Each patient suffered from skeletal muscle weakness and wasting, starting in the limbs and spreading to the trunk and neck, with very limited bulbar and no upper motor neuron involvement. Death from respiratory failure occurred from 13 to 48 months (mean, 28 months) after first symptoms. Dramatically decreased number of spinal motor neurons was the most characteristic neuropathologic feature in two autopsied cases. Most of the remaining degenerating neurons contained intracytoplasmic hyaline inclusion bodies. A D101N mutation in exon 4 of the SOD1 gene was identified in a PMA/ALS patient and in one of her three unaffected children. Our data support the view that some subtypes of familial ALS associated with SOD1 mutations may present as PMA. Diagnostic criteria of ALS should be accordingly modified to include the PMA variant of familial ALS.

Tick-borne encephalitis (TBE) epidemiology in the Brest Province of the Republic of Belarus.

Prevalence of tick-borne encephalitis (TBE) and number of TBE infected ticks was analysed in the Brest Province since 1955. They revealed increasing tendency, which reached the highest values in the last years. TBE incidence was the highest in June and it was observed predominantly in people between 30 and 39 of age. The studies of TBE incidence rate regularities have disclosed its cyclical nature.

[Morphologic diagnosis of a seronegative case of HIV-infection]. / Morfologicheskaia diagnostika seronegativnogo nabliudeniia.

Morphological diagnosis is described of a seronegative case of HIV-infection resulting in AIDS-dementia and development of a generalized herpes and aspergillosis. The presence of HIV-antigens in the central nervous system and the spleen histologic sections confirmed morphological diagnosis.

[The characteristics of progressive dementias in patients with herpetic encephalitis]. / Osobennosti progressiruiushchikh dementsii u bol'nykh gerpeticheskim éntsefalitom.

9 lethal cases of progressive dementias were analysed, both psychical and neurological disturbances characteristics on different stages of herpetic dementias were presented, a number of clinical morphological criteria of diagnosis ascertaining was described. It was determined that progressive dementias turned out to be one of the clinical forms of chronic CNS herpetic infection which was conditioned by prolonged persistence and activation of herpes simplex virus in brain tissue.

[Chronic herpetic encephalitis with a progressive epileptic syndrome]. / Khronicheskii gerpeticheskii éntsefalit s progressiruiushchim épileticheskim sindromom.

Basing on the evidence provided by clinicomorphological and immunobiological studies, three, patients with progressive epileptic seizures were found to have chronic herpetic inflammation in the brain. The seizures took serial pattern with development of status epilepticus.

[A variant of amyotrophic lateral sclerosis]. / Variant amiotroficheskogo bokovogo skleroza.

The results of the clinico-morphological studies carried out in 30 patients with the anterior-horn variety of amyotrophic lateral sclerosis (ALS) (CNS morphology was investigated in 6 cases) demonstrate this variety to be characterized by early fading of the tendinous periosteal reflexes, rapid development of amyotrophies, weak intensity of pyramid system lesions. This is supported by morphological studies of the CNS and allows advancing an opinion in favour of the priority of motoneuronal injury.

[Clinical forms of acute herpetic infections of the central nervous system in adults]. / Klinicheskie formy ostrykh gerpeticheskikh porazhenii tsentral'noi nervnoi sistemy u vzroslykh.

The authors describe the course of herpetic encephalitis in 52 patients aged 16 to 64 years. Five types of the initial manifestations of herpetic infection of the CNS were revealed. In 44.3% of cases the disease started from the general cerebral symptomatology and consciousness disturbance; in 13.6% it started in a brain stroke-like manner followed by the development of the comatose status; in 13.6% of cases from memory disorder and unmotivated actions; in the same percentage of cases, the disease onset was marked by the dominance of dizziness, diplopia, ataxia and central hemiplegia ; in 15.9% the disease started from pains in the stomach, loin and lower limbs. Hemispheric and pseudotumorous stem encephalitides (48.1 and 13.6% respectively) were predominant; in 25% meningoencephalitides and in the remainder, encephalomyelitis running their course in the form of disseminated encephalomyelitis (5.7%), focal myelitis (5.7%) or opticomyelitis (1.9%). The data presented attest to the pleomorphism of the clinical picture of herpetic lesions of the CNS.

[The clinico-pathogenetic characteristics of Kaposi's sarcoma]. / Kliniko-patogeneticheskie osobennosti sarkomy Kaposhi.

The paper is concerned with clinicomorphological characteristics of endemic, immune and epidemic (AIDS-associated) forms of Kaposi's sarcoma (KS). Recent experimental and clinical evidence suggests pathogenic contribution to KS of virus-induced depression of sex-linked genes and immunosuppression. Combined action of these factors should be regarded when specifying current presentation of KS.

[The efficacy of lincomycin in tick-borne encephalitis]. / Ob éffektivnosti linkomitsina pri kleshevom éntsefalite.

Lincomycin was found to inhibit tick-borne encephalitis (TBE) virus. To test antiviral potential of this drug, a clinical trial was initiated entering TBE patients from known focuses of the disease (Novosibirsk, Kemerovo, Perm and Irkutsk Provinces). The drug was given to 23 patients with meningeal and meningoencephalitic TBE. A control group of 22 matched subjects received specific immunoglobulin. Resultant efficacy of lincomycin appeared not inferior to that of anti-TBE immunoglobulin. Lincomycin can be successfully introduced in the treatment of meningeal and meningoencephalitic TBE.