[Thrombosis of developmental venous anomaly and consecutive venous infarction]. / Thrombose eines venösen Angioms mit sekundärem Stauungsinfarkt.

Developmental venous anomalies (DVA) are congenital aberrations in the cerebral venous system reflecting failure of normal embryogenesis. They are considered the most common intracranial vascular malformation. Although they are generally regarded as benign entities, their clinical significance remains controversial--case reports do exist considering DVA to be the origin of thrombosis, cerebral ischemia, and hemorrhage. We report a 26-year-old patient suffering from thrombosis of a DVA.

[Chronic facial pain due to a brainstem cavernoma]. / Therapieresistenter Gesichtsschmerz durch ein Hirnstammkavernom.

Idiopathic trigeminal neuralgia is almost always associated with pathological nerve/vessel contact. Symptomatic forms of trigeminal neuralgia include cases of multiple sclerosis, infratentorial tumours and postherpetic neuralgia. Vascular malformations causing neuralgia have rarely been reported. We present the case of a 55-year old woman, who suffered from facial pain and ptosis on her left side. Repeated neurological examinations as well as repeated magnetic resonance imaging did not lead to a definite diagnosis or therapy. The pain suddenly stopped three weeks before admission and only a slight left sided facial hypaesthesia persisted. Reevaluating the older MRI we found a small signal alteration of 2 mm in the caudal part of the left trigeminal nucleus. A new MRI showed a subacute haemorrhage into a small brainstem cavernoma, which must have caused the pain and later on the hypaesthesia. Small vascular malformations are a rare cause of neuropathic facial pain.

Functional connectivity between cortical hand motor and language areas during recovery from aphasia.

Previous data indicate that in healthy subjects, there is a connectivity between cortical areas for hand movement and language on the left hemisphere. This link is possibly mediated by the so-called mirror neuron system. The present study investigated the functional relationship between linguistic and hand movement processing in patients who were recovering from post-stroke aphasia. The excitability of the right- and left-hand motor cortex during language production in patients who were recovering from post-stroke aphasia and age-matched controls was investigated. As control, phonation was investigated. Hand motor cortex excitability was assessed with Motor Evoked Potentials which were elicited by Transcranial Magnetic Stimulation (TMS). In patients, reading aloud enhanced the excitability of the right hemispheric hand motor cortex, whereas phonation had no effect on hand motor cortex excitability. In the control group, an increased excitability of the left hemispheric hand motor system was found during reading aloud in accordance with previous data. The present data suggest a functional connectivity between regions mediating hand movements and reading. This may indicate that the right hemisphere participates in language processing as far as involved in single word reading in patients recovering from aphasia. The coactivation between cerebral representations of hand movements and language may be used therapeutically for aphasia rehabilitation.

Playing piano in the mind--an fMRI study on music imagery and performance in pianists.

Reading of musical notes and playing piano is a very complex motor task which requires years of practice. In addition to motor skills, rapid and effective visuomotor transformation as well as processing of the different components of music like pitch, rhythm and musical texture are involved. The aim of the present study was the investigation of the cortical network which mediates music performance compared to music imagery in 12 music academy students playing the right hand part of a Bartok piece using functional magnetic resonance imaging (fMRI). In both conditions, fMRI activations of a bilateral frontoparietal network comprising the premotor areas, the precuneus and the medial part of Brodmann Area 40 were found. During music performance but not during imagery the contralateral primary motor cortex and posterior parietal cortex (PPC) bilaterally was active. This reflects the role of primary motor cortex for motor execution but not imagery and the higher visuomotor integration requirements during music performance compared to simulation. The notion that the same areas are involved in visuomotor transformation/motor planning and music processing emphasizes the multimodal properties of cortical areas involved in music and motor imagery in musicians.

Involuntary stretching during yawning in patients with pyramidal tract lesions: further evidence for the existence of an independent emotional motor system.

A variety of associated movements have been described in patients with pyramidal tract lesions. We report three patients in whom involuntary stretching of an otherwise plegic arm could be observed during yawning. These patients had radiologically verified lesions at different levels of the pyramidal tract. As yawning and stretching are an automatic behavioural pattern in animals, it is likely that stretches during yawning in man are also an automatic motor pattern, usually inhibited in the presence of an intact corticospinal tract. The physiological function of yawning is unclear at present. Yawning might be the somatomotor manifestation of a particular emotional state characterized by boredom and fatigue. Our observation that movements of an otherwise plegic arm occur in patients with pyramidal tract lesions supports therefore the concept of an emotional motor system which has an independent input to motoneurones in the brain stem and the spinal cord.

