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Dysarthria is a frequent symptom in cerebral ischaemia. However, speech characteristics of these patients have not previously been investigated in relation to lesion site in a prospective study. We investigated the auditory perceptual features in 62 consecutive patients with dysarthria due to a single, non-space-occupying cerebral infarction confirmed by MRI. Standardized speech samples of all patients were stored within 72 h after stroke onset using a digital tape recorder. Speech samples were assessed independently by two experienced speech therapists, who were unaware of the clinical and neuroradiological findings, using an interval scale ranging from 0 to 6. Separately assessed were features of articulation, phonation, prosody, and the global severity for a total of 31 items. Extracerebellar infarctions (85.5%) were located in the lower motor cortex (14.5%), striatocapsular region (46.8%) and base of the pons (24.2%). Isolated cerebellar infarctions were present in 14.5% of patients. There was a strong correlation between the findings of both examiners, showing identical scores, or only minor differences (<1 on the assessment scale) for 80% of all items. The average severity of dysarthria was 2.9 ± 1.3. Articulatory abnormalities were the predominant deviation characteristics, affecting in particular the production of consonants. However, phonatory and prosodic abnormalities were also frequently observed speech characteristics. As revealed by factor analysis of speech characteristics the total severity of dysarthria was mainly influenced by the impairment of articulation. Speech parameters describing characteristics of articulation and prosody showed significant side-to-side and area differences, while this effect was lacking for any voice parameter. Left cerebral lesions showed a more severe overall impairment of speech and articulation, independent of lesion topography. Thirty-eight of 62 patients were available for follow-up. Speech evaluation showed normal speech within weeks in 15 out of 38 patients (39.5%). In the other 23 patients overall severity of dysarthria was mild. This is the first prospective study which describes speech characteristics of dysarthria due to acute unilateral cerebral infarctions. We could demonstrate that dysarthria in extracerebellar infarctions was more frequently caused by left-sided lesions and that the severity of dysarthria was more pronounced in left-sided lesions independent from lesion topography. All extracerebellar lesions were located along the course of the cortico-bulbar tract fibres. Compatible with a common pathophysiological basis of dysarthria in these patients, none of the 31 speech items differed significantly between subcortical and brainstem lesions.

Die Kombination eines Angiotensinkonversionsenzym- (ACE-)Hemmers mit einem Spironolacton bei Herzinsuffizienz ist derzeit ein anerkanntes und effizientes Therapiekonzept. Eine wichtige Nebenwirkung ist dabei jedoch die Entwicklung von Hyperkaliämien, die durch bestimmte Risikofaktoren (Diabetes mellitus, präterminale Niereninsuffizienz, fortgeschrittenes Alter) noch begünstigt werden. Wir berichten im vorliegenden Fall über eine Patientin, die unter genannter Kombinationstherapie eine exzessive Hyperkaliämie (11,4 mmol/l) mit konsekutivem Herzstillstand erlitt. Unter kardiopulmonaler Reanimation wurde eine kontinuierliche venovenöse Hämodiafiltration („continuous veno-venous hemodiafiltration“, CVVHDF) durchgeführt, so dass etwa 30 min nach Beginn der Reanimationsmaßnahmen ein stabiler Sinusrhythmus wiederhergestellt werden konnte (Serumkalium 1 h nach Beginn der Hämodiafiltration: 7,0 mmol/l). Die Patientin überlebte ohne neurologisches Defizit.

Synthesis of 2,3,2',3'-tetrahydro-1H,1'H-[2,2']biindenyl 2 from the dimerization reaction of indene with lithium naphthalenide was carried out and we here report its single crystal X-ray structure. Synthesis and characterization of compounds 3-8 have also been carried out successfully. The stability of biindenyl and its stereochemistry at position 2 and 2' was investigated using density functional theory.

