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Biomedical subjects

W Waespe

Publications and source records attributed to W Waespe.

At least 19 recordsLinked to original sources

[Herpes-simplex encephalitis: case example, diagnosis and therapy].

Herpes simplex encephalitis (HSE) is the most common nonepidemic cause of acute viral encephalitis. Since successful therapy depends on a high level of suspicion that HSE is present and on the early administration of antiviral treatment, knowledge of clinical and laboratory findings of HSE is of great importance. The clinical hallmark of HSE are signs of both focal and diffuse neurologic involvement. Our case report exemplifies the diagnostic problems that can occur in HSE-patients. The validity of the different ancillary examinations is discussed. Up to the present time brain biopsy has been the method of choice for a reliable early diagnosis of HSE. In the foreseeable future early diagnosis is likely to become available in a non-invasive way by the polymerase-chain reaction. Immediate antiviral therapy with acyclovir in HSE has proved to be useful in rigorously controlled trials. The clinical picture of the acyclovir-induced encephalopathy represents a disorder that can be probably avoided by means of a sufficient hydration.

Acyclovir

[Functional gait disorders].

The spectrum of hysterical or functional gait disorders in 47 patients is discussed. Some patients experience a single episode or a transient monoparesis of one leg, but there are patients who are left with a chronic paraplegia. To characterize neurological symptoms for which no organic origin can be demonstrated, we prefer the term "functional". The reasons for using this term are discussed.

Adult

Firing characteristics of vestibular nuclei neurons in the alert monkey after bilateral vestibular neurectomy.

After destruction of the peripheral vestibular system which is not activated by moving large-field visual stimulation, not only labyrinthine-ocular reflexes but also optokinetic-ocular responses related to the "velocity storage" mechanism are abolished. In the normal monkey optokinetic-ocular responses are reflected in sustained activity changes of central vestibular neurons within the vestibular nuclei. To account for the loss of optokinetic responses after labyrinthectomy, inactivation of central vestibular neurons consequent on the loss of primary vestibular activity is assumed to be of major importance. To test this hypothesis we recorded the neural activity within the vestibular nuclear complex in two chronically prepared Rhesus monkeys during a period from one up to 9 and 12 months after both vestibular nerves had been cut. The discharge characteristics of 829 cells were studied in relation to eye fixation, and to a moving small and large (optokinetic) visual stimulus producing smooth pursuit (SP) eye movements and optokinetic nystagmus (OKN). Units were grouped into different subclasses. After chronic bilateral vestibular neurectomy (BVN) we have found: (1) a rich variety of spontaneously active cells within the vestibular nuclear complex, which--as far as comparison before and after BVN is possible--belong to all subclasses of neurons functionally defined in normal monkey; and (2) no sustained activity changes which are related to the activation of the "velocity storage" mechanism; this is especially true for "pure-vestibular", "vestibular-pause" and "tonic-vestibular-pause" cells in normal monkey which show a "pure", "pause" and "tonic-pause" firing pattern after BVN. Neurons which are modulated by eye position are, however, modulated with the velocity of slow eye movements with comparable sensitivity during SP and OKN. Retinal slip is extremely rarely encoded. The results of the present study do not directly answer the question why the "velocity storage" mechanism is abolished after BVN but they suggest that only a small number of central vestibular cells may be inactivated by neurectomy.

Animals

Habituation and adaptation of the vestibuloocular reflex: a model of differential control by the vestibulocerebellum.

