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Biomedical subjects

W Huk

Publications and source records attributed to W Huk.

At least 19 recordsLinked to original sources

Open surgery of giant paraclinoid aneurysms improved by intraoperative angiography and endovascular retrograde suction decompression.

In three consecutive cases of giant left sided paraclinoid aneurysms we employed an endovascular retrograde suction decompression technique in combination with intra-operative angiography. A double-lumen balloon catheter was placed in the left internal carotid artery by the transfemoral route. After balloon inflation and placement of a temporary clip distal to the aneurysm blood was aspirated and the aneurysm collapsed. Thus further dissection of the aneurysm could easily be achieved and clips could be placed. Afterwards real-time digital subtraction angiography was performed. Intra-operative angiography led to clip repositioning in all cases either due to a clip induced stenosis of the parent vessel, or because of incomplete aneurysm obliteration. Afterwards successful clipping could be confirmed in all cases. Outcome was excellent in one case, good in the other. The third case, extremely complicated by an accompanying craniopharyngioma, showed a satisfactory outcome, but presented new neurological deficits.

Cerebral Angiography

Posterior fossa neurovascular anomalies in essential hypertension.

Intraoperative observations, necropsy, and angiographic studies support the presumption that neurovascular compression of the left ventrolateral medulla may cause neurogenic hypertension. Pulsatile irritation of the ventrolateral medulla at the root-entry zone of cranial nerves IX and X increases blood pressure in animals. To identify and assess the distribution of neurovascular compression at the ventrolateral medulla in human beings, we did a prospective single-blind study in 24 patients with essential hypertension, in 14 patients with renal hypertension, and in 14 normal subjects. To detect neurovascular compression, we used axial and coronal double-echo and magnetic-resonance angiography sequences. Blood pressure control and duration of hypertension were not different in the two groups of patients. 20 patients with essential hypertension had magnetic tomographic evidence of left-sided neurovascular compression at the ventrolateral medulla; 2 patients with renal hypertension and 1 of the normal subjects had a positive finding on the left. On the right side, we found signs of neurovascular compression in 4 patients with essential hypertension, in 4 with renal hypertension, and in 2 of the normal subjects. With magnetic resonance tomography, it is possible to evaluate the neurovascular relations in the posterior fossa and detect neurovascular compression at the ventrolateral medulla. These data in living subjects give further evidence of an association between neurovascular compression at the left ventrolateral medulla and essential hypertension.

Adult

Genetic counseling of cleft sternum.

Cleft sternum is a rare malformation, either superior, inferior or total, which is frequently associated with supraumbilical raphé. Such a case is reported. Sometimes cavernous hemangiomata are noted. It occurs subsequent to disturbances of abdominal wall midline fusion. The etiology is unknown. The recurrence risk is probably nil, but one familial case must be taken into consideration.

Adult

QUEST--a quick echo split NMR imaging technique.

A novel millisecond NMR imaging method is introduced, which generalizes the principle of stopped-pulse experiments. It has been dubbed QUEST for QUick Echo Split imaging Technique. Repeated dephasing and excitation is used to divide a primary free induction decay into an exponentially growing number of echoes. Very few gradient lobes and RF pulses already generate large numbers of echoes, so neither gradient switching speed nor RF absorption represents any real limitation. True spin echoes are easily produced.

Brain

Hydantoin syndrome with holoprosencephaly: a possible rare teratogenic effect.

We report on a newborn girl with lobar holoprosencephaly and clinical signs of hydantoin syndrome. During pregnancy the mother was treated with diphenylhydantoin and primidon for psychomotor and petit mal seizures. The possible relationship between hydantoin syndrome, holoprosencephaly, and maternal intake of anticonvulsants during pregnancy is discussed.

Abnormalities, Drug-Induced

Pontine-mesencephalic cavernomas: indications for surgery and operative results.