We report a case of a 74-year-old male patient, in whom cardiogenic shock with fluid lung was induced by perforation of an abdominal aortic aneurysm in the inferior vena cava forming an aortocaval fistula. The diagnosis was made by computed tomography (contrast enhanced) and transabdominal duplex sonography. After the operation (running suture of the inferior vena cava and exclusion of the aneurysm with a Dacron bifurcation graft), the patient eventually recovered after 28 days of mechanical ventilation and 15 days of continuous veno-venous hemodiafiltration. The patient was discharged home 68 days after the operation in good functional and neurological status. Wir berichten in der vorliegenden Kasuistik über einen 74-jährigen Patienten, bei dem es nach Perforation eines Bauchaortenaneurysmas in die Vena cava inferior durch den konsekutiven Links-Rechts-Shunt zu einem akuten Lungenödem mit kardiogenem Schock gekommen war. Die Diagnostik wurde durch Kontrastmittel-unterstützte Computertomographie sowie transabdominelle Duplex-Sonographie gestellt. Nach erfolgreicher Operation (Verschluss der Perforationsstelle an der Vena cava inferior sowie Interposition einer Bifurkationsprothese aortoiliacal) konnte der Patient nach insgesamt 30-tägiger intensivmedizinischer Behandlung (28 Tage Beatmung, 15 Tage Hämodiafiltration postoperativ) in die Anschlussheilbehandlung verlegt werden. Die Entlassung aus der Rehabilitationsbehandlung erfolgte nach weiteren 38 Tagen, der Patient erholte sich vollständig.

Rod-shaped 2,3-di-alkoxytetracenes, soluble in common organic solvents, have been synthesized and studied for their gelling ability in organic solvents and their unusual UV−visible spectroscopic properties.

Zusammenfassung

Zusammenfassung 1928 beschrieb Hugo Friedrich Kufs erstmalig eine Familie mit zerebralen, retinalen und kutanen Kavernomen. Mittlerweile wurden über 300 weitere Familien beschrieben. Ebenfalls wurden drei Genloci 7q21-q22 (mit dem Gen CCM1), 7p15-p13 (Gen CCM2) und 3q25.2-q27 (Gen CCM3) beschrieben, in denen Mutationen zu Kavernomen führen. Das Genprodukt von CCM1 ist das Protein Krit1 (Krev Interaction Trapped 1), das über verschiedene Mechanismen mit der Angiogenese interagiert. Das neu entdeckte CCM2-Gen enkodiert ein Protein, das möglicherweise eine dem Krit1 ähnliche Funktion in der Regulation der Angiogenese hat. Das CCM3-Gen wurde noch nicht beschrieben. In dieser Arbeit werden sowohl die klinischen und genetischen Befunde bei 15 deutschen Familien beschrieben.

The mechanisms of perphenylbutenyne reactivity are examined through B3LYP and multireference ab initio calculations on model systems. Calculations for the formation of a naphthalene derivative suggest a process similar to that seen previously in the literature. A new mechanism for perphenylbutenyne dimerization to form a semibullvalene derivative is proposed and supported by calculations.