The electrically elicited R2 blink reflex consists of distinct subcomponents.

In previous studies the later parts of the electrically evoked R2 blink reflex has been found to be more susceptible to the inhibitory effects of repetitive stimulation and prepulses. Consequently, it has been suggested that the R2 reflex may be composed of distinct subcomponents. However, as of yet, no study revealed electromyographical evidence for this hypothesis, probably due to a putative temporal overlap that makes it difficult to clearly separate distinct R2 subcomponents. In the present study we investigated the electrically evoked blink reflex in 26 healthy volunteers by using the paradigm of self-stimulation, which has been found to inhibit the R2 and may thus help to differentiate putative R2 subcomponents. In most of the participants self-stimulation resulted in inhibition of the reflex involving predominantly later sections of R2. In addition, in three subjects it was possible to clearly separate distinct R2 subcomponents. These findings suggest that the electrically elicited R2 blink reflex--like the acoustic blink response--consists of superimposed distinct subcomponents. Based on their differential modulation evoked by habituation, pre-stimulation and self-elicitation it is hypothesized that the early portions of the R2 represent the electrically evoked blink reflex, whereas the later sections belong to the generalized startle reaction in man. However, due to their temporal overlap a differentiation might not be possible in conventional recordings.

Hypertrophic nerve roots in a case of Roussy-Lévy syndrome.

Hypertrophic radiculopathy is a rare feature of neuropathies. Single cases of enlarged nerve roots have been described in hereditary motor sensory neuropathies (HMSN) and chronic inflammatory demyelinating diseases (CIDP). This is the first description of hypertrophied nerve roots in a patient with Roussy-Lévy syndrome. MRI did not show contrast enhancement of the enlarged nerve roots or nodular lesions.

[Neurologic complications in inflammatory bowel diseases]. / Neurologische Komplikationen bei entzündlichen Darmerkrankungen.

Some inflammatory diseases of the gastrointestinal system are associated with neurological symptoms which, in rare cases, may precede the onset of the gastrointestinal manifestation of the disease. Celiac sprue is characterized by an intolerance to the wheat protein gluten. The typical neurological manifestation of celiac sprue is cerebellar ataxia. The ataxia as well as the gastrointestinal symptoms can be treated with a strictly gluten-free diet. The neurological symptoms of Whipple's disease, a disorder caused by gram-positive bacilli, are variable. Typical symptoms of cerebral Whipple's disease include dementia, ophthalmoplegia, movement disorders, and signs of hypothalamic dysfunction. Nowadays, the diagnosis of cerebral Whipple's disease is made by PCR detection of the bacilli in the CSF. Crohn's disease and ulcerative colitis are associated with neurological symptoms to a similar proportion. Various forms of polyneuropathies have been observed. The CNS manifestations of inflammatory bowel diseases include thromboembolic stroke, cerebral venous thrombosis, and cerebral vasculitis.

Activation in primary and secondary motor areas in patients with CNS neoplasms and weakness.

OBJECTIVE: To demonstrate whether cortical activation within different cortical motor regions in neurosurgical patients varies with the degree of paresis induced by mass lesions near the central region. METHODS: A total of 110 patients with brain tumors infiltrating the central region and with varying degrees of paresis were investigated employing fMRI during the performance of hand motor tasks. The percent signal change between rest and activation was calculated for four cortical regions: primary motor cortex (M1), supplementary motor area, premotor area, and superior parietal lobule. RESULTS: Significant decreases in activation with increasing degrees of paresis were found in M1, whereas significant increases in activation were noted in secondary motor areas that were not affected by the tumor. CONCLUSIONS: The signal loss in areas adjacent to tumor tissue may relate either to tumor-induced changes in cerebral hemodynamics or to a direct loss of cortical neurons resulting in a lesser degree of hemodynamic changes after motor activation. The increase in activation within secondary motor areas with increasing degrees of paresis supports the growing evidence of a practice- and lesion-dependent reorganization of the cortical motor system and the ability of the brain to modulate its excitatory output according to external demands.

Repetitive transcranial magnetic stimulation effects on language function depend on the stimulation parameters.

