Coma due to meteorism and increased intra-abdominal pressure subsequent to ventriculoperitoneal shunt dysfunction.
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Coma↗
Biomedical subjects
Joachim K Krauss
Publications and source records attributed to Joachim K Krauss.
Dystonic movement disorders and spinal degenerative disease.
The occurrence of degenerative spinal disease subsequent to dystonic movement disorders has been neglected and has received more attention only recently. Spinal surgery is challenging with regard to continuous mechanical stress when treatment of the underlying movement disorder is insufficient. To characterize better the particular features of degenerative spinal disease in patients with dystonia and to analyze operative strategies, we reviewed the available published data. Epidemiologic studies reveal that degenerative spinal disorders in patients with dystonia and choreoathetosis occur much earlier than in the physiological aging process. Dystonic movement disorders more often affect the spine at higher cervical levels (C(2-5)), in contrast to spinal degeneration with age which manifests more frequently at the middle and lower cervical spine (C(5-7)). Degenerative changes of the cervical spine are more likely to occur on the side where the chin is rotated or tilted to. Various operative approaches for treatment of spinal pathologies have been advocated in patients with dystonic movement disorders. The available data do not allow making firm statements regarding the superiority of one approach over the other. Posterior approaches were first used for decompression, but additional anterior fusion became necessary in many instances. Anterior approaches with or without instrumented fusion yielded more favorable results, but drawbacks are pseudarthrosis and adjacent-level disease. Parallel to the development of posterior fusion techniques, circumferential surgery was suggested to provide a maximum degree of cord decompression and a higher fusion rate. Perioperative local injections of botulinum toxin were used initially to enhance patient comfort with halo immobilization, but they are also applied in patients without external fixation nowadays. Treatment algorithms directed at the underlying movement disorder itself, taking advantage of new techniques of functional neurosurgery, combined with spinal surgery have recently been introduced and show promising results.
Dystonic Disorders↗
Endoscopic third ventriculostomy in the treatment of shunt-related over-drainage: Preliminary experience with a new approach how to render ventricles navigable.
OBJECTIVE: Endoscopic third ventriculostomy (ETV) is increasingly used for the treatment of shunt-related complications in hydrocephalic patients, particularly if the etiology of the underlying hydrocephalus is of obstructive nature. PURPOSE: Due to the slit-like configuration of the ventricles, ventricular dilatation must be achieved prior to ETV in patients with problems due to over-drainage. This has been accomplished by ligating or explanting the shunt. Here we present an alternative procedure using a gravitational antisiphon device. MATERIALS AND METHODS: In two patients with over-drainage due to shunted occlusive hydrocephalus ventricles were dilated by integrating an antisiphon device (Miethke ShuntAssistant, Aesculap AG, Tuttlingen, Germany) into their shunt systems. The resistance of the antisiphon device, which is available in configurations from 10 to 35 cm H2O was chosen 10 cm H2O higher than necessary to prevent siphoning in the individual patient. RESULTS: Both patients gradually recovered from their over-drainage symptoms and the ventricles enlarged enough to allow access with an endoscope. Using a standard procedure, ETV was performed 7 days and 1 month later, respectively. In the same operative session the shunts were occluded. Shunts were removed within 6 weeks after ETV. During follow-up of more than 3 years, both patients remained free of symptoms. CONCLUSION: The incorporation of an antisiphon device with resistance level selected 10 cm H2O higher than needed to prevent anti-siphoning into a pre-existing shunt system in patients suffering from shunt-related over-drainage is a safe and effective technique to render ventricles large enough to allow endoscopic access for ETV.
Adult↗
Posttraumatic peripherally-induced dystonia and multifocal deep brain stimulation: case report.
OBJECTIVE: We report on the effect of multifocal deep brain stimulation for the treatment of posttraumatic peripherally-induced dystonia. CLINICAL PRESENTATION: A 34-year-old woman presented with an 8-year history of painful tonic dystonia starting in her left leg after injury of the third metatarsal bone. She did not benefit from right-sided pallidal stimulation by an electrode misplaced in the globus pallidus externus in another hospital. INTERVENTION: Quadripolar deep brain stimulation electrodes were placed in the globus pallidus internus and the ventrolateral thalamus by computed tomographic-guided stereotactic surgery and microelectrode recording contralateral to the side of dystonia. The Burke-Fahn-Marsden motor score of 34 did not improve with chronic pallidal or thalamic stimulation. CONCLUSION: Although deep brain stimulation is received with great enthusiasm, it is important to identify its limitations in certain subtypes of dystonia.