We habituated the dominant time constant of the horizontal vestibuloocular reflex (VOR) of rhesus and cynomolgus monkeys by repeated testing with steps of velocity about a vertical axis and adapted the gain of the VOR by altering visual input with magnifying and reducing lenses. After baseline values were established, the nodulus and ventral uvula of the vestibulocerebellum were ablated in two monkeys, and the effects of nodulouvulectomy and flocculectomy on VOR gain adaptation and habituation were compared. The VOR time constant decreased with repeated testing, rapidly at first and more slowly thereafter. The gain of the VOR was unaffected. Massed trials were more effective than distributed trials in producing habituation. Regardless of the schedule of testing, the VOR time constant never fell below the time constant of the semicircular canals (approximately 5 s). This finding indicates that only the slow component of the vestibular response, the component produced by velocity storage, was habituated. In agreement with this, the time constant of optokinetic after-nystagmus (OKAN) was habituated concurrently with the VOR. Average values for VOR habituation were obtained on a per session basis for six animals. The VOR gain was adapted by natural head movements in partially habituated monkeys while they wore x 2.2 magnifying or x 0.5 reducing lenses. Adaptation occurred rapidly and reached about +/- 30%, similar to values obtained using forced rotation. VOR gain adaptation did not cause additional habituation of the time constant. When the VOR gain was reduced in animals with a long VOR time constant, there were overshoots in eye velocity that peaked at about 6-8 s after the onset or end of constant-velocity rotation. These overshoots occurred at times when the velocity storage integrator would have been maximally activated by semicircular canal input. Since the activity generated in the canals is not altered by visual adaptation, this finding indicates that the gain element that controls rapid changes in eye velocity in the VOR is separate from that which couples afferent input to velocity storage. Nodulouvulectomy caused a prompt and permanent loss of habituation, returning VOR time constants to initial values. VOR gain adaptation, which is lost after flocculectomy, was unaffected by nodulouvulectomy. Flocculectomy did not alter habituation of the VOR or of OKAN. Using a simplified model of the VOR, the decrease in the duration of vestibular nystagmus due to habituation was related to a decrement in the dominant time constant of the velocity storage integrator (1/h0).(ABSTRACT TRUNCATED AT 400 WORDS)

Acclimatization

Anterior spinal artery syndrome of the cervical hemicord.

Three patients developed signs of a unilateral cervical cord lesion 6 to 36 h after the acute onset of severe cervico-brachial pain. The neurological deficit progressed over 6 to 18 h. On the painful side a central Horner's syndrome, a hemiparesis with plegia of the hand, and a slight pallhypaesthesia were found. On the opposite side thermhypaesthesia and hypalgesia were noted with a level at the dermatome C5 or C6. T2-weighted MR images revealed in one patient a small area of increased signal intensity restricted to one half of the cervical cord, and electromyography in another patient showed after 6 months evidence of segmental chronic denervation. Both abnormalities were found at the clinically expected level. The findings are consistent with a small infarction of the cervical cord in the perfusion territory of a central (sulco-commissural) artery, a duplicated anterior spinal artery or an anterior spinal branch of the vertebral artery.

Cerebellum

Small infarctions of cochlear, retinal, and encephalic tissue in young women.

BACKGROUND AND PURPOSE: Recently, a rare syndrome that involves uniformly the brain, inner ear, and retina in previously healthy young women has been described. Brain biopsies and ophthalmologic examinations disclosed small infarcts as a pathoanatomical substrate of the disease. In previous reports, an autoimmune disorder or a coagulopathy have been suggested as possible etiologies. CASE DESCRIPTIONS: Both patients (aged 22 and 20 years) had brain involvement with neurological and neuropsychological deficits. Multifocal small hyperintensities were shown in magnetic resonance imaging of the brain. Findings of cerebrospinal fluid examination and electroencephalography were pathological in case 1 and of cerebral angiography in case 2. Both patients had a neurosensory hearing loss and multiple retinal branch arteriolar occlusions. Both women were on fenfluramine before onset of the disease. In case 1, attacks recurred during a follow-up of 34 months. At onset of the disease the 5-hydroxyindoleacetic acid and homovanillic acid levels of the cerebrospinal fluid were reduced; 13 months later the 5-hydroxyindoleacetic acid level was still reduced and the homovanillic acid level was low-normal. In case 2, with the longest follow-up of 13 years, the disease was active during only the initial 2 1/4 years. During this period a combination of oral anticoagulant and antiplatelet agents was ineffective. CONCLUSIONS: Our findings could not support current etiologic hypotheses. Whether changes in 5-hydroxyindoleacetic acid and homovanillic acid levels in the cerebrospinal fluid and/or fenfluramine intake play a role in the pathogenesis of the disease remains to be elucidated.

Adult

Enduring dysmetria and impaired gain adaptivity of saccadic eye movements in Wallenberg's lateral medullary syndrome.