In previous studies it has been shown that picture-naming latencies can be facilitated with both suprathreshold single and repetitive transcranial magnetic stimulation (TMS/rTMS) over Wernicke's area. The aim of this study was to investigate whether low-frequency rTMS (1 Hz) or high-frequency rTMS (20 Hz) at subthreshold intensities is also capable of influencing picture naming. In 16 healthy right-hand male subjects, trains of 1 Hz or 20 Hz were applied over either Wernicke's area, Broca's area, or the primary visual cortex. The subjects had to name 20 black-and-white line drawings, which were shown immediately after rTMS and again 2 minutes later. Naming latency could be facilitated only immediately after Wernicke's area stimulation at a frequency of 20 Hz and at an intensity of 55% of the maximal stimulator output, which was more than the motor threshold. All other stimulation procedures failed to influence naming latencies. These results indicate that language functions can be facilitated in healthy subjects only by high-frequency rTMS with intensities at or above the motor threshold.

Precision grip deficits in cerebellar disorders in man.

OBJECTIVE: To investigate the effect of a variety of cerebellar pathologies on a functional motor task (lifting an object in a precision grip). METHODS: The study involved 8 patients with unilateral damage in the region of the posterior inferior cerebellar artery (PICA), 6 with damage in the region of the superior cerebellar artery (SUPCA), 12 patients with familiar or idiopathic cortical cerebellar degeneration, and 45 age-matched normal subjects. Subjects lifted an object of unpredictable load (internally guided task) or responded to a sudden load increase while holding the object steadily (externally guided task). RESULTS: Damage to the dentate nucleus (SUPCA) or its afferent input (cerebellar atrophy) resulted in disruption of the close coordination normally seen between proximal muscles (lifting the object) and the fingers (gripping the object) during a self-paced lift. Both the SUPCA group and, more markedly, the atrophy group, showed exaggerated levels of grip force. All patients showed a normal rate of grip force development. Damage in the PICA region had no significant effect on any of the measured lifting parameters. All patient groups retained the ability to scale grip force to different object loads. The automatic grip force response to unexpected load increase of a hand held object showed normal latency and time course in all patient groups. The response was modulated by the rate of the load change. Response magnitude was exaggerated in the atrophy patients at all 3 rates tested. CONCLUSIONS: Disturbances associated with cerebellar disorders differed from those seen following damage to the basal ganglia, with no evidence of slowed rates of grip force development. Disruption of temporal coordination between the proximal muscles (lifting) and the fingers (gripping) in a lift was apparent, supporting the role of the cerebellum in coordinating the timing of multi-joint movement sequences. Exaggeration of grip force levels was found in association with damage to the dentate nucleus or, in particular, to its afferent input. This could support a role or the cerebellum in sensorimotor processing, but might also represent a failure to time correctly the duration of grip force generation.

Time course of glial proliferation and glial apoptosis following excitotoxic CNS injury.

Activation of microglial cells and astrocytes after CNS injury results in changes in their morphology, immunophenotype and proliferative activity and has neurotrophic as well as neurotoxic consequences. However, little is known about the exact time course of glial activation as regards their proliferative activity and their fate. In this study, quantification of the densities of proliferating and non-proliferating microglial cells and astrocytes was carried out over 30 days by counting differentially labeled cells in the striatum and substantia nigra pars reticulata (SNr) after injection of quinolinic acid into the rat striatum. The TdT-mediated dUTP nick end labeling (TUNEL)-reaction was used to detect possible apoptotic mechanisms which limit the glial reaction. At 1 day post injection (p.i.) non-proliferating ameboid microglia/macrophages were seen in the striatum, but at 3 and 5 days p.i. many proliferating, ameboid microglia/macrophages and hypertrophic microglia were detected. At 10 days p.i., the time point with the highest density of hypertrophic microglia, TUNEL-positive microglial cells were observed indicating that apoptotic processes play a role in restricting this reaction. In contrast to this, at early time points, a reduction in the density and glial fibrillary acidic protein (GFAP)-immunoreactivity of astrocytes in the striatum was detected. At later time points, a dense astrogliosis with proliferating astrocytes developed in the dorsal and medial striatum. At 30 days p.i., in the entire striatum a dense astrogliosis was detected. The SNr showed a short period of microglial activation and proliferation and a long lasting astrogliosis without proliferation

Motor control in simple bimanual movements: a transcranial magnetic stimulation and reaction time study.