Our current understanding of brainstem reflex physiology comes chiefly from the classic anatomical–functional correlation studies that traced the central circuits underlying brainstem reflexes and establishing reflex abnormalities as markers for specific areas of lesion. These studies nevertheless had the disadvantage of deriving from post-mortem findings in only a few patients. We developed a voxel-based model of the human brainstem designed to import and normalize MRIs, select groups of patients with or without a given dysfunction, compare their MRIs statistically, and construct three-plane maps showing the statistical probability of lesion. Using this method, we studied 180 patients with focal brainstem infarction. All subjects underwent a dedicated MRI study of the brainstem and the whole series of brainstem tests currently used in clinical neurophysiology: early (R1) and late (R2) blink reflex, early (SP1) and late (SP2) masseter inhibitory reflex, and the jaw jerk to chin tapping. Significance levels were highest for R1, SP1 and R2 afferent abnormalities. Patients with abnormalities in all three reflexes had lesions involving the primary sensory neurons in the ventral pons, before the afferents directed to the respective reflex circuits diverge. Patients with an isolated abnormality of R1 and SP1 responses had lesions that involved the ipsilateral dorsal pons, near the fourth ventricle floor, and lay close to each other. The area with the highest probabilities of lesion for the R2-afferent abnormality was in the ipsilateral dorsal–lateral medulla at the inferior olive level. SP2 abnormalities reached a low level of significance, in the same region as R2. Only few patients had a crossed-type abnormality of SP1, SP2 or R2; that of SP1 reached significance in the median pontine tegmentum rostral to the main trigeminal nucleus. Although abnormal in 38 patients, the jaw jerk appeared to have no cluster location. Because our voxel-based model quantitatively compares lesions in patients with or without a given reflex abnormality, it minimizes the risk that the significant areas depict vascular territories rather than common spots within the territory housing the reflex circuit. By analysing statistical data for a large cohort of patients, it also identifies the most frequent lesion location for each response. The finding of multireflex abnormalities reflects damage of the primary afferent neurons; hence it provides no evidence of an intra-axial lesion. The jaw jerk, perhaps the brainstem reflex most widely used in clinical neurophysiology, had no apparent topodiagnostic value, probably because it depends strongly on peripheral variables, including dental occlusion.

Irradiation of 5,12-didecyloxytetracene (1) leads to photodimers P1 (planosymmetric) and P2 (centrosymmetric). P1 is characterized by naphthalene excimer fluorescence, whereas P2 emits naphthalene monomer fluorescence.

We acquired high-resolution MRI and anisotropically diffusion-weighted images (DWI) with direction-selective gradients of the brain stem in 20 healthy volunteers, to identify brain-stem structures such as white-matter tracts and nuclei which show diffusion anisotropy. After averaging and superposition of individual cuts, the images were projected onto appropriate plates of the Schaltenbrand and Wahren anatomical atlas. We identified 20 structures – white-matter tracts and some nuclei – with high contrast. The direction of fibres could be determined as areas of increased (parallel to) or decreased diffusion (perpendicular to the gradient). This study may contribute to understanding of the functional anatomy of the brain stem.

Transcranial magnetic stimulation was used to investigate the corticofacial projections in 53 patients with (n = 28) and without (n = 25) central facial paresis due to unifocal ischaemic lesions at different brainstem levels. Lesion topography documented by MRI studies was correlated with the electrophysiological findings. In the majority of patients the corticofacial fibres travel within the ventromedial base of the pons and cross the midline at the level of the facial nucleus. In some individuals, however, we found evidence that corticolingual fibres form an 'aberrant bundle' in a paralemniscal position at the dorsal edge of the pontine base. In other patients the corticofacial fibres loop down into the ventral part of the upper medulla, cross the midline and ascend in the dorsolateral medullary region ipsilaterally to the facial nucleus. The findings suggest that facial paresis due to a brainstem lesion may present as contralateral supranuclear facial paresis by a lesion of the cerebral peduncle, pontine base, the aberrant bundle and the ventral medulla. Supranuclear facial paresis ipsilateral to the lesion side may result from a lesion in the lateral medulla, and facial paresis of the supranuclear type may be imitated by a lesion of the peripheral facial nerve in the dorsolateral medulla with involvement of the lower pons.