Saccadic eye movements and the adaptive control of their amplitudes were examined in patients with Wallenberg's lateral medullary syndrome. Half of the patients had permanent saccadic dysmetria. Their primary saccades had asymmetric amplitudes: those made in response to an ipsilateral target step (i.e. to the lesion side) tended to be hypermetric and saccades made in response to a contralateral target step were strongly hypometric. Multiple correction saccades were needed for target fixation. The adjustment of the amplitude of artificially induced hypermetric saccades, called gain adaptivity, was examined experimentally by using double target steps. The first target step elicited the primary saccade which triggered a further target displacement. This second, intra-saccadic target displacement was opposite to the first target step and caused the primary saccade to overshoot the final target position. In this way a post-saccadic target position error was generated which had to be corrected for foveal fixation. With repetition of this stimulus sequence the saccadic control system of normal subjects made an adjustment in amplitude of the main saccade such that the overshooting gradually diminished. After a few hundred trials primary saccades became orthometric with respect to the final target position; in respect to the first target step they were, however, strongly hypometric. The experimental data show that patients with Wallenberg's syndrome had a reduced capability to readjust saccadic amplitude. This observation together with the enduring saccadic dysmetria suggest that adaptive gain control of saccades is impaired in patients with lesions restricted to the dorsolateral medulla. It is speculated that these lesions most likely disrupt olivo-cerebellar pathways which are believed to be of paramount importance in visuo-motor adaptation of the cerebellum.

Adaptation, Physiological

[Cerebrotendinous xanthomatosis].

Cerebrotendinous xanthomatosis (CTX) is a rare lipid storage disorder due to an autosomal-recessive inherited defect of the hepatic mitochondrial steroid 26-hydroxylase. The resultant reduced biosynthesis of cholic and especially chenodeoxycholic acid and the increased production and accumulation of cholestanol and cholesterol in most tissues is described and pathogenetic aspects as well as typical pathological findings are discussed. In the light of three personal observations the clinical symptoms and the results of auxiliary investigations are discussed and compared with the literature. The suspected diagnosis of CTX may be confirmed by demonstration of a pathologically elevated concentration of cholestanol or biliary alcohols in serum and urine respectively. The chronically progressive neurologic deficit can be halted or is in some cases partially reversible by treatment with chenodeoxycholic acid. Therefore, early diagnosis is mandatory and CTX should be considered in every patient presenting with intellectual impairment, spastic-ataxic signs, juvenile cataracts and tendon xanthomas.

Achilles Tendon

[Fever, headache and paralysis of the left leg].

A 17 year old man was hospitalized because of fever, headache and a paresis of his left leg. Radiologic findings demonstrated a subdural interhemispheric empyema on the right side as a complication of ipsilateral pansinusitis. Streptococcus milleri was cultured as the only pathogen from maxillary sinus suppuration. Pathogenesis and therapy of subdural empyema are discussed. Cure was achieved with ceftriaxone, flucloxacilline and ornidazole during one week followed by ceftriaxone as monotherapy during further five weeks. The importance of streptococcus milleri as causing agent of purulent lesions in internal organs is stressed.

Adolescent

[Triplegia in bilateral anterior-media watershed infarcts].

Deterioration of a neurologic deficit was observed in a 47-year-old patient with high-grade bilateral internal carotid artery stenoses. She presented monoplegia of the right leg with progression to triplegia (bicrural and left arm) following antihypertensive treatment of a suspected "hypertensive crisis". Triplegia is a very rare syndrome and highly suggestive of bilateral pathology of the carotid arteries. Antihypertensive treatment is contraindicated because sufficient blood pressure is needed for adequate perfusion of the brain, especially in the border zones between the great cerebral arteries. Allowing a high blood pressure and low head positioning led to slow recovery from the neurologic deficit, which continued after bilateral carotid endarterectomy. The patient became ambulatory without assistance and with minimal residual paraspasticity. Triplegia and other patterns of motor deficit in cerebrovascular disorders are discussed and the clinical picture of hypertensive encephalopathy is reviewed.

Arm

Long-term plasma exchange in a case of Refsum's disease.

Refsum's disease (Heredopathia atactica polyneuritiformis) is caused by accumulation of phytanic acid in all body tissues due to an inherited failure of alpha-oxidation of branched chain fatty acids. Plasmapheresis has been reported to be beneficial by removal of phytanic acid from the blood. We describe a patient with Refsum's disease in whom long-term plasmapheresis did not improve clinical, biochemical or electrophysiological parameters.

Adult

[Leukoencephalopathy following inhalation of heroin pyrolysate].

