[The quality of medical care of patients with amyotrophic lateral sclerosis in the Russian Federation]. / Kachestvo okazaniia pomoshchi bol'nym bokovym amiotroficheskim sklerozom v Rossiiskoi Federatsii.

The quality of medical care for patients with amyotrophic lateral sclerosis (ALS) in the Russian Federation is analyzed taking into account the cooperation of patients. Two hundred and fifty-one patients with ALS were observed. Ninety-nine patients, including 16 who were followed up, were examined. Other patients (n=152) were consulted indirectly including 28 followed up. It has been shown that 79.8% of the patients are characterized by the low level of cooperation with neurologists regardless of the quality of medical care; 8.3% of patients are managed in facilities (or by physicians), which don't use the International standard of ALS management. Only 11.9% of patients receive medical care in accordance with this standard and in these cases, the high level of cooperation of the patients with medical services was recorded. A dual system that combines the services provided by medical insurance, private treatment facilities, charity organizations, structures of medical/social care with different levels of availability and methods of consumer-provider interactions is the most effective.

[The quality of medical care and cooperation of patients with amyotrophic lateral sclerosis in the Russian Federation]. / Kachestvo okazaniia pomoshchi bol'nym bokovym amiotroficheskim sklerozom v Rossiiskoi Federatsii.

The quality of medical care for patients with amyotrophic lateral sclerosis (ALS) in the Russian Federation is analyzed taking into account the cooperation of patients. Two hundred and fifty-one patients with ALS were observed. Ninety-nine patients, including 16 who were followed up, were examined. Other patients (n=152) were consulted indirectly including 28 followed up. It has been shown that 79,8% of the patients are characterized by the low level of cooperation with neurologists regardless of the quality of medical care; 8.3% of patients are managed in facilities (or by physicians), which don't use the International standard of ALS management. Only 11.9% of patients receive medical care in accordance with this standard and in these cases, the high level of cooperation of the patients with medical services was recorded. A dual system that combines the services provided by medical insurance, private treatment facilities, charity organizations, structures of medical/social care with different levels of availability and methods of consumer-provider interactions is the most effective.

[The differential diagnosis of amyotrophic lateral sclerosis and subacute herpes virus myelitis]. / Differentsial'naya diagnostika podostrogo gerpeticheskogo mielita s bokovym amiotroficheskim sklerozom.

Differential diagnosis of incurable and potentially curable neurological diseases is an urgent problem of modern neurology. The authors present a case report of subacute herpes virus myelitis, a rare complication of herpes infection by Varicella-Zoster virus. The differential diagnosis with amyotrophic lateral sclerosis is described.

[Mental disorders in patients with lateral amyotrophic sclerosis and their family members].

OBJECTIVE: Mental disorders in patients with lateral amyotrophic sclerosis (AMS) and their family members were studied. MATERIAL AND METHODS: Authors examined 118 AMS patients and 97 their family members. Mental status was assessed using Hamilton scale and the frontotemporal dementia scale. RESULTS: Mental disorders were identified in 101 (85%) of AMS patients and in 51 (52.5%) of their family members. The patients had situational disorders, with a slight prevalence of situational depression. Sometimes AMS was comorbid to endogenous mental diseases. Cognitive impairment that reached the diagnostic threshold of dementia was observed in 4.2% of the patients. The family members had only situational disorders with distinct predominance (28%) of anxiety states. Common intoxications (tobacco smoking, drug addiction etc) were noted in 49 (41.5%) of AMS patients and 80 (82.4%) of their family members. Anxiety and other mental disorders often resulted in the noncompliance with physician recommendation and refusal of treatment. CONCLUSION: Permanent patronage of AMC families by specialists of neurologic and psychotherapeutic services and assistance based on the mental state and general medical situation are recommended.

[Cognitive impairment in amyotrophic lateral sclerosis]. / Kognitivnye narusheniya pri bokovom amiotroficheskom skleroze.

OBJECTIVE: To determine cognitive and emotional status in patients with amyotrophic lateral sclerosis (ALS). MATERIAL AND METHODS: One hundred sixteen patients were examined. RESULTS AND CONCLUSION: Frontotemporal cognitive changes (CC) were found in 35.3% of patients (medium log rank on the Frontotemporal dementia rating scale was 1.47 [1.92; 1.26], and 4.2% patients had frank dementia (medium log rank -0.4 [1.07; -3.09]). The average degree by the Montreal cognitive scale in patients with CC was 21.7±4.4. 48,9% patients had mixed (executive and speech) symptoms, 34% had frontal (executive) symptoms, 4.2% had temporal (visual and spatial) deficits, and 4.2% had isolated behavioral symptoms. There was no patients with isolated speech deficits. ALS patients with CC were significantly older than patients without CC (64.5±7.7 and 52.5±12.1 years, р=0,0001). ALS progressed more rapidly in patients with CC (13.5±9.3 and 10.4±9.1 ALSFRS-R scores per month, p=0.033). Patients with lumbar onset of ALS prevailed in the group without CC (17% and 36%, χ2=3.94; р=0.047), and patients with bulbar onset of ALS prevailed in the group with CC at the trend level. A comparison of estimates by different scores of emotional state and quality of life (the Hamilton depression scale, CNSLS, ALSAQ40) allows to conclude that CC had no influence on the emotional state of ALS patients and their total quality of life.

Sporadic ALS associated with the D90A Cu,Zn superoxide dismutase mutation in Russia.

Twenty blood samples from Russian patients (Moscow) with idiopathic motor neurone disease were analysed for mutations in the Cu,Zn superoxide dismutase (Cu,Zn SOD) gene. Two patients (10%) with the amyotrophic lateral sclerosis (ALS) form of the disease were found to have a disease-related mutation. One patient appears to have autosomal recessive adult-onset ALS associated with homozygosity for D90A and presents the characteristic phenotype of very slowly ascending paresis with both lower and upper motor neurone signs. Another patient, heterozygous for D90A, presents ALS with lumbar onset and rapid progression. This is the first report of a Cu,Zn SOD mutation in ALS in Russia.