Toxisch bedingte Myopathien

Sammelbezeichnung fur Arzneimittel-bedingte Myopathien mit vornehmlich subakuter und akuter Verlaufsform, wobei es bis zur Rhabdomyolyse kommen kann. Besserung oder vollstandige Ruckbildung nach Absetzen der Medikation. Der zeitliche Zusammenhang (24 Stunden bis zu Monaten) und die Dosisabhangigkeit sind unterschiedlich. Klinisch finden sich sowohl schmerzhafte als auch schmerzlose Verlaufsformen, sowie Ubergange zu Myalgien, Muskelkrampf-Syndromen bzw. chronisch-atrophierenden Verlaufsformen. Lokalisation vorwiegend proximal an der Stammuskulatur; besonders beim akuten Syndrom kann es zu generalisierten Formen mit haufig kardialer Beteiligung kommen. Reflexe meist auslosbar. Klinisch finde…

The behaviour of OKT3-, OKT4- and OKT8-positive cells during phases of elevated spontaneous chemiluminescence activity (CL-A) in multiple sclerosis patients. A serial study.

The chemiluminescence activity (CL-A; synonym = burst activity, BA) and the percentage of OKT3-, OKT4- and OKT8-positive peripheral blood cells were serially examined in four control persons and in eight patients with multiple sclerosis. When the OKT values obtained in phases of increased CL-A (clinical remission) were compared with those of the control group, the percentage of OKT3-positive cells was reduced (P = 0.014), and that of OKT4-positive cells increased (P = 0.014); there were no significant changes in the percentage of OKT8-positive cells (P = 0.171). After the CL-A had returned to normal values, the OKT4-positive cells remained elevated (P = 0.029), whereas the OKT3- (P = 0.342)…

Indication of a common origin of German and American Families with Familial Amyloidneuropathy Typ II

Abstract The classification of familial amyloid neuropathies (FAP) is traditionally based on clinical and regional aspects. In the last 10 years more than 40 mutations of the transthyretin gene have been found to be responsible for different clinical forms of amyloidosis including familial FAP.FAP II is caused by a mutation on the codon 58 of the transthyretin gene. Only two american kindreds (the Maryland/German and the Ohio family) have previously been reported with FAP II starting in the 3rd or 4th decade by sensory disturbances of the hands typically as a carpal tunnel syndrome. We report on a german family with FAP II from the rhine river area south of Mainz. Four members with typical …

Indications of the occurrence of inflammatory reactions in the clinical improvement phase in multiple sclerosis patients.

– In patients with multiple sclerosis (MS) the spontaneous burst activity (BA) of peripheral blood mononuclear cells was related to the clinical course of the disease. In five patients clinical improvement was found while the BA was significantly increased (more than 300% of the controls). During the appearance of new or deteriorating signs and in the period without clinical changes, the BA was not at all or not markedly increased. In two patients without clinical improvement the BA did not reach levels above 300% of the controls. Our findings suggest that inflammatory reactions represented by the BA occur in the phase of clinical improvement. Since burst-stimulating activity was found in t…

Brainstem reflex circuits revisited

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…

Anxiety disorders and other psychiatric subgroups in patients complaining of dizziness.

Two hundred and two consecutive patients with dizziness were evaluated using blind neuro-otological testing and examination, blind psychiatric examination, including structured interviews (according to DSM-IV), the Symptom Check-List (SCL 90 R), and the State-Trait Anxiety Inventory (STAI). In 28% of the patients (N=50) dizziness was of organic origin (O group); in 55.3% (N=99) of psychogenic origin (P group) and in 16.8% comorbid psychiatric disorders were found (Mixed group). In 5.3% (N=10) neither organic nor psychiatric results could be found, which could explain the dizziness (Ideopathic group). Compared with the Organic group the patients with psychiatric disorders (P and Mixed group)…

P25. Sensitivity of imaging and electrophysiological brainstem testing in the diagnosis of acute vertebrobasilar ischemia

The course of corticofacial projections in the human brainstem.

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 loo…

Emergency MRI in Clinical Peracute Brain Stem and Hemispheric Stroke

We show the capability and results of MRI examinations of peracute brainstem and hemispheric stroke patients in clinical emergency situations. The hypothesis that CT scan is faster and more accurate under this conditions is to be disproved.

