Alla A. Vein

Clinical aspects of multifocal or generalized tonic dystonia in reflex sympathetic dystrophy

The authors describe 10 patients with reflex sympathetic dystrophy that progressed to a multifocal or generalized tonic dystonia. The neuropsychologic profile was similar to that of other patients with chronic pain, irrespective of its cause. The distribution pattern of dystonia, the stretch reflex abnormalities, and the worsening of dystonia after tactile and auditory stimuli suggest impairment of interneuronal circuits at the brainstem or spinal level. Antibody titers for glutamic acid decarboxylase, tetanus, and Sjögren antigens were all normal.

EEG markers of future cognitive performance in the elderly.

This exploratory follow-up study investigated whether EEG parameters can predict future cognitive performance. Forty elderly subjects, ranging from cognitively unimpaired to those with Alzheimer disease underwent EEG registration at baseline and neuropsychological examination at both baseline and follow-up. We assessed relations between EEG measures and future cognitive performance (i.e., global cognition, memory, language, and executive functioning) controlling for age, follow-up time, and baseline cognitive performance. Regression models were constructed to predict performance on the Cambridge Cognitive Examination, a widely used tool within dementia screenings. Baseline EEG measures, i.e., increased theta activity (4–8 Hz) during eyes closed and less alpha reactivity (8–13 Hz) during eyes open and memory activation, indicated lower global cognitive, language (trend significant), and executive performance at follow-up. A regression model combining baseline cognitive and EEG measures provided the best prediction of future Cambridge Cognitive Examination performance (93%). EEG and cognitive measures alone predicted, respectively, 43% and 92% of variance. EEG and cognitive measures combined provided the best prediction of future cognitive performance. Although the “cognition only” model showed similar predictive power, the EEG provided significant additional value. The added value of EEG registration in the diagnostic work-up of dementia should be further assessed in larger samples.

Memory activation reveals abnormal EEG in preclinical Huntington's disease.

The EEG is potentially useful as a marker of early Huntingtons disease (HD). In dementia, the EEG during a memory activation challenge showed abnormalities where the resting EEG did not. We investigated whether memory activation also reveals EEG abnormalities in preclinical HD. Sixteen mutation carriers for HD and 13 nonmutation carriers underwent neurological, neuropsychological, MRI and EEG investigations. The EEG was registered during a rest condition, i.e. eyes closed, and a working memory task. In each condition we determined absolute power in the theta (4–8 Hz) and alpha (8–13 Hz) bands and subsequently calculated relative alpha power. The EEG during eyes closed did not differ between groups. The EEG during memory activation showed less relative alpha power in mutation carriers as compared to nonmutation carriers, even though memory performance was similar [F (1,27) = 10.87; P = 0.003]. Absolute powers also showed less alpha power [F (1,27) = 7.02; P = 0.013] but similar theta power. No correlations were found between absolute and relative alpha power on the one hand and neuropsychological scores, motor scores or number of CAG repeats on the other. In conclusion, memory activation reveals functional brain changes in Huntingtons disease before clinical signs become overt.

Single-fiber EMG in familial hemiplegic migraine

Twelve familial hemiplegic migraine (FHM) patients (6 with the I1811L mutation in CACNA1A, 3 with M731T mutation in ATP1A2, and 3 without known mutations) and 10 control subjects underwent single-fiber EMG. Mean jitter did not differ significantly between patients and control subjects or among patients. No blocking was found. The results suggest that neuromuscular function is normal in FHM.

Space Headache: A New Secondary Headache

Headache is a common, but rarely voiced, complaint during space flights, usually attributed to space motion sickness (SMS). We used a specifically designed questionnaire based on the criteria of the International Classification of Headache Disorders, 2nd edn (ICHD-II). Of the 16 male and one female astronauts who participated in the survey, 12 (71%) reported having experienced at least one headache episode while in space, whereas they had not suffered from headache when on earth. There were in total 21 space headache episodes, of moderate to severe intensity in 71%. In two astronauts (12%) the headache and associated symptoms would match the ICHD-II criteria for migraine and in three (18%) astronauts for tension-type headache; in 12 (70%) astronauts the headache was non-specific. The vast majority of headache episodes (76%) were not associated with symptoms of SMS. We conclude that space flights may trigger headaches without other SMS symptoms in otherwise ‘super-healthy’ male subjects. We propose to classify space headache as a separate entity among the secondary headaches attributed to disorders of homeostasis.