Adult↗
A prospective randomized comparison of rectangular titanium cage fusion and iliac crest autograft fusion in patients undergoing anterior cervical discectomy.
OBJECT: The authors compare clinical outcome and fusion rates after iliac crest autograft (ICAG)- and rectangular titanium cage (RTC)-augmented fusion in patients undergoing anterior cervical discectomy (ACD). METHODS: One hundred consecutive patients with 127 levels of cervical disc disease refractory to conservative treatment were randomized into one of the two treatment groups (ICAG/RTC fusion). The visual analog scale was used by the patient to rate overall pain and head, neck, arm, and donor site pain separately. Myelopathy was documented according to Japanese Orthopaedic Association and Nurick grading systems. Outcome was analyzed using Odom criteria, the 36-Item Short Form (SF-36), and Patient Satisfaction Index scales. Fusion rates were assessed on standard and flexion-extension radiographs. Follow-up data of at least 12 months' duration were available for 95 patients. More residual overall pain after 12 months was documented in patients who underwent ICAG fusion (3.3 +/- 2.5 [ICAG] and 2.2 +/- 2.4 [RTC]; p < 0.05). Although arm and head pain were minimal in both groups, neck pain proved to be the predominant symptom (2.7 +/- 2.5 [ICAG] and 1.9 +/- 2.1 [RTC]), which resolved in only 67 and 48% of RTC- and ICAG-treated patients, respectively (p < 0.05). Myelopathy improved comparably in both groups. Regardless of increased pain in ICAG-treated patients, PSI and SF-36 scores were not significantly different between groups (only four [8%] of 47 ICAG-treated patients and five [10%] of 48 RTC-treated patients were unsatisfied). Good to excellent functional recovery according to Odom criteria was observed in 75 and 79% of ICAG- and RTC-treated patients, respectively. Fusion rates were 81 and 74%, respectively (p = 0.51). CONCLUSIONS: Fusion rates and clinical outcome at 12 months after ACD were comparable between patients who underwent ICAG and RTC fusion. The use of rectangular cages, however, avoids donor site morbidity and reduces overall pain and, thus, seems to be an advantageous treatment alternative.
Adult↗
Recurrent sciatica due to periligamentous trapped epidural gas after spinal sequestrectomy.
The authors describe the unusual case of a 50-year-old woman who suffered from sciatic pain due to periligamentous trapped epidural gas after lumbar sequestrectomy. The patient underwent removal of free herniated disc material via a translaminar approach through the L-5 lamina without discectomy. Four days later she suffered from recurrent pain, and neuroimaging studies revealed an epidural gas formation at the site of the sequestrectomy. After evacuation of the gas, her pain resolved. Postoperative intraspinal gas may be symptomatic in the rare case.
Epidural Space↗
Long-term deep brain stimulation in elderly patients with cardiac pacemakers.
OBJECT: Deep brain stimulation (DBS) has become an accepted therapy for movement disorders such as Parkinson disease (PD) and essential tremor (ET), when these conditions are refractory to medical treatment. The presence of a cardiac pacemaker is still considered a contraindication for DBS in functional neurosurgery. The goal of this study was to evaluate the technical and clinical management of DBS for the treatment of movement disorders in elderly patients with cardiac pacemakers. METHODS: Six patients with cardiac pacemakers underwent clinical and cardiac examinations to analyze the safety of DBS in the treatment of movement disorders. Four patients suffered from advanced PD and two patients had ET. The mean age of these patients at surgery was 69.5 years (range 63-79 years). The settings of the pacemakers were programmed in a manner considered to minimize the chance of interference between the two systems. There were no adverse events during surgery. Four patients underwent stimulation of the thalamic ventralis intermedius nucleus (VIM), and two patients stimulation of the subthalamic nucleus. In general, bipolar sensing was chosen for the cardiac pacemakers. In all but one patient the quadripolar DBS electrodes were programmed for bipolar stimulation. Several control electrocardiography studies, including 24-hour monitoring, did not show any interference between the two systems. At the time this paper was written the patients had been followed up for a mean of 25.3 months (range 4-48 months). CONCLUSIONS: In certain conditions it is safe for patients with cardiac pacemakers to receive DBS for treatment of concomitant movement disorders. Cardiac pacemakers should not be viewed as a general contraindication for DBS in patients with movement disorders.
Aged↗
Propriospinal myoclonus due to cervical disc herniation. Case report.
Propriospinal myoclonus is a rare form of spinal myoclonus. In most cases the cause has remained unclear. Secondary propriospinal myoclonus has been described secondary to various disorders including trauma, tumor, and infection. Thus far, propriospinal myoclonus caused by cervical disc herniation has not been reported. In the present report, the authors describe the case of a 53-year-old man who presented with radicular symptoms of the right C-6 nerve root and myoclonic twitches predominantly affecting the abdominal muscles but spreading to adjacent muscles. The spread was triggered and enforced by certain movements. Magnetic resonance imaging studies revealed a C-6 nerve root compression at the C5-6 level on the right side but no cervical myelopathy. Electromyography studies confirmed the diagnosis of propriospinal myoclonus. After discectomy and cage-augmented fusion via an anterior approach, the myoclonic movement disorder gradually subsided. To the authors' knowledge, this is the first report on successful treatment of propriospinal myoclonus by spinal disc surgery.
Cervical Vertebrae↗
Juxtaposition of an epidural intraforaminal cavernous hemangioma and an intradural schwannoma.
STUDY DESIGN: A case of juxtaposition of a purely extradural cavernous hemangioma and an intradural schwannoma in the thoracic spine is reported. OBJECTIVES: The objectives of this study were to present a rare and before-surgery unexpected combination of spinal tumors, which may complicate surgical removal; and to discuss the radiologic features and the possible pathogenesis of this combination of tumors and its implications on surgical therapy. SUMMARY OF BACKGROUND DATA: Juxtaposition of different spinal tumors is exceedingly rare in patients without neurofibromatosis. To the authors' knowledge, no combination of a purely epidural spinal cavernous hemangioma with an intradural schwannoma has been previously reported. MATERIAL AND METHODS: A 47-year-old woman presented with progressive paraparesis. Magnetic resonance imaging revealed a partly cystic intradural mass at the T6-T7 level and a solid extradural intraforaminal component that demonstrated slightly different signal characteristics. An intra-/extradural schwannoma was assumed. RESULTS: Surgical exposure displayed a highly vascularized lobulated mass within the left neuroforamen at T6-T7. There was no continuity with the larger intradural, cystic, and mildly vascularized lesion. Histologic examination diagnosed an extradural cavernous hemangioma and an intradural schwannoma. CONCLUSIONS: The segmental juxtaposition of lesions should be considered in the differential diagnosis of spinal pathology.
Epidural Neoplasms↗
Center median-parafascicular complex and pain control. Review from a neurosurgical perspective.
The center median-parafascicular (CM-Pf) complex, which constitutes the major portion of the intralaminar thalamus in man, has long been known to be involved in the processing of pain under normal and pathological conditions. Yet, these 'forgotten' nuclei with their rich connectivity to other thalamic nuclei, the basal ganglia and cortical areas have received only relatively little attention over the past two decades. With regard to the recent reinterest in functional stereotactic neurosurgery as a treatment option for chronic refractory pain, the CM-Pf complex has been reconsidered as a target. This review provides a systematic overview on the current knowledge about the anatomy and connectivity of the CM-Pf complex, neurophysiological studies, and on concepts of its role in pain processing under various conditions. We also review the previous experience with ablative surgery and deep brain stimulation of the CM-Pf complex. Studies in men and experimental animals indicate that the CM-Pf complex is part of a medial pain system, which appears to be involved primarily in affective and motivational dimensions of pain. Single-unit recordings from the CM-Pf complex have shown that the activity of CM-Pf cells is modified by painful stimuli. Under pathological conditions, bursting firing patterns and altered discharge rates were found. Thalamotomies targeting at the CM-Pf complex yielded beneficial results for chronic pain, but interpretation of the results is limited. With bifocal deep brain stimulation, short-term effects of CM-Pf stimulation were superior to those of somatosensory thalamic stimulation in neuropathic pain. There is evidence, that the CM-Pf complex might also be involved in the mediation of the beneficial effects of somatosensory thalamic stimulation and periventricular grey stimulation.
Animals↗
Dystonia in the Mohr-Tranebjaerg syndrome responds to GABAergic substances.
Mutations in the X-linked deafness-dystonia peptide 1 (DDP1) gene cause Mohr-Tranebjaerg syndrome (MTS), a rare form of deafness associated with dystonia. In the patient presented here, improvement of dystonic symptoms upon treatment with alcohol and GABAergic substances is demonstrated for the first time.
Adult↗
Movement disorders after intervertebral disc surgery: coincidence or causal relationship?