Paradoxical heat sensation in patients with multiple sclerosis

Temperature thresholds were determined in 16 patients with probable or definite multiple sclerosis, in six patients with possible but unconfirmed multiple sclerosis and in 34 healthy subjects, using the method of limits and the thermal sensory limen (TSL) of the MarStock technique. A significant proportion of the patients had thresholds outside the 2.5 SD range for normal subjects, both for warmth detection threshold and TSL. In addition, 10 patients with probable or definite multiple sclerosis and one patient with possible multiple sclerosis reported a paradoxical heat sensation, i.e. a sensation of warmth elicited by a cold stimulus. This illusion was almost exclusively observed with the …

Cranial nerve function in workers exposed to polychlorinated dioxins and furans

OBJECTIVE: To look for possible effects of polychlorinated dioxins and furans (PCDD/F) on cranial nerve function. MATERIAL AND METHODS: Clinical and neurophysiological examinations [visual and brainstem auditory evoked potentials (VEP and BAEP), blink reflex] in 121 PCDD/F exposed workers of one pesticide producing plant. RESULTS: BAEP abnormalities were more frequent in workers with chloracne (6 of 33 workers, 18.2%) than in those without chloracne (7 of 84, 8.3%), but this was not statistically significant (chi2: 2.33). VEP abnormalities were seen in one worker with and two without chloracne. Clinically visual functions were normal except in one worker, who was amaurotic since birth. Blin…

Dyskephalie-Katarakt-Hypotrichose-Syndrom (Synonyma: DCH; Ullrich-Fremerey-Dohna; Hallermann-Streiff; Francois)

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”. In our case in addition to the above mentioned and well known manifestations, extrapyramidal hyperkinesia of the choreoanthetotic type and servere mental deficiency accompanied by mild cerebral atrophy (revea…

Dysarthria in acute ischemic stroke: lesion topography, clinicoradiologic correlation, and etiology.

Background and purpose: Although dysarthria is a frequent symptom in cerebral ischemia, there is little information on its anatomic specificity, spectrum of associated clinical characteristics, and etiologic mechanisms. Methods: An investigation of 68 consecutive patients with sudden onset of dysarthria due to a single infarction confirmed by MRI or CT was conducted. Results: Dysarthria was associated with a classic lacunar stroke syndrome in 52.9% of patients. Isolated dysarthria and dysarthria–central facial and lingual paresis occurred in 2.9% (n = 2) and 10.3% (n = 7), respectively. Dysarthria–clumsy hand syndrome was observed in 11.7% (n = 8) of patients and associated with pure motor …

Effects of the NMDA-receptor antagonist ketamine on perceptual correlates of long-term potentiation within the nociceptive system

We recently reported perceptual correlates of long-term potentiation (LTP) of synaptic strength within the nociceptive system demonstrating the functional relevance of LTP for human pain sensation. LTP is generally classified as NMDA-receptor dependent or independent. Here we show that low doses of the NMDA-receptor antagonist ketamine (0.25 mg/kg) prevented the long-term increase in perceived pain to electrical test stimuli, which was induced by high-frequency electrical stimulation (HFS) of nociceptive afferents. Whereas in a control experiment HFS led to a stable increase in perceived pain by 51% for the entire observation period of 1 h HFS given 4 min after i.v. ketamine was ineffective…

Persistent Tonic Facial Contraction: A Local Brain-Stem Sign

Impairment of facial motility associated with contralateral hemiparesis is characteristic of pontine lesions. In the Millard-Gubler and Foville syndromes, involvement of the facial nerve is nuclear or infranuclear, resulting in a peripheral-type palsy. Conditions of pontine origin showing hyperactivity of facial muscles are facial myokymia and Brissaud’s syndrome. Brissaud and Sicard [4] reported low-frequency phasic cramplike facial contractions and contralateral hemiparesis in inflammatory brain-stem processes.

Periodische dyskaliämische Lähmungen

Sammelbezeichnung fur anfallsweise (episodisch) auftretende Muskelschwache, verbunden mit Anderung des Kaliumspiegels im Serum.

