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K Reiners

Publications and source records attributed to K Reiners.

At least 37 records · Page 2Linked to original sources
PubMed1997

Comparison of transcranial sonography, magnetic resonance imaging, and single photon emission computed tomography findings in idiopathic spasmodic torticollis.

Various lines of evidence suggest that the basal ganglia and thalamus are involved in the pathogenesis of idiopathic dystonia, but unfortunately neuroradiological and pathological data are sparse and controversial. In this study, we have examined 10 patients with spasmodic torticollis by neuroimaging techniques, including transcranial sonography (TS; n = 10), conventional (n = 10) and diffusion-weighted (n = 5) magnetic resonance imaging (MRI), and single photon emission computed tomography (SPECT; n = 10), employing [123I]iodobenzamide (IBZM) as a ligand with a high affinity to the D2 receptor. In seven patients, TS showed small hyperechogenic lesions in the medial segments of the lentiform nucleus contralateral to the side of head deviation. In accordance with the site of TS abnormalities, diffusion-weighted MRI displayed a hyperintense lesion in only one patient, while standard MRI of this area was normal in all patients. SPECT revealed a slight but statistically nonsignificant reduction of IBZM tracer uptake in an area corresponding to the dorsal portions of the striatum in 9 of the 10 patients. TS findings support the hypothesis that structural alternations of the pallidothalamic circuit contralateral to the side of head deviation are involved in the pathogenesis of idiopathic spasmodic torticollis. TS may be more sensitive in detecting basal ganglia alterations than MRI.

Adult↗
PubMed1997

[123I]beta-CIT single-photon emission tomography in DOPA-responsive dystonia.

The radiotracer [123I]beta-CIT is a sensitive marker of dopamine uptake sites that can be used to visualize dopaminergic nerve endings in vivo in the human brain. We report on [123I]beta-CIT single-photon emission computed tomography (SPECT) findings in a patient with DOPA-responsive dystonia (DRD). [123I]beta-CIT SPECT showed a striatal radiotracer uptake in the upper range of normal, indicating intact dopamine transporters and structural integrity of nigrostriatal neurons. This differentiates DRD from clinically similar cases with juvenile-onset parkinsonism with dystonia that have a considerable poorer prognosis. [123I]-beta-CIT SPECT may provide a method equally as useful as fluorodopa positron emission tomography in DRD.

Adult↗
PubMed1997

Power spectrum analysis of heart rate variability in Guillain-Barré syndrome. A longitudinal study.

Power spectrum analysis of heart rate variability was repeatedly carried out on 13 patients with Guillain-Barré syndrome for up to 1 year by Fourier analysis of regular beat-to-beat (R-R) intervals which were recorded for 5 min, converted into a continuous function by linear interpolation and resampled at 5 Hz. Low-frequency (LF) power (reflecting a mixture of parasympathetic and sympathetic activity) and high-frequency (HF) power (reflecting parasympathetic tone) were calculated by integrating the spectra from 0.04 to 0.15 Hz and from 0.15 to 0.4 Hz, respectively. At the height of the disease, the HF component was significantly decreased. The LF:HF ratio, which has been suggested to be an indicator for sympathetic activity, was increased compared with the follow-up value after 1 year. Both measures returned to normal gradually over time. Pooled-data analysis suggested that both HF and LF power were significantly related to the responses of standardized parasympathetic function tests, while the LF:HF ratio was inversely correlated with sympathetic vasomotor activity. In patients presenting with tachycardia, LF and HF power were strikingly decreased compared with patients with normal heart rates, while in patients showing vagal over-reactivity, the power of both spectral bands was significantly increased. The results suggest that spectral analysis of heart rate variability is useful for investigating the cardiovascular neural regulation in patients with Guillain-Barré syndrome. In this disorder, the sympathovagal balance is clearly shifted to sympathetic predominance at the height of the disease.

Adult↗
PubMed1997

Long-latency reflexes of hand muscles in idiopathic focal dystonia and their modification by botulinum toxin.

Long-latency reflexes (LLR) in thenar muscles were elicited by electrical median nerve stimulation in 34 patients with idiopathic focal dystonia and 20 healthy control subjects. Twenty-seven patients had cervical dystonia and seven patients had upper limb dystonia. In about one-quarter of all patients the early LLR (LLR 1, occurring at approximately 40 ms) was abnormal with either increased amplitudes or only unilateral occurrence, mostly on the clinically affected side. Later responses (LLR 2, occurring at approximately 50 ms) were obtained bilaterally in all controls but were reduced or absent in some patients, mostly on the clinically affected side. In 12 dystonia patients, LLR studies were also performed after clinically effective injection of botulinum toxin. Following botulinum toxin treatment there was a significant reduction of LLR 2 amplitudes on the clinically affected side. Our findings suggest a differential involvement of LLR generators in idiopathic dystonia with an antagonism between LLR 1 and LLR 2 on the affected sides. We propose that the reduction of the LLR 2 response may arise from overactivity of the supplementary motor area, confirming the current concept that dystonia results from cortical overflow due to disinhibited thalamocortical pathways projecting to the supplementary motor area. In addition, the dystonic motor pattern seems open to afferent modifications induced by peripheral botulinum toxin treatment.

Adult↗
PubMed1997

Parkinson's disease and depression: evidence for an alteration of the basal limbic system detected by transcranial sonography.

OBJECTIVES: Depression is a frequent symptom in Parkinson's disease. Compelling evidence suggests a role of the brainstem in the control of mood and cognition. In patients with unipolar depression transcranial sonography (TS) studies have shown structural alteration of the mesencephalic brainstem raