Vol 6, No 4 (2012)

Original articles

MRI morphometry in primary focal dystonia

Timerbaeva S.L., Konovalov R.N., Illarioshkin S.N.

Abstract

Primary blepharospasm (BS) is one of most frequent forms of focal dystonia characterized by excessive involuntary eye closure. Pathophysiology of primary BS remains obscure. The purpose of this study: to determine changes of the cerebral gray matter volume that may be pathogenically important in primary BS. We examined 23 right-handed patients with primary BS (6 males and 17 females) and 16 healthy age- and sex-matched individuals who underwent voxel-based morphometry (VOM) – a method of assessment of fine regional quantitative changes of gray matter volume. In 15 patients VOM studies were performed twice, before and one month after injections of botulinum toxin type A (BTA). Compared to controls, BS patients were characterized by the decrease in gray matter volume in the head of the right caudate nucleus, anterior and posterior lobes of the right cerebellar hemisphere, and the right fusiform gurus. Multiplefactor analysis did not show relationships between gray matter changes and age of patients, age at the debut of BS, and duration of the disease or BTA treatment. On repeat examination after local BTA injections in the circular orbicular muscles (aimed at reducing dystonic spasms in BS patients), the increase in gray matter volume in both fusiform gyri, the opercular parts of the left Rolandic gyrus, the right middle and the left inferior temporal gyri, the left inferior frontal gyrus, and the left cingular gyrus was observed. The obtained data demonstrate the presence of structural brain changes in primary BS, confirming a significant role of the striatum and the cerebellum in pathophysiology of this form of focal dystonia.

 
Annals of Clinical and Experimental Neurology. 2012;6(4):
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Camptocormia in Parkinson’s disease: clinical and pathogenetic features

Gamaleya A.A., Fedorova N.V., Tomskiy A.A., Shabalov V.A., Bril E.V., Belgusheva M.E., Orehova O.A.

Abstract

Cаmptocormia (CC), an abnormal posture with involuntary forward flexion of the trunk, occurs in many neurological disorders, most frequent among which is Parkinson’s disease (PD). Axial segmental dystonia and/or focal myopathy of paravertebral muscles are considered to be possible causes of CC in PD, but exact mechanisms remain unclear. CC is a highly disabling condition leading to dependency in daily living and gait problems. Treatment of CC in PD include adjustment of antiparkinsonian medication, injections of botulinum toxin and orthopedical interventions; however, the results are mainly disappointing. Some authors report the efficacy of deep brain stimulation (DBS) for CC in PD. We describe four patients with PD and CC who underwent implantation of DBS systems bilaterally into subthalamic nucleus (STN) or globus pallidus internus (GPI). In two cases of DBS STN, marked alleviation of bradykinesia, rigidity, and motor fluctuations were noticed. In only one patient with L-dopa responsive CC, we observed significant reduction of trunk flexion, and in the other patient the favorable effect was limited due to the fixed skeletal deformity. In two cases of DBS GPI, parkinsonian state improved moderately without changes in CC severity. Thus, the benefit of DBS STN or GPI for CC associated with PD remains indefinite. Our observations confirm an important predictive role in operation outcome of such factors as the sensitivity of CC to L-dopa and the presence of degenerative spinal disease. Better understanding of CC pathogenesis and studies on larger cohorts of patients are necessary to elaborate an appropriate treatment algorithm.

 
Annals of Clinical and Experimental Neurology. 2012;6(4):
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Guillain-Barre syndrome: recovery patterns in demyelinating and axonal forms

Grishinа D.A., Suponeva N.A., Piradov M.A.

Abstract

We carried out an examination of 74 patients who have had, from 3 months to 36 years ago, Guillain–Barre syndrome (GBS) with severe impairmenet of motor functions. The average follow-up period was 2.3 years. Fifty-eight subjects had acute inflammatory demyelinating polyneuropathy (AIDP), and the remaining 16 patients had axonal forms. In the structure of residual neurologic deficits of AIDP patients at all stages of early recovery period (follow-up period <1 year) and in late (>1 year) period sensory disorders and pain syndrome prevailed. The majority of AIDP patients (90%) began to walk with support after 1 month of onset, and occasionally (3%) six months later.
Patients with axonal forms could stand only after 6 months (88%) and later (12% patients – up to 12 months). After 1 year of onset, all AIDP patients walked unassisted, and most of them return to their job. Over the equal follow-up period every third patient with an axonal form remained dependent in their daily living activities. This confirms that GBS is a disease with a favorable prognosis: even in severe cases most patients resumed walking after half a year, regardless of the disease form. Axonal forms show lower recovery rate and greater severity of residual symptoms compared to AIDP, which affects quality of life in respective patients.
Annals of Clinical and Experimental Neurology. 2012;6(4):
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Hyperglycemia and free radical imbalance as prognostic factors in acute stroke

Rumyantceva S.A., Silina E.V., Orlova A.S., Orlov V.A., Bolevich S.B.