Superficial siderosis of the CNS as a rare differential diagnosis of chronic low back pain

Topodiagnostic significance of hemiataxia: An MRI based mapping analysis

Muskelfehlbildungen, Aplasien und Anlagevarianten

Totales oder partielles Fehlen eines funktionell wichtigen Muskels. Vorkommen einseitig oder doppelseitig. Kombination mit Entwicklungsstorungen von Integument und Skelett (obere Extremitat) und/oder von inneren Organen (z. B. beim Bauchmuskeldefekt).

Vertigo, Masseter Paresis or Masseter Reflex Abnormality, and Impaired Caloric Response

Symptoms and signs of acute peripheral-type vestibular abnormalities occasionally occur in patients with multiple sclerosis and brain-stem ischemia. Such patients may show reduced excitability of the lateral semicircular canal. From this observation, the question arises as to the location of the causative lesion.

Abolished laser-evoked potentials and normal blink reflex in midlateral medullary infarction.

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.

Scalp, basal epidural and intravascular far-field recordings after median nerve stimulation: evidence for a separate N18a potential

Far-field somatosensory evoked potentials (SSEPs) after median nerve stimulation were recorded from scalp- (Fz), epidural- (ED) and intravascular electrodes (basilar artery [Bas]) to study the nature of the controversial N18a component of the widespread N18 potential. In healthy volunteers frequently an N18a potential was recorded at Fz. Simultaneous Fz and ED recordings at the pontomesencephalic junction as well as Bas-recordings at the caudal basilar artery showed N18a components identical in latency and shape. With intravascular recordings the shapes differed between the top of the basilar artery and the caudal artery recordings. These findings support the existence of a separate N18a po…

Physikalisch bedingte Myopathien

Verletzung des Muskelgewebes durch mechanische Einwirkung (Uberdehnung, Kontraktion, ausere Gewalt).

A new method to investigate brain stem structural-functional correlations using digital post-processing MRI - reliability in ischemic internuclear ophthalmoplegia

We investigated the reliability of a new digital post-processing magnetic resonance imaging (MRI) technique in ischemic brain stem lesions to identify relations of the lesion to anatomical brain stem structures. The target was a medial longitudinal fasciculus (MLF) lesion, which was evident from ipsilateral internuclear ophthalmoplegia (INO). Sixteen patients with acute unilateral INO and an isolated acute brain stem lesion in T2- and EPI-diffusion weighted MRI within 2 days after the onset of symptoms were studied. The MRI slice direction was parallel and perpendicular to a slice selection of a stereotactic anatomical atlas. The individual slices were normalized and projected in the digita…

Blink reflex R2 changes and localisation of lesions in the lower brainstem (Wallenberg's syndrome): an electrophysiological and MRI study

OBJECTIVES—Pathways of late blink reflexes are detected by high resolution MRI. Electronically matched stroke lesions superimposed to an anatomical atlas show the suspected course. METHODS—Fifteen patients with infarction of the lower brainstem, MRI lesions and electrically elicited blink reflexes were examined. The involved structures in patients with R2 and R2c blink reflex changes were identified by biplane high resolution MRI with individual slices matched to an anatomical atlas at 10 different levels using digital postprocessing methods. RESULTS—The blink reflexes were normal in five of 15 patients (33%) and showed loss or delay of R2 and R2c to stimulation ipsilaterally to lesion (R2-…

Diffusionsgewichtetes MRT bei vertebrobasil�ren Isch�mien

The aim of this study was to evaluate the applicability, sensitivity, and predictive power of diffusion-weighted MR imaging (DWI) in the diagnosis of vertebrobasilar infarction. From 1997 to 2002, we prospectively recruited 268 patients with acute signs and symptoms suspective of vertebrobasilar ischemia. The patients underwent biplanar EPI-T2 and EPI DWI within 24 h after onset of symptoms and high-resolution MRI as a control within 7 days. One hundred twenty-one patients had additional CT scanning. The DWI revealed acute vertebrobasilar infarction in 71.0%. The mean time exposure of DWI was 8 min and thus no more than that of CT imaging. It showed significantly more acute lesions than CT …

Electrophysiological brainstem investigations in obstructive sleep apnoea syndrome.