It is well known that brain injury or central traumatic lesions may result in the subsequent appearance of movement disorders such as dystonia or tremor. The concept that peripheral lesions to neural structures may be involved in the pathogenesis of movement disorders has been discussed controversely but has gained more widespread acceptance only recently. Here, we report on 6 patients who developed movement disorders after spinal disc surgery. The movement disorders became manifest with a delay of 1 day to 12 months after surgery. Of the six patients, 4 underwent cervical disc surgery, and 2 patients were operated on for lumbar disc herniation; 2 patients presented with paroxysmal kinesigenic segmental dystonia, 1 patient with focal dystonia, 2 with unilateral tremor, and 1 with bilateral tremor. The appearance of the movement disorder was associated with persistent dermatomal or segmental pain. In all patients, the anatomic distribution of the movement disorder was related to the nerve root or spinal segment of the corresponding disc level and the manifestation was in close temporal relation to the surgery. We conclude that spinal disc surgery may be another, thus far neglected, cause for movement disorders. The postoperative pain syndrome in all patients should be considered as an important factor of pathogenesis. Overall, movement disorders associated with disc surgery appear to be rare, yet they may cause significant discomfort to the affected individual.
Adult↗
Severe complications after intrathecal methotrexate (MTX) for treatment of primary central nervous system lymphoma (PCNSL).
Primary central nervous system lymphoma (PCNSL) is a rare and highly malignant tumor with increasing incidence. Survival has improved with the use of nonsurgical treatment modalities, of which methotrexate (MTX)-based intrathecal chemotherapy has been an important option. Here, we describe devastating complications in three patients with diffuse large B-cell lymphomas. After intrathecal MTX therapy two patients died secondary to fulminant brain edema. A third patient developed severe dementia, gait disturbance and urinary incontinence due to leukencephalopathy. Since bulky disease was present in all three patients, CSF flow may have been impaired. This might have exposed adjacent tissue to prolonged toxic drug concentrations. Regarding the severe complications seen in these patients, it is useful to consider high-dose intravenous MTX treatment only in periventricular PCNSL.
Adult↗
Functional stereotactic surgery for treatment of cervical dystonia: review of the experience from the lesional era.
BACKGROUND: Functional stereotactic neurosurgery has been rediscovered as a powerful tool to treat various manifestations of medically refractory dystonia. The purpose of this study was to provide the first systematic review of previous experience with thalamic and basal ganglia surgery in the treatment of cervical dystonia (CD). METHODS: A comprehensive review of the literature published between 1950 and 1990 was performed. In addition, data from an unpublished doctoral thesis on 162 patients with CD operated on by Mundinger between 1972 and 1986 were analyzed. RESULTS: Overall, there is published documentation of experience with more than 300 patients with CD who underwent functional stereotactic surgery between the late 1950s and the early 1980s. In general, amelioration of CD was achieved in about 50-70% of patients in most studies. Bilateral surgery generally provided better outcomes than unilateral surgery. Postoperative benefit was reported to become evident only after a delay in several studies. Some reports demonstrated sustained benefit after follow-up of more than 5 years. Bilateral procedures, in particular bilateral thalamotomies, were clearly associated with a higher rate of postoperative side effects, such as dysarthria, dysphagia and ataxia, ranging from 20 to 70%. In the first decade after its introduction, thalamotomy was the preferred procedure. Later, the subthalamic area was targeted more frequently in order to affect pallidal outflow. Experience with targeting the pallidum proper was limited. Functional stereotactic surgery for CD was abandoned in the late 1970s for several reasons, including the general decline in movement disorder surgery at that time, the introduction of selective peripheral denervation and the widespread and beneficial use of botulinum toxin soon thereafter. CONCLUSIONS: Functional stereotactic surgery for treatment of CD has a rich history. There are several lessons to be learned from review of earlier experience. Contemporary techniques have led to a significant improvement in the benefit to risk ratio, in particular regarding bilateral surgery.
Humans↗
Patterning of globus pallidus local field potentials differs between Parkinson's disease and dystonia.
