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Biomedical subjects

W Huk

Publications and source records attributed to W Huk.

At least 19 recordsLinked to original sources
PubMed1999

Surgical treatment of craniopharyngiomas: experience with 168 patients.

OBJECT: The goal of this study was to assess the outcome of surgical management in 168 consecutive patients harboring craniopharyngiomas treated between January 1983 and April 1997. METHODS: In 148 patients undergoing initial (primary) surgery, the pterional approach was most frequently used (39.2%), followed by the transsphenoidal approach (23.6%). For large retrochiasmatic craniopharyngiomas, the bifrontal interhemispheric approach was used increasingly over the pterional approach and led to improved surgical results. Total tumor removal was accomplished in 45.7% of transcranial and 85.7% of transsphenoidal procedures. The main reasons for incomplete removal were attachment to and/or infiltration of the hypothalamus, major calcifications, and attachment to vascular structures. The success rate in total tumor removal was inferior in the cases of tumor recurrence. The operative mortality rate in transcranial surgery was 1.1% in primary cases and 10.5% in cases of tumor recurrence. No patient died in the group that underwent transsphenoidal surgery. The rate of recurrence-free survival after total removal was 86.9% at 5 years and 81.3% at 10 years. In contrast, the 5-year recurrence-free survival rate was only 48.8% after subtotal removal and 41.5% after partial removal. Following primary surgery, the actuarial survival rate was 92.7% at 10 years, with the best results after complete tumor removal. At last follow up, 117 (79%) of 148 patients who underwent primary surgery were independent and without impairment. CONCLUSIONS: Total tumor removal while avoiding hazardous intraoperative manipulation provides favorable early results and a high rate of long-term control in craniopharyngiomas.

Adolescent↗
PubMed1998

Ichthyosis follicularis, alopecia, and photophobia (IFAP) syndrome: clinical and neuropathological observations in a 33-year-old man.

The syndrome of ichthyosis follicularis, alopecia, and photophobia (IFAP) is an uncommon neuroichthyosis described in only 10 males so far. We report on a man with congenital ichthyosis and alopecia with apparently normal development in early infancy. Photophobia and generalized myoclonicastatic seizures began during or after the first year of age and were associated with progressive impairment of motor skills and mental abilities. He died at 33 years of age. Neuropathological findings showed an unusual deformation of the temporal lobes and olivocerebellar atrophy. Cytogenetic and molecular studies did not uncover deletions in either Xp22.2 to 3 or in Xq27.3 to qter.

Abnormalities, Multiple↗
PubMed1998

Intraoperative magnetic resonance imaging with the magnetom open scanner: concepts, neurosurgical indications, and procedures: a preliminary report.

OBJECTIVE: Intraoperative magnetic resonance imaging (MRI) is now available with the General Electric MRI system for dedicated intraoperative use. Alternatively, non-dedicated MRI systems require fewer specific adaptations of instrumentation and surgical techniques. In this report, clinical experiences with such a system are presented. METHODS: All patients were surgically treated in a "twin operating theater," consisting of a conventional operating theater with complete neuronavigation equipment (StealthStation and MKM), which allowed surgery with magnetically incompatible instruments, conventional instrumentation and operating microscope, and a radiofrequency-shielded operating room designed for use with an intraoperative MRI scanner (Magnetom Open; Siemens AG, Erlangen, Germany). The Magnetom Open is a 0.2-T MRI scanner with a resistive magnet and specific adaptations that are necessary to integrate the scanner into the surgical environment. The operating theaters lie close together, and patients can be intraoperatively transported from one room to the other. This retrospective analysis includes 55 patients with cerebral lesions, all of whom were surgically treated between March 1996 and September 1997. RESULTS: Thirty-one patients with supratentorial tumors were surgically treated (with navigational guidance) in the conventional operating room, with intraoperative MRI for resection control. For 5 of these 31 patients, intraoperative resection control revealed significant tumor remnants, which led to further tumor resection guided by the information provided by intraoperative MRI. Intraoperative MRI resection control was performed in 18 transsphenoidal operations. In cases with suspected tumor remnants, the surgeon reexplored the sellar region; additional tumor tissue was removed in three of five cases. Follow-up scans were obtained for all patients 1 week and 2 to 3 months after surgery. For 14 of the 18 patients, the images obtained intraoperatively were comparable to those obtained after 2 to 3 months. Intraoperative MRI was also used for six patients undergoing temporal lobe resections for treatment of pharmacoresistant seizures. For these patients, the extent of neocortical and mesial resection was tailored to fit the preoperative findings of morphological and electrophysiological alterations, as well as intraoperative electrocorticographic findings. CONCLUSION: Intraoperative MRI with the Magnetom Open provides considerable additional information to optimize resection during surgical treatment of supratentorial tumors, pituitary adenomas, and epilepsy. The twin operating theater is a true alternative to a dedicated MRI system. Additional efforts are necessary to improve patient transportation time and instrument guidance within the scanner.

Adolescent↗
PubMed1997

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↗
PubMed1994

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↗
PubMed1994

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↗
PubMed1993

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↗
PubMed1993

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↗
PubMed1991

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↗
PubMed1991

[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↗
PubMed1991

[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↗
PubMed1990

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↗
PubMed1990

[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↗
PubMed1990

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↗
PubMed1990

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↗