Phasic inspiratory genioglossus activity prevents pharyngeal airway collapse in healthy subjects during sleep and is diminished or absent in obstructive sleep apnoea syndrome (OSAS), thus leading to pharyngeal obstruction. Case reports of OSAS after pontomedullary lesions indicate that impaired inspiratory genioglossal activity may result from brainstem lesions. We therefore investigated brainstem functions in 18 awake patients with OSAS using brainstem auditory evoked potentials, blink reflex, masseter reflex, masseter inhibitory reflex (in 11 of 18 patients), magnetic evoked potentials of the tongue and electrooculography with vestibular testing. Fifteen of 18 patients showed no electroph…

Adaptive frequency decomposition of EEG with subsequent expert system analysis.

We present a hybrid system for automatic analysis of clinical routine EEG, comprising a spectral analysis and an expert system. EEG raw data are transformed into the time-frequency domain by the so-called adaptive frequency decomposition. The resulting frequency components are converted into pseudo-linguistic facts via fuzzification. Finally, an expert system applies symbolic rules formulated by the neurologist to evaluate the extracted EEG features. The system detects artefacts, describes alpha rhythm by frequency, amplitude, and stability and after artefact rejection detects pathologic slow activity. All results are displayed as linguistic terms, numerical values and maps of temporal exte…

Electrophysiological brain stem investigations in idiopathic narcolepsy.

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…

A topodiagnostic investigation on body lateropulsion in medullary infarcts.

Body lateropulsion may occur without signs of vestibular dysfunction and vestibular nucleus involvement. The authors examined 10 such patients with three-dimensional brainstem mapping. Body lateropulsion without limb ataxia reflected an impairment of vestibulospinal postural control caused by a lesion of the descending lateral vestibulospinal tract, whereas body lateropulsion with limb ataxia was probably the consequence of impaired or absent proprioceptive information caused by a lesion of the ascending dorsal spino-cerebellar tract.

The spontaneous burst activity of peripheral blood monocytes in patients with acute polyradiculoneuritis, lymphocytic meningoencephalitis, and multiple sclerosis

Abstract The course of the spontaneous burst activity (BA) of peripheral blood monocytes was examined in patients with acute polyradiculoneuritis (PN), lymphocytic meningoencephalitis (LE), and multiple sclerosis (MS) and the BA was compared with the clinical course. In 4 patients with postinfectious acute PN the BA was significantly increased up to values around 60000 counts/10 s. The BA and the clinical course were closely correlated in these patients (mean of r = 0.83). In 4 patients with lymphocytic LE the BA initially was moderately increased to values between 4000 and 5000 counts/10 s and showed again a very close correlation with the clinical course (mean of r = 0.99) In 13 MS patien…

The Oculo-auricular Phenomenon in Brain-Stem Disease

The oculo-auricular phenomenon (OAP), as described by Wilson in 1908 [26], consists of bilateral coactivation of external ear muscles during lateral gaze. Two ear muscles are innervated by motoneurons of the medial part of the facial nucleus [9, 25]: the transverse auricular muscle (TAM) (Fig. 1), which adducts and curls the helix of the auricle, and the posterior auricular muscle (Fig. 2), which moves the whole auricle backwards [3, 16, 22].

Diffusion weighted magnetic resonance imaging in the diagnosis of reversible ischaemic deficits of the brainstem

Objectives: To evaluate the sensitivity of diffusion weighted magnetic resonance imaging (MRI) for the diagnosis of clinically suspected reversible ischaemic deficits of the brainstem. Methods: A total of 158 consecutive patients presenting with acute signs of brainstem dysfunction were investigated using EPI diffusion weighted MRI within 24 hours of the onset of symptoms. High resolution T1 and T2 weighted imaging was performed as a follow up after a median of six days Results: Fourteen of the 158 patients had a complete clinical recovery within 24 hours (transitory ischaemic attack (TIA)), and 19 patients recovered in less than one week (prolonged reversible neurological deficit (RIND)). …

Dysarthria and Pathological Laughter/Crying as Presenting Symptoms of Corticobasal- Ganglionic Degeneration Syndrome

Isolated voluntary facial paresis due to pontine ischemia

We describe a patient withpatientwith 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 corticolin-gual 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.