OBJECTIVE: Simple reaction time (RT) can be influenced by transcranial magnetic stimulation (TMS) to the motor cortex. Since TMS differentially affects RT of ipsilateral and contralateral muscles a combined RT and TMS investigation sheds light on cortical motor control of bimanual movements. METHODS: Ten normal subjects and one subject with congenital mirror movements (MM) were investigated with a RT paradigm in which they had to move one or both hands in response to a visual go-signal. Suprathreshold TMS was applied to the motor cortex ipsilateral or contralateral to the moving hand at various interstimulus intervals (ISIs) after presentation of the go-signal. EMG recordings from the thenar muscles of both hands were used to determine the RT. RESULTS: TMS applied to the ipsilateral motor cortex shortened RT when TMS was delivered simultaneously with the go-signal. With increasing ISI between TMS and go-signal the RT was progressively delayed. This delay was more pronounced if TMS was applied contralateral to the moving hand. When normal subjects performed bimanual movements the TMS-induced changes in RT were essentially the same as if they had used the hand in an unimanual task. In the subject with MM, TMS given at the time of the go-signal facilitated both the voluntary and the MM. With increasing ISI, however, RT for voluntary movements and MM increased in parallel. CONCLUSIONS: Ipsilateral TMS affects the timing of hand movements to the same extent regardless of whether the hand is engaged in an unimanual or a bimanual movement. It can be concluded, therefore, that in normal subjects simple bimanual movements are controlled by each motor cortex independently. The results obtained in the subject with MM are consistent with the hypothesis that mirror movements originate from uncrossed corticospinal fibres. The alternative hypothesis that a deficit in transcallosal inhibition leads to MM in the contralateral motor cortex is not compatible with the presented data, because TMS applied to the motor cortex ipsilateral to a voluntary moved hand affected voluntary movements and MM to the same extent.

Sleep stage dependant changes of the high-frequency part of the somatosensory evoked potentials at the thalamus and cortex.

OBJECTIVES: It is known that the high-frequency oscillations (above 400 Hz) of the somatosensory evoked potentials (SEPs) diminish during sleep while the N20 persists (Neurology 38 (1988) 64; Electroenceph clin Neurophysiol 70 (1988) 126; Electroenceph clin Neurophysiol 100 (1996) 189). We investigated possible differential effects of sleep on the 600 Hz SEPs at the thalamus and cortex. METHODS: SEPs from 10 subjects were recorded using 64 channels following electric stimulation at the wrist during awake state and sleep stages II, IV and REM. Dipole source analysis was applied to separate brain-stem, thalamic and cortical activity in the low-frequency (20-450 Hz) and the high-frequency (450-750 Hz) part of the signal. RESULTS: The low-frequency SEPs showed a non-significant increase of the latency of the N20 and a bifid change of the waveform in 3 subjects. The high-frequency SEPs showed a significant decrease of their amplitude at the level of the thalamus and cortex but not at the brain-stem. This decrease in amplitude at the thalamus and cortex were significantly correlated. There was no effect on the latency of the signal. In addition, at the cortex, differential effects on early and late parts of the 600 Hz oscillations were found by time-frequency analysis using a wavelet transformation. CONCLUSIONS: Sleep dependent decrease of the high-frequency SEPs were first observed at the thalamus pointing to the known function of the reticular thalamic nucleus regulating arousal. The results presented here provide further evidence for a thalamic origin of the 600 Hz oscillations. In addition, on the basis of the differential effects on early (up to the N20 peak) and late (between 20 and 25 ms) parts of the signal, at least one intracortical generator of these oscillations is proposed. In general, the high-frequency SEPs (600 Hz oscillations) are supposed to reflect activity of a somatosensory arousal system.

[Recurrent dreams as migraine aura symptoms]. / Wiederkehrende Träume als Aurasymptome der Migräne.

Elementary geometric imagery seen in the visual aura of migraine can be experienced as incorporated into the content of a dream which precedes the awakening with a migraine headache. Furthermore, recurrent dreams featuring complex visual imagery, often terrifying nightmares, can occur as migraine aura symptoms. The said phenomena are illustrated by two original case reports and discussed against the background of a review of the literature.

Modulation of the neuronal circuitry subserving working memory in healthy human subjects by repetitive transcranial magnetic stimulation.

We studied the effect of repetitive transcranial magnetic stimulation (rTMS) on changes in regional cerebral blood flow (rCBF) as revealed by positron emission tomography (PET) while subjects performed a 2-back verbal working memory (WM) task. rTMS to the right or left dorsolateral prefrontal cortex (DLPFC), but not to the midline frontal cortex, significantly worsened performance in the WM task while inducing significant reductions in rCBF at the stimulation site and in distant brain regions. These results for the first time demonstrate the ability of rTMS to produce temporary functional lesions in elements of a neuronal network thus changing its distributed activations and resulting in behavioral consequences.