Abstract

We evaluated 395 patients with acute stroke: diabetes mellitus was diagnosed in 19.5%, hyperglycemia was observed in 41.8% patients on day 1 and in 48.9% on day 2 after admission. Hyperglycemia and diabetes were associated with more severe free radical imbalance and worsened the outcome in stroke patients, decreasing
rehabilitation potential. The increase of malonic dialdehyde (MDA), decrease of antiperoxide plasma activity (APA), hyperglycemia and high leucocyte count are shown to be prognostic factors of adverse outcome (death, severe disability), which should be taken into account during antioxidant therapy. The highest mortality rate, which showed correlation with the size of stroke, was observed in patients with hyperglycemia persisting more than 3 days after stroke onset, and it was 1.8-fold higher than in patients with normoglycemia. Indications for target antioxidant therapy are: blood glucose > 6.6 mmol/l, leucocyte count >9 700, APC level <3, and MDA level >4 μmol/l
Annals of Clinical and Experimental Neurology. 2012;6(4):
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A platform for studies of Huntington’s disease on the basis of induced pluripotent stem cells

Nekrasov E.D., Lebedeva O.S., Vasina E.M., Bogomazova A.N., Chestkov I.V., Kiselev S.L., Lagarkova M.A., Klyushnikov S.A., Illarioshkin S.N., Grivennikov I.A.

Abstract

Huntington’s disease (HD) is one of the most severe hereditary neurodegenerative disorders caused by CAG repeats expansion in the HTT gene. A recently elaborated technology of genetic reprogramming allows obtaining induced pluripotent stem (iPS) cells from fibroblasts and other differentiated somatic cells. These iPS cells can grow in culture and differentiate in any cell types, including neurons, necessary for studies of molecular mechanisms of HD and other neurodegenerative diseases. We obtained, with the use of lentivirus transfection, iPS cells from primary fibroblasts biopsied from three female patients with HD (42–46 copies of the CAG repeats in the mutant allele). The efficiency of reprogramming was approximately 0.2%. The embryoid bodies were obtained from some clones of iPS cells, and derivatives of all the three embryo layers were shown to be formed as a result of spontaneous iPC cells differentiation. At present, our cell lines represent a unique platform for studies of HD. It may be used for establishing an effective system aimed at discoveries of molecular mechanisms undelaying HD and high-throughput search for novel neuroprotective drugs.

 
Annals of Clinical and Experimental Neurology. 2012;6(4):
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Mirror visual feedback – mirror therapy in neurorehabilitation

Nazarova M.A., Piradov M.A., Chernikova L.A.

Abstract

Mirror is a well-established tool in the movement rehabilitation, providing real-time visual feedback to assist in the retraining. However, mirror visual feedback may be used also in therapy in its own right. The “mirror therapy” (MT) is a method based on creating artificial visual feedback. MT was proposed for treating pain syndrome, such as phantom pain and complex regional pain syndrome of type 1 and 2, as well as for motor rehabilitation in stroke patients with hemiparesis and in patients after arm surgery. The main principle of MT is thought to be restoration of damaged feedback from ill/amputated limb. One of possible mechanisms of MT is activation of “mirror neurons”, acting both during execution and observation of action. Clear advantages of MT are its simplicity, availability and, most importantly, its patient-directed character.

 
Annals of Clinical and Experimental Neurology. 2012;6(4):
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Blood-brain barrier models in vitro: current situation and prospects

Morgun A.V., Kuvacheva N.V., Kоmleva Y.K., Pozhilenkova E.A., Kutishcheva I.A., Gagarina E.S., Taranushenko T.E., Ozerskaya A.V., Okuneva O.S., Salmina A.B.

Abstract

Current experimental models of the blood-brain barrier (BBB) in vitro used for studying mechanisms of permeability and intercellular communication are discussed in this review. At present, monolayer, multilayer and computer models are in use for the above-mentioned purposes. Primary isolated cells that make up the models in vitro may have brain and non-brain origin. In addition, transplantable cell lines and co-cultured cells could be used for modeling BBB in vitro.

 
Annals of Clinical and Experimental Neurology. 2012;6(4):
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A course of speech restoration in amnestic aphasia with literal agnosia and alexia

Berdnikovich E.S., Shakhparonova N.V., Kаdykov A.S., Kashina E.M., Krotenkova M.V., Belopasova A.V.

Abstract

Speech problems are the second most significant defect (after movement disorders) in patients suffering from stroke. According to stroke registry data from Research Center of Neurology RAMS, in the acute phase of stroke aphasia occurs in 36.9% of cases, and dysarthria in 13%. In this paper, a clinical example of amnestic aphasia syndrome with literal agnosia and alexia is presented, and foreground methods of correction directed to restoration of abnormal functions in this relatively rare form of speech disorders are discussed. Following medication therapy and daily logopedic training with the use of an adequate personal program, the patient clearly succeeded in alleviating  the literal agnosia and forming the letter image.

 
Annals of Clinical and Experimental Neurology. 2012;6(4):
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