Spontanes Liquorunterdrucksyndrom

Wir berichten 11 Patienten mit orthostatischem Kopfschmerz bei spontanem Liquorunterdrucksyndrom (SLUDS). Ubelkeit und Abduzensparesen (bei 3 bzw. 2 Patienten) waren die haufigsten zusatzlichen Symptome. Zehn Patienten hatten eine Pleozytose (6 bis 43 Zellen/μl) und/oder Eiweiserhohung (581 bis 1668 mg/l) im Liquor. Die CT und/oder MRT zeigte bei 5 Patienten bifrontal betonte subdurale Hamatome oder Hygrome, die MRT bei allen 4 untersuchten Patienten eine diffuse Gadolinium-Aufnahme der Meningen, bei einem mit Kaudalverlagerung des Hirntammes. Liquorszintigraphisch (9 untersuchte Patienten) war immer ein fruher Tracer-Nachweis in der Blase und eine verminderte oder fehlende Aktivitat uber d…

Hinweise auf einen gemeinsamen Ursprung einer deutschen und amerikanischen Familie mit hereditärer Amyloidneuropathie Typ II

Die Einteilung der familiaren Amyloidneuropathien (FAP) erfolgte nach regionalen und klinischen Aspekten. In den letzten 10 Jahren haben sich mehr als 40 Punktmutationen auf dem Transthyretin (TTR)-Gen als Ursache fur die FAP und andere Amyloidosen nachweisen lassen. Die FAP II, ausgelost durch eine Punktmutation am Codon 58 des TTR-Gens, ist bislang nur bei zwei Sippen aus Maryland und Ohio in den USA stammend bekannt. Die Erkrankung beginnt im 3.–4. Lebensjahrzehnt mit sensiblen Storungen an den Handen typischerweise im Rahmen eines Karpaltunnelsyndroms. Wir berichten von einer deutschen aus der Rheinregion sudlich von Mainz stammenden Familie mit einer FAP II. Vier Familienmitglieder mit…

Kongenitale Myopathien und Muskelkrankheiten mit speziellen Strukturanomalien

Sammelbezeichnung fur uberwiegend strukturell definierte, haufig erbliche Muskelkrankheiten, die sich oft im fruhen Sauglingsalter unter dem klinischen Bild einer Muskelhypotonie und -schwache sowie einer Abschwachung der Muskeleigenreflexe manifestieren. In vielen Fallen betrifft die Schwache die Rumpf- und proximale Extremitatenmuskulatur; in einigen ist sie generalisiert unter Einschlus der mimischen Muskulatur. Dysmorphiesymptome kommen vor. Die klinische Symptomatik ist in der Mehrzahl der Falle nicht oder nur wenig progredient; maligne Verlaufe kommen vor.

Myopathien bei definierten Stoffwechselstörungen

Befall der Muskulatur bei familiaren sowie sog. primaren und sekundaren Formen der Amyloidose. Klinisch progrediente Schwache und Ermudbarkeit der Muskulatur sowie Verlangsamung der Kontraktion und der Erschlaffung. Aufgrund der Amyloidablagerungen besteht eine Verfestigung der Skelettmuskulatur.

Rhabdomyolyse und Myoglobinurie

Sporadisch, selten auch bei Geschwistern vorkommende Krankheit. Erstmanifestation meist vor dem 25. Lebensjahr, oft nach einem fieberhaften Infekt oder nach korperlicher Belastung. Akutes Auftreten von Muskelschmerzen und partiellen, meist proximal betonten oder generalisierten Lahmungen. Nach Stunden beobachtet man eine Myoglobinurie. Oft ist die Muskulatur verkrampft oder odematos geschwollen. Bei ausgepragter Myoglobinurie ist Nierenversagen moglich. Ruckbildung der Muskelparesen und Myoglobinurie nach Stunden bis Tagen. Nur selten findet man bleibende Muskelschaden.