Inhalation of pre-heated heroin ("chinese blowing") is known to cause a spongiform leukoencephalopathy with marked neurological deficits. We report on 2 patients who developed severe cerebellar symptoms several days after interruption of heroin inhalation. The MRI findings suggested myelin damage to fibre tracts in the central nervous system, which are specifically involved in this disorder. Both patients survived with severe cerebellar deficits.

Administration, Inhalation

[Imminent paraplegia].

Acute and subacute paraparesis are discussed, using case reports. Some important anamnestic, anatomic and clinical investigational features to be considered in patients with lesions of the spinal cord are emphasized. Most frequent and for the practitioner most important are lesions due to compression, mainly localized in the thoracic cord. Such lesions need rapid evaluation.

Adult

[Neurosyphilis 1982-1989].

We report the signs, symptoms, serological and CSF findings in 27 patients with syphilis and inflammatory involvement of the central nervous system. In 24 patients the neurosyphilis was symptomatic. Most patients did not report previous signs of early syphilis. A CSF pleocytosis was present in 25 patients (greater than 90%) and the CSF VDRL was reactive with a titer of 1:2 or above in 19 patients (70%). The symptomatology seemed not to be atypical for neurosyphilis although a precise classification was often not possible and the diagnosis was seldom suspected on clinical grounds. In most patients the clinician can monitor the effectiveness of antibiotic therapy by simple laboratory parameters which reflect the activity of the inflammatory process. It is disquieting, however, to note how inconsistently this follow-up was performed.

Adult

Oculomotor disturbances during visual-vestibular interaction in Wallenberg's lateral medullary syndrome.

Transient and lasting oculomotor disturbances during visual-vestibular interaction are described in 9 patients with Wallenberg's lateral medullary syndrome. In all patients magnetic resonance imaging (MRI) demonstrated a single focal area of pathological signal intensity in the (dorso)-lateral medulla suggesting infarction. In 2 of these 9 patients and in 3 further patients with no medullary signs, the infarction involved the cerebellar territory of the posterior inferior cerebellar artery (PICA). Acutely, all patients with Wallenberg's syndrome (except 1) had saccadic lateropulsion and spontaneous nystagmus in light with the horizontal fast component beating to the contralateral normal side. The velocity of the slow drift to the side of the lesion was dependent on eye position and induced a characteristic asymmetry of the visually and vestibularly elicited slow eye movements. In most patients smooth pursuit, optokinetic nystagmus and visual suppression of the vestibulo-ocular reflex were still impaired when this spontaneous drift was minimal or absent. The oculomotor disturbances in patients with and without cerebellar infarction are compared. The following conclusions are made. (1) The spontaneous drift that is dependent on eye position is mostly created by 'ocular lateropulsion', that is, a tonic bias within the oculomotor system which may have several sources. (2) The abnormalities and asymmetries of oculomotor responses during visual-vestibular stimulation cannot solely be explained by this spontaneous drift and its interaction with otherwise normal eye movements. Instead, structures and pathways are damaged in Wallenberg's syndrome which mediate visual and/or motor signals important for the cerebellar control of visually-guided slow eye movements. (3) Damage to these pathways occurs in the lateral medulla, as the MRI findings show that in most patients the cerebellum is rarely involved, but no definite conclusion can be made as to which of the fibres travelling in the inferior peduncle to the cerebellum may be interrupted.

Adult

The velocity response of vestibular nucleus neurons during vestibular, visual, and combined angular acceleration.

In alert Rhesus monkeys neuronal activity in the vestibular nuclei was measured during horizontal angular acceleration in darkness, acceleration of an optokinetic stimulus, and combined visual-vestibular stimulation. The working ranges for visual input velocity and acceleration extend up to 60 degrees/s and 5 degrees/s2. The corresponding working range for vestibular input acceleration is wider and time-dependent. During combined stimulation, that is acceleration of the monkey in the light, a linear relation between neuronal activity and velocity could be established for all neurons. Type I vestibular plus eye movement neurons displayed the greatest sensitivity and had a small linear range of operation. Other vestibular neurons were less sensitive but had a larger range of linear response to different values of acceleration. Accelerating the animal and visual surround, simultaneously but in opposite directions, results in neuronal activity proportional to relative velocity over a limited range.

Acceleration