Science and Fate: Lina Stern (1878–1968), A Neurophysiologist and Biochemist

Lina Stern (1878–1968), a neurophysiologist and biochemist, was born in Russia. She studied at the University of Geneva, Switzerland, where, after graduating, she conducted original research in physiology and biochemistry. In 1918, Stern was the first woman to be awarded a professional title at the University of Geneva and headed the department of Physiological Chemistry. She is deservedly considered to be one of the first scientists to entertain the concept of a blood-brain barrier. In 1929, Stern founded the Institute of Physiology in Moscow, of which she was director until 1948, when it was discontinued. Under her leadership, multidisciplinary groups of colleagues worked on the problems of the blood-brain and tissue-brain barriers and homeostasis of the brain. In 1939, Stern was elected full member of the Academy of Sciences and became its first female member ever. Most scientists manage to conduct their research by adjusting to the political and social situations surrounding them. Lina Stern did not follow this path. This small woman of complete devotion to science took the drastic decisions that altered her life. Though destiny was not kind to her, Lina Stern did not compromise. Despite a threat of execution, prolonged imprisonment, and exile she was never broken as a scientist and always maintained her dignity.

Kozhevnikov epilepsy: the disease and its eponym.

Eponyms continue to have their place in medicine but there are pitfalls associated with their use. “Priorities” may be debatable, misattributions are not uncommon, and knowledge of the original papers is often insufficient. A. Ya. Kozhevnikov (1836–1902) is considered to be the founder of the Russian neurology, best known in the West for his work on epilepsia partialis continua (EPC), published in 1894. Kozhevnikov considered various natures for this disorder but thought chronic infectious etiology to be the most probable. Shortly the eponym Kozhevnikov epilepsy was coined and used in clinical practice and writing. Thirty‐five years after Kozhevnikov’s death, in 1937, a new form of viral encephalitis, Russian spring‐summer tick‐borne encephalitis (RTBE), was discovered, which was strongly associated with EPC and at times incorrectly attributed to Kozhevnikov by Russian (Soviet) and West‐European scientists, although he never specifically identified or even could have recognized this disease entity. When, in 1958, Canadian scientists published about persisting focal epilepsy due to chronic focal encephalitis in children, a new disease was proclaimed: Rasmussen syndrome or Rasmussen chronic encephalitis. The only reference to Kozhevnikov in the Canadian papers was the incorrect suggestion that Kozhevnikov himself described EPC in RTBE. This historical error resulted in continuing misquotations of Kozhevnikov in the current literature and controversies concerning the place of Kozhevnikov epilepsy in the Classification Scheme of the International League Against Epilepsy (ILAE). The history of Kozhevnikov epilepsy thereby offers an illustrative example of the successive misunderstandings, errors, and controversies that arise due to insufficient knowledge or understanding of the original publications, questionable post hoc interpretations of earlier findings, misquoting of secondary papers, or a combination of all these.

Cardiovascular Autonomic Function Tests in Cluster Headache

While facial autonomic signs are prominent during cluster headache (CH) attacks, cardiovascular autonomic changes have been described in few CH patients. Cardiovascular autonomic function tests (AFT) can be used to assess general autonomic function in CH patients in different stages of the disease. We aimed to assess whether general autonomic function is changed in CH patients during a cluster period. AFT was performed both during a cluster period, but outside an actual attack, and outside a cluster period in 18 patients. Heart rate variability was studied at rest, during deep breathing, after standing up and during a Valsalva manoeuvre. Blood pressure (BP) changes were recorded at rest, during standing up and during sustained handgrip. Measurements during and outside the cluster period were compared using the paired t-test. AFT measurements revealed no significant differences between the two measurements, except for diastolic BP in rest, which was higher during the cluster period [80.3 (SD 12.2) vs. 74.8 (SD 9.0), P = 0.04]. Autonomic dysfunction during a cluster period, but outside an attack, does not include systemic cardiovascular control.

The Moscow Clinic for Nervous Diseases - Walking Along the Portraits

The history of the Clinic for Nervous Diseases of the Moscow University reflects in its entirety the history of the Moscow neurological school itself. A.Ya. Kozhevnikov, the founder of the clinic, was the first professor of neurology in Russia. The clinic opened its doors in 1890 and became the first specialized medical facility in Europe devoted to the treatment of neurological disorders. Kozhevnikov brought up a number of talented followers, who later worked all over Russia, and some of them became in charge of the Clinic for Nervous Diseases. This paper looks into contributions Kozhevnikov, his pupils V.K. Rot, V.A. Muratov, G.I. Rossolimo, E.K. Sepp, and some others who were responsible for the development of the neurological science.

Reproducibility and feasibility of neurophysiological assessment of the sensory trigeminal system for future application to paroxysmal headaches.

As the distribution of pain in primary headaches suggests involvement of the trigeminal sensory pathways, trigeminal somatosensory evoked potentials (TSEP) and blink reflexes (BR) may provide important information about their functional integrity. Functional differences between symptomatic and non-symptomatic sides and between measurements during and outside attacks may be particularly informative. These tests should therefore be reproducible and should require a suitable number of patients for future studies in patients with primary, paroxysmal headaches. We performed TSEP and BR twice in 22 healthy volunteers, in order to calculate sample sizes based on reproducibility data. This is, to our knowledge, the first study investigating the reproducibility of TSEP and BR measurements. Latencies of TSEP and BR are appropriate for future studies, as their reproducibility allows practical sample sizes (less than 25 subjects). Duration, amplitude and area parameters of the BR responses were less appropriate for longitudinal studies.