We determined the incidence of persistent back pain (PBP) after non-obstetrical spinal anaesthesia (SPA) and investigated factors predisposing to such pain in a prospective 1 yr follow-up study in 245 patients undergoing elective general or trauma surgery (218 patients undergoing single SPA, 27 undergoing two to six SPAs). All patients received a first questionnaire 3 months after the last SPA, and those reporting PBP after 3 months were sent a second questionnaire I year after the operation. Variables were PBP before and within 5 days, at 3 months and I year after SPA, patient satisfaction with SPA, patient characteristics and technical data. Statistical analysis was by contingency tables with Fisher's exact test and an unpaired t-test with logistic regression (P < 0.001 after Bonferroni correction was taken as significant). The response rate in patients who had a single SPA was 56% (122/218). Twenty-three of these 122 patients (18.9%) complained of back pain before SPA compared with 12/122 (10.7%, P = 0.0015) within 5 days after SPA. After 3 months, 15/122 patients (12.3%) reported PBP with 14 complaining of PBP before SPA (P < 0.0001), corresponding to an incidence of new PBP of 1/122 (0.8%). Multiple logistic regression revealed that pre-existing back pain was the only variable associated with PBP after 3 months (P < 0.0001). Patient characteristics and technical factors were not associated with PBP. Nine of the 15 patients with PBP after 3 months returned the second questionnaire: four still reported PBP (three of these had suffered from PBP before SPA). Despite PBP after 3 months, 13/15 patients said they would opt for SPA again. The response rate and results in patients who had had multiple SPAs were similiar to those who had had a single SPA.

The molecular structure and conformations of butyronitrile have been studied experimentally by the gas electron diffraction method. A conformational mixture of 75.1% gaucheand 24.9% anti, with standard deviation equal to 6.0%, was observed. Results from ab initio MP2/6-31G∗optimization calculations are in excellent agreement with those observed.

The molecular structure and conformational composition of 3-hexyn-1,6-diol have been studied by the gas electron diffraction method and by ab initio calculations. Four conformers (AA, G+G−, G+G+ and GA) have been taken into account. The experimental data are in accordance with a conformational mixture consisting of 62.2% GG(equal parts of G+G− and G+G+) and 37.8% GAwith a standard deviation of 10.3%. Similar results were, however, obtained when a small contribution (0≤x≥12%) from the high energy AAconformer was introduced and the other forms were properly corrected (GG(62.2+x%); GA(37.8−2x%)). From the experimental data the total amount of gauchecontribution in the two parts of the molecule is accordingly estimated to be in the region 76.0–86.3%. The GGand GAconformers are characterized by the formation of two, respectively one, OH⋯π hydrogen bonds.

We describe two patients with upper medullary infarctions showing ipsilateral facial weakness and relative sparing of the upper facial muscles. Electrophysiological follow-up using transcranial magnetic stimulation of the motor cortex in combination with stimulation of the peripheral facial nerve disclosed a supranuclear (corticofacial) tract lesion in one patient and a partial nuclear/infranuclear intra-axial facial nerve lesion in another.

The molecular structure and conformations of 1-pentyne have been studied experimentally by the gas electron diffraction method. A conformational mixture of 68.6% gaucheand 31.4% anti, with standard deviation equal to 4.5%, was observed. Results from ab initio MP2/6-31G∗optimization calculations are in excellent agreement with those observed.

Abstract: We performed MRI, including diffusion-weighted imaging, in 15 patients with recurrent strokes with acute ischaemia and at least one old lesion according to the clinical history and/or CT. Routine MRI showed similar signal intensity changes in both situations. Diffusion-weighted images, however, were positive in all acute or subacute infarcts. The high signal of acutely disturbed diffusion due to intracellular oedema could also be identified in small brain stem lesions. Spatial resolution was increased by applying separate gradients in each axis instead of creating anisotropy-independent trace images.

We investigated two patients presenting with the rare finding of almost isolated hemianalgesia with a sensory level on the contralateral side sparing the face. Clinical findings, electrophysiological studies (absent laser-evoked pain-related somatosensory potentials, normal electrically evoked somatosensory potentials, magnetically evoked potentials, and blink reflexes), and magnetic resonance imaging showed the ventrolateral medullar tegmentum containing the spinothalamic tract to be affected by lacunar infarction. The blink reflex R2 component was unimpaired in both patients.