Here we test the hypothesis that there are distinct temporal patterns of synchronized neuronal activity in the pallidum that characterize untreated and treated parkinsonism and dystonia. To this end we recorded local field potentials (LFPs) from the caudal and rostral contact pairs of macroelectrodes implanted into the pallidum of patients for the treatment of Parkinson's disease (12 cases recorded on and off medication, 17 macroelectrodes) and dystonia (10 cases, 19 macroelectrodes). Percentage LFP power in the 11-30 Hz band was decreased and that in the 4-10 Hz band increased across both contact pairs in treated Parkinson's disease compared with untreated Parkinson's disease. Dystonic patients had even less 11-30 Hz power and greater 4-10 Hz power compared with untreated or treated Parkinson's disease patients. The change in the 4-10 Hz band in patients with dystonia was particularly manifest in the more rostral contact pair, presumed to be within or bridging the globus pallidus externus. We conclude that untreated and treated Parkinson's disease and dystonia are characterized by different spatiotemporal patterns of activity in the human pallidum.
Adult↗
A prospective clinical comparison of rectangular titanium cages and iliac crest autografts in anterior cervical discectomy and fusion.
The complications of autogenous bone grafting cause spinal surgeons to seek alternative methods for cervical spinal fusion. This prospective study was conducted to evaluate the safety and efficacy of rectangular titanium cages as compared to the widely performed iliac crest autograft fusion. Thirty-six patients with cervical disc disease in whom an anterior cervical approach was indicated for discectomy were included in a prospective controlled study protocol with 1-year follow-up. The first 18 consecutive patients received iliac crest autograft, while the next 18 received rectangular titanium cages. According to Odom's criteria, 15 of 18 (83%) patients in both groups experienced good to excellent functional recovery. According to the patient satisfaction index, 17 of 18 (94%) in both groups were satisfied. There were no significant differences in neck or arm pain. Fusion was present after 1 year in 16 of 18 (89%) patients in the iliac crest autograft group and 15 of 18 (83%) in the rectangular titanium cage group. In the autograft group, one case of pseudarthrosis was present, and marked hip pain was observed in four patients. There were no implant-related complications in the cage group. The authors conclude that titanium cages in anterior cervical discectomy constitute a safe and efficient alternative to iliac crest bone autograft.
Adult↗
Bilateral pallidal stimulation for blepharospasm-oromandibular dystonia (Meige syndrome).
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Electric Stimulation Therapy↗
Clinical and molecular findings in a patient with a novel mutation in the deafness-dystonia peptide (DDP1) gene.
The Mohr-Tranebjaerg syndrome (MTS) is a rare neurodegenerative disorder characterized by early-onset deafness, dystonia and further neurological abnormalities such as cortical blindness, spasticity, dementia and mental retardation. Causative mutations were identified within the deafness-dystonia peptide (DDP1/TIMM8a) gene on the X-chromosome. The DDP1 protein is located in the intermembrane space of human mitochondria. Here, it acts in a complex together with its partner protein Tim13 in a chaperone-like manner to facilitate the import of nuclear-encoded precursor proteins into the mitochondrial inner membrane. Thus, MTS is a novel type of mitochondrial disorder. To obtain more insight into the pathophysiology of this neurodegenerative disorder, we performed for the first time a comprehensive clinical and functional characterization of a patient suffering from MTS. This patient exhibited a typical combination of deafness, dystonia and visual loss. Sequence analysis of the patient's DDP1 gene revealed a G to C transversion at nucleotide position 38 of the first exon. The mutation affects the ATG start codon, thereby changing methionine to isoleucine (M1I), and leads to a complete absence of the DDP1 protein. In addition, the partner protein Tim13 was found to be significantly reduced, suggesting that Tim13 requires the presence of DDP1 for its stabilization. The assessment of mitochondrial functions showed the enzyme activities of the mitochondrial energy-generating systems to be normal in the muscle biopsy. Structural abnormalities or aggregations of mitochondria were absent. Electron microscopy revealed only a mild neurogenic atrophy. Neurophysiological investigations showed cochlear dysfunction and disturbance of visual pathways. PET and MRI studies revealed a multifocal pattern of neurodegeneration with hypometabolic areas predominantly located over the right striatum and parietal cortex and marked atrophy of the occipital lobes. Although the visual loss is caused predominantly by neurodegeneration of the visual cortex, degeneration of the retina and the optic nerve contributes to the visual impairment. The pathological changes in basal ganglia and sensory cortex demonstrate the disintegration of subcortico-cortical circuits and correlate well with the clinical presentation of multifocal dystonia. The data presented here showed that, in contrast to most of the known mitochondrial disorders, MTS appears not to be associated with a functional defect of the energy generation system of the mitochondria. Whereas the specific mitochondrial dysfunction leading to neuronal loss in MTS remains to be clarified, the electrophysiological and neuroimaging findings allowed the multifocal manifestation of neurodegenerative lesions in MTS to be characterized specifically.
Adult↗