Cavernous haemangiomas cavernomas of the brainstem can be diagnosed by MRI and safely removed when elective surgery is performed in the subacute stage after haemorrhage. Recurrent haemorrhage and/or neurological deterioration are indications for surgery. In cases with additional venous malformation the cavernoma should be selectively removed. The experiences with our own series of 18 cases are presented.

Adolescent

[4-Tesla 1H MR spectroscopy in patients with temporal lobe epilepsy].

7 patients with drug-resistant temporal lobe epilepsy (TLE) and localized EEG-focus were investigated with a 4 Tesla whole body MR-scanner. Proton (1H) magnetic resonance (MR) spectra were analyzed quantitatively and compared to the healthy side. MRS allowed the differentiation of the following metabolites in 5 patients: N-acetyl-aspartate (NAA), creatine and phosphocreatine, phosphorylcholine and glycerophosphorylcholine, beta- and gamma-glutamate (GLU). To compare the results with those of an already evaluated normal population, these metabolites were measured also in parietal region. The standard deviation was 42-46% in the patients. Unfortunately, in the temporal region, the field homogeneity was worse than parietal and thus the spectral analysis less distinct especially for GLU with a standard deviation of 45% for NAA and 66% for GLU on the healthy side. Thus, no significant findings were seen on focus side. There was only a tendency to an elevation of glutamate and a reduction of N-acetyl-aspartate.

Adolescent

[Quantitative and qualitative MRI changes in temporal lobe epilepsy. A comparison with normal parameters of nuclear magnetic resonance tomography].

Magnetic resonance imaging (MRI) often reveals no pathology in patients with temporal lobe epilepsy (TLE) who are candidates for surgery, even though post-surgery histology reveals atrophy or sclerosis. To differentiate these pathologies in MRI, we performed a standardized acquisition and a quantitative analysis of MRI data (qMRI). The standard values of 43 healthy subjects (both sex, left and right handed) were compared with those of 10 patients with TLE and unilateral EEG focus. A reduced estimated total brain extension, a significant atrophy of both temporal lobes with accentuation on the focus side and a significant hippocampal atrophy on focus-side were seen even in patients with no visually detectable pathology. The analysis of the signal intensities did not give additional information. Thus, qMRI is a helpful additional tool in the course of presurgical evaluation for focus lateralization.

Adolescent

Fatal complications after myelography with meglumine diatrizoate.

A case of inadvertent intrathecal injection of diatrizoate meglumine is presented. After myelography with 10 ml i.e. 6.5 g Angiografin, a 76-year-old man rapidly developed myoclonus, drowsiness and excessive metabolic acidosis. He died only a few hours later. Postmortem showed non-specific brain edema. RP-HPL-Chromatography confirmed high concentration of the contrast medium in CSF (6 mg/ml) which must have induced refractory central nervous dysregulation. The lethal effects of the misapplication of this agent on the nervous system are discussed.

Aged

[Diagnostic differentiation of dementia diseases by modern imaging procedures].

We present a review on recent neuroimaging techniques, like x-ray computed tomography (XCT), magnetic resonance imaging (MRI), positron emission tomography (PET) and single photon emission tomography (SPECT) in dementia and related diseases. Significant new findings have been obtained using techniques reflecting proton density, regional brain perfusion and brain metabolism. In dementia of the Alzheimer type, for example, temporoparietal and sometimes also frontal reductions in cerebral blood flow and metabolism are characteristic. The infarctions found in multi-infarct dementia are especially well visualized on T2-weighted MRI images. Pick's disease is characterized by brain atrophy and decrease of radiotracer activity in the frontal lobes. In huntington's chorea the metabolic rate on PET scan in the area of the caudate nuclei may be reduced even before signs and symptoms become apparent. Furthermore, neuroimaging provides us with fairly typical finding in Creutzfeld-Jakob's disease, alcoholic dementia, Wilson's disease, hydrocephalus, Parkinson's disease, progressive supranuclear ophthalmoplegia, Fahr's disease, and the olivopontocerebellar ataxias. Neuroimaging techniques, however, have always to be interpreted in conjunction with clinical findings, thus disclosing their full range of information.