The infrared spectra of 1-cyano-3-butyne were recorded in the region 4000-50 cm-1in the vapour and liquid states, as an amorphous and as two different crystalline solids at 90 K. In addition the compound was studied as a high pressure crystalline solid at ambient temperature. Raman spectra of the liquid, including polarization measurements, and of the amorphous and the two crystalline solids at 90 K, were obtained.

We describe a patient with isolated voluntary facial paresis due to a unilateral lacunar lesion in the contralateral mediodorsal middle base of the pons. Transcranial magnetic stimulation confirmed the involvement of supranuclear corticofacial tract fibers and sparing of the corticolingual and corticospinal connections. This observation demonstrates that the fibers conveying voluntary orofacial activation descend mediodorsally at the level of the middle pons and that the fibers conveying emotional activation may be assumed to converge below this level.

An acute limbic-cerebellar syndrome was seen in six industrial workers who inhaled trimethyltin (TMT). Clinical features included hearing loss, disorientation, confabulation, amnesia, aggressiveness, hyperphagia, disturbed sexual behavior, complex partial and tonic-clonic seizures, nystagmus, ataxia, and mild sensory neuropathy. Severity paralleled maximal urinary organotin levels. One patient died and two remained seriously disabled.

Acute organophosphate intoxication resulting from suicide attempts in 14 patients produced a series of electrophysiologic abnormalities that correlated with the clinical course. Spontaneous repetitive firing of single evoked compound muscle action potentials (CMAP) was the earliest and most sensitive indicator of the acetylcholinesterase inhibition. A decrement of evoked CMAP following repetitive nerve stimulation was the most severe abnormality. At the height of the intoxication no CMAP was evoked after the first few stimuli. The decrement-increment phenomenon occurred only at milder stages of intoxication and its features are characteristic of acetylcholinesterase inhibition. These electrophysiologic features proved to be the most useful for determining initial severity and clinical course of the acute organophosphate intoxication and differentiated this syndrome from those of myasthenia gravis, Eaton-Lambert syndrome, and botulism.

Phosphorescence in ethanol at 77 K of the cyclophanes 1-14 has been investigated. It is shown that the phosphorescence characteristics are strongly determined by transannular interaction between the benzene rings. This interaction does not occur in cyclophanes 9 and 14. Phosphorescence quenching by silver Perchlorate of paracyclophanes and benzene derivatives 15-20 is due to the formation of arene/Ag+ground state complexes. From the quenching data it is concluded that the complexes of paracyclophanes with Ag+ions are more stable than the corresponding complexes of the benzene derivatives by approximately two orders of magnitude. Various factors are discussed regarding this marked difference in complex stability.

Two patients are reported with symptoms and signs of peripheral vestibular dysfunction, including severe rotatory vertigo, abolished caloric response in the involved ear, and spontaneous nystagmus with the fast component away from that ear. In addition, paresis of the masseter, temporal, and pterygoid muscles and impairment of the masseter reflex were found on the affected side. It is suggested that the vertigo and masseter paresis syndrome is a brainstem syndrome which is probably due to a vascular lesion affecting a very small dorsolateral pontine area.

VEPs were measured after pattern reversal in 135 MS patients and 30 control subjects. Neurological findings were documented in a standard manner. An extensive ophthalmological examination of all subjects was part of the study. The latency of P2 was abnormally delayed in 82% of the “definite”, in 60% of the “probable” and in 65% of the “possible” MS groups respectively. The VEP was more often delayed in relation to ophthalmological disturbances. Changes in the MS classification had to be made in more than 10% of the patients, due to delay of VEP latency. These were patients with a “spinal” form of MS, which is known to create diagnostic problems.

In a 43-year-old man dyscephalia, cataracta congenita, and hypotrichosis were the outstanding features. These signs were first described in 1953 by Ullrich and Fremerey-Dohna as a clinical entity. Since 1958 the DCH syndrome was published under the synonyms of “Francois syndrome” and of “Hallermann-Streiff syndrome”. However, as these authors did not add any essential details relevant for the classification of the syndrome we prefer to retain the term “Ullrich-Fremerey-Dohna syndrome”.