Increased risk of sensory neuropathy in workers with chloracne after exposure to 2,3,7,8-polychlorinated dioxins and furans

Objective - The existence of a peripheral neuropathy after exposure to polychlorinated dioxins (PCDD) is still discussed, as studies concerning dioxin effects on the peripheral nervous system are rare and contradictory. Material and methods - Clinical and neurophysiological examinations (motor conduction velocity of the peroneal nerve, sensory conduction velocities of the sural and ulnar nerves) were made in 156 dioxin exposed workers (42 with, 114 without cloracne) from one pesticide producing plant. Because of known risk factors for peripheral neuropathy, 7 workers with and 28 without cloracne were excluded from further analysis. Results - Workers with chloracne had a significantly higher…

Influence of artificial blood contamination of the analysis of cerebrospinal fluid

The influence of artificial blood contamination on the quantitative values obtained in the routine examination of the CSF was investigated. On the basis of dilution series from CSF and blood, the correlation between the number of added erythrocytes and the results of leukocyte counts, protein, glucose and IgG estimation was studied. In addition, the influence of the time between CSF sampling and investigation on erythrocytes and leukocytes, IgG, glucose, pH value and ammonia content in CSF contaminated with blood was investigated. The following data relevant to routine examinations resulted: there are linear correlations between the number of erythrocytes artificially added to the CSF and t…

FMRI of the Cerebellum: Rostral Paravermal Activation in Tongue and Lip

We investigated with fMRI the cerebellar representation of articulatory vertical movements of the tongue and orofacial muscles in healthy volunteers and its correspondence to the area known to be affected in cerebellar dysarthria. fMRI stimulation is mainly unilateral in agreement with the frequent occurrence of dysarthria in unilateral infarction.

P14. Impaired balance with brain stem infarcts

Abduction nystagmus in internuclear ophthalmoplegia

Direct current electro-oculography revealed abduction nystagmus with hypermetric abduction saccades in 35 of 64 patients with unilateral and 55 of 66 patients with bilateral internuclear ophthalmoplegia. Slowing of abduction saccades occurred in 27 unilateral cases, mainly ipsilateral to the paretic eye, and in 36 bilateral cases. Abduction nystagmus with hypermetric abduction saccades of normal velocity is explained by an increased phasic innervation adjusted to adduction paresis. Slowed abduction saccades are attributed to impaired inhibition of the medial rectus muscle. Superposition of impaired medial rectus inhibition and increased phasic innervation best explains abduction nystagmus w…

Changes of sensory conduction velocity and refractory periods with decreasing tissue temperature in man.

Changes with temperature of maximum sensory nerve conduction velocity as well as absolute and relative refractory periods were tested in 14 human subjects. Corresponding to previously published findings maximum conduction velocity decreased with cooling following a Q10 of +1.4. The absolute and relative refractory periods were increased by cooling, the Q10 being -3.1 and -3.35 respectively. There was a tendency showing a more pronounced temperature effect at low temperatures. The Q10 and the steepness of the regressionline changed at the level of 26.9 degrees C, but were significant for the relative refractory period only.

The location by early auditory evoked potentials (EAEP) of acoustic nerve and brainstem demyelination in multiple sclerosis (MS).

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 an…

Myositiden bei Gefäß-Bindegewebskrankheiten

Sammelbezeichnung fur Muskelveranderungen vom Typ der „interstitiellen Myositis“ bei Panarteriitis nodosa, Lupus erythematodes, Sklerodermie, rheumatoider Polyarthritis, Wegener-Granulomatose, Sjogren-Syndrom (Sicca-Komplex) u. a.

Intermittent Horner's syndrome on alternate sides: a hint for locating spinal lesions.

A patient with a cervical intramedullary ependymoma is described who exhibited a Horner's syndrome on alternate sides. Pharmacological study demonstrated damage to the pre-ganglionic sympathetic neurons. Disregarding cases with the Shy-Drager syndrome, three comparable case reports have been found in the literature where the fluctuating expression of Horner's syndrome, alternating from one side to the other, was attributable to a lesion of the cervical spinal cord. In contrast to the present patient, the pharmacological responses of these patients indicated a lesion of the central sympathetic pathway.

1.2. 3-D brainstem mapping

Impaired cortico-bulbar tract function in dysarthria due to hemispheric stroke. Functional testing using transcranial magnetic stimulation.