Brain

Surgical removal of pontomesencephalic cavernous hemangiomas.

Cavernous hemangiomas of the brain stem are usually discovered accidentally during evacuation of a hematoma, and successful surgical treatment of these lesions is seldom achieved. With the increasing use of magnetic resonance imaging, the presence of a cavernous hemangioma can be detected before surgery, allowing an elective surgical approach. We successfully removed pontomesencephalic cavernous hemangiomas from 2 patients and pontomedullary hemangiomas from 2 others. Elective surgery was performed with perioperative bimodal monitoring of somatosensory and auditory evoked potentials. Performing surgery soon after the hemorrhage minimizes the risk of additional postoperative neurological deficit, since surgical excision is facilitated when the hematoma is not completely organized. Pontine hemangiomas are approached via the 4th ventricle. Mesencephalic hemangiomas are removed by a midline supracerebellar approach when they are lateralized by using a subtemporal approach. The lesion can be removed through a small incision in the brain stem at the site of the lesion. The favorable results, which include marked improvement of preoperative neurological deficits and documentation of complete removal of the lesion by magnetic resonance imaging, support a more aggressive approach to the treatment of symptomatic cavernous hemangiomas of the brain stem. Further investigation of the natural history of these lesions is mandatory.

Adolescent

Hypomelanosis of Ito (incontinentia pigmenti achromians). Ophthalmological evidence for somatic mosaicism.

The authors report on a ten-year-old boy with hypomelanosis of Ito. He suffered from epileptic seizures and exhibited typical generalized partial skin hypomelanosis in whorl-like and striated pattern following Blaschko's lines. The fundi showed patchy, mottled hypopigmentations becoming increasingly striated in the periphery with a general orientation to the optic nerve head. This pattern of affection reminds of the retinal findings in carrier women for X-linked ocular albinism. Magnetic resonance imaging revealed multiple small areas of increased relaxation time scattered in the white matter of the brain, which are interpreted as porencephalic cysts. These clinical findings suggest somatic cell mosaicism even though the cytogenetic study was not conclusive.

Brain Diseases

Fat deposition surrounding intracerebral hemorrhage in a patient suffering from Zieve syndrome.

In a 42-year-old man, admitted a few hours after an acute cerebrovascular event, CT demonstrated a hyperdense hemorrhage surrounded by a hypodense rim similar to perifocal edema or liquefying blood, thus raising doubts about the acuteness of the event. Laboratory findings revealed Zieve-syndrome (alcoholic hyperlipemia, hemolytic anemia, and alcoholic fatty liver) and negative Hounsfield Unit measurement of the hypodense rim finally identified it as a layer of fat around the clot.

Adult

[Borrelia burgdorferi infection with bilateral optic neuritis and intracerebral demyelinization lesions].

In September, 1987, the authors saw a 25-year-old female patient with retinal perivasculitis, cystoid macular edema and papilledema in her right eye. The left eye was normal. Visual acuity was 0.2 (OD), 1.2 (OS). After conventional infections had been ruled out systemic methylprednisolone therapy was instituted, but the patient's condition deteriorated. In May 1988 she presented with papilledema and a "neuroretinitis"-like finding in her left eye; in her right eye advanced optic nerve atrophy; visual acuity was 0.1 (OD) and 0.07 (OS). The laboratory workup revealed an acute phase of a chronic Borrelia burgdorferi infection, with total immunoglobulins (immunofluorescence test) of 1:1280 and an IgM of 1:650 (normal ranges: total Ig up to 1.80, IgM up to 1:40). MRI showed multiple paraventricular and subcortical demyelinating lesions. However, the cranial CT scan was normal. After 14 days' treatment with doxycycline 200 mg/d, visual fields and acuity improved to sc 0.2 (OD) and sc 0.1 (OS) (July, 1988). This case of intracranial demyelinizating lesions associated with bilateral optic neuritis in a serologically determined Borrelia burgdorferi infection is the first of its kind described in the literature.

Adult