An die relative Refraktärperiode schließt sich eine supernormale Phase an und daran wiederum eine späte subnormale Phase. Die Wirkung dieser beiden letzten Phasen auf die Leitgeschwindigkeit wurde an sensiblen Nervenfasern untersucht. 80% der Untersuchten zeigten während der supernormalen Phase (4–10 msec nach der ersten Aktivierung) eine Beschleunigung der Leitgeschwindigkeit auf 106% des Normwertes. Die Beschleunigung ist weniger ausgeprägt, wenn die Gewebstemperatur erniedrigt ist. Bei 65% der Versuchspersonen wurde eine Verzögerung der Impulsfortleitung während der subnormalen Phase (10–100 msec nach voraufgegangener Aktivierung) beobachtet. Die Veränderungen der Leitgeschwindigkeit laufen zu den bekannten Veränderungen der Membranerregbarkeit parallel.

Tone pips of suprathreshold intensities elicit an acoustic nerve response (I) and six low amplitude brainstem potentials (II–VII) during the initial 10 ms. Seven waves were studied in 40 control subjects and 5 waves (I–V) in 47 patients with MS. The results suggest involvement of the auditory pathway of 24 of 27 patients in the clinically “definite”, of 5 of 9 cases in the “probable” and in none of 5 patients in the “possible” MS groups. EAEPs were normal in 6 cases with a spinal form with one exception where changes of potential were indicative of a midbrain lesion. Dysfunction within the acoustic pathway was observed at the level of the acoustic nerve and in the medulla oblongata, pons and midbrain. The significance of the bilateral EAEP abnormalities found in some patients at different levels is discussed with regard to a polytopic location of the underlying lesion.

Diazepam exhibits various effects on motor nerves and skeletal muscle: 1. The maximum conduction velocity is decreased, 2. the duration of the negative component of the muscle action potential is increased, 3. the latency between the electrical and the mechanical response is increased, 4. the muscle contracts and 5. relaxes more slowly, and 6. the muscle force is reduced particularly with repetitive activation.

Zusammenfassung: Aufgrund der Ergebnisse einer mehrjährigen interdisziplinären empirischen Untersuchung an Patienten mit der Leitsymptomatik „Schwindel” zum Thema „Psychosomatische Aspekte des Schwindels” und auf dem Hintergrund psychodynamischer Theorien zu Angsterkrankungen wird der von Brandt und Dieterich 1986 eingeführte Begriff des „phobischen Schwankschwindels” diskutiert. Deutlich wird, daß es sich dabei um eine nicht differenzierende Beschreibung handelt, die unterschiedliche somatoforme (psychogene und psychosomatische) Schwindelzustände umfaßt. Die Autoren plädieren für eine nosologische Subgruppen berücksichtigende Klassifikation psychogen bedingter Schwindelzustände.

Abstract: Narcolepsy is associated with various rapid eye movement (REM) sleep abnormalities. Distinct brain stem areas seem to play a prominent role in REM sleep regulation. Recent magnetic resonance imaging (MRI) studies have led to conflicting findings concerning the presence of structural brain stem lesions in patients with idiopathic narcoleptic syndrome. However, multimodal electrophysiological brain stem investigations may reveal functional brain stem abnormalities even in the absence of MRI abnormality. Therefore we investigated brain stem function in 12 idiopathic narcoleptic patients by systematically studying tegmental brain stem pathways. All of the patients met the diagnostic criteria of the International Classification of Sleep Disorders, with typical changes in polysomnography and the multiple sleep latency test. Electrophysiological investigations comprised masseter reflex, blink reflex, masseter inhibitory reflex, early auditory evoked potentials and electrooculography with vestibular testing. In no patient were electrophysiological brain stem abnormalities observed. Our findings do not support the existence of a relevant brain stem lesion in narcoleptic patients with normal neurological status.