We investigated cortico-lingual and cortico-orofacial tract function utilizing transcranial magnetic stimulation in 18 consecutive patients with dysarthria due to hemispheric stroke. Delayed responses (conduction time > mean + 2.5 SD of that of 43 controls) or absent responses were considered abnormal. In all patients, motor-cortex stimulation of the lesion side demonstrated absent (13 patients) or delayed (five patients) responses to the tongue bilaterally (17 patients) or unilaterally (one patient). In 14 patients the contralateral orofacial responses were either absent (13 patients) or delayed (one patient). According to the electrophysiological findings, all lesions revealed by CT or MR…

Perceptual correlates of nociceptive long-term potentiation and long-term depression in humans.

Long-term potentiation (LTP) and long-term depression (LTD) of synaptic strength are ubiquitous mechanisms of synaptic plasticity, but their functional relevance in humans remains obscure. Here we report that a long-term increase in perceived pain to electrical test stimuli was induced by high-frequency electrical stimulation (HFS) (5 × 1 sec at 100 Hz) of peptidergic cutaneous afferents (27% above baseline, undiminished for >3 hr). In contrast, a long-term decrease in perceived pain (27% below baseline, undiminished for 1 hr) was induced by low-frequency stimulation (LFS) (17 min at 1 Hz). Pain testing with punctate mechanical probes (200 μm diameter) in skin adjacent to the HFS–LFS con…

Myopathien bei endokrinen Störungen

Meist leichte proximale →Myopathie mit generalisierter Muskelschwache, Muskelhypertrophie und vorzeitige Ermudbarkeit. Eine Kombination neurogener und myogener Veranderungen bei hypophysarem Gigantismus wurde beschrieben.

Different short-term modulation of cortical motor output to distal and proximal upper-limb muscles during painful sensory nerve stimulation

The pattern of upper-limb muscle activation following painful stimulation has not been clarified in detail. We investigated the short-term inhibitory and excitatory effects of painful electrical digital stimulation on the motoneuron pools of distal and proximal upper-limb muscles. Transcranial magnetic stimulation (TMS) was used as test stimulus, and painful digital nerve stimulation as conditioning stimulus for motor evoked potential (MEP) recordings over the abductor digiti minimi (ADM), abductor pollicis brevis (APB), biceps brachii (BB), and deltoid muscles. Inhibition of the conditioned MEP response was most prominent in the distal muscles, whereas BB and deltoid muscles were only weak…

„Phobischer Schwankschwindel”

Due to the results of an interdisciplinary study on patients with vertigo as the chief symptom and on the background of psychodynamic theories concerning anxiety disorders the term of phobic postural vertigo (Brandt & Dieterich 1986) is discussed. It becomes obvious that phobic postural vertigo is a generalizing term which encompasses different forms of psychogenic vertigo. The authors plead for a more differentiated diagnosis and subgroup oriented classification of vertigo caused by psychiatric disorders.

Corticobulbar tract involvement in amyotrophic lateral sclerosis. A transcranial magnetic stimulation study

We investigated corticobulbar tract function by recording from the tongue and orofacial muscles and using transcranial magnetic stimulation (TMS) in 30 patients with amyotrophic lateral sclerosis (ALS) in order to improve the diagnostic yield in the detection of subclinical upper motor neuron (UMN) dysfunction. A UMN lesion was assumed when the peripheral conduction time and amplitude of the M-wave were within normal range and either the response to cortical stimulation was absent, or the central conduction time was delayed (> mean + 2.5 SD). Only two patients showed clinical evidence of UMN involvement in the cranial nerves, while TMS demonstrated corticobulbar tract dysfunction in the oro…

Ipsilateral facial weakness in upper medullary infarction-supranuclear or infranuclear origin?

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.

Wichtige Symptome und pathophysiologische Begriffe

Verzogerte Erschlaffung der Muskulatur nach willkurlich, elektrisch oder mechanisch ausgeloster Muskelkontraktion. Muskelaktivitat verringert die Myotonie meistens (Ubungseffekt).

P24. Classical brain stem syndromes: Myth or reality?

Left-hemispheric dominance for articulation: a prospective study on acute ischaemic dysarthria at different localizations.

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 fea…

Idiopathic trigeminal sensory neuropathy with gadolinium enhancement in the cisternal segment

The authors report two patients with idiopathic trigeminal sensory neuropathy who showed gadolinium enhancement of the cisternal segment of the corresponding trigeminal nerve in cranial MRI. The resolution of these lesions in a repeat MRI suggests a similarity to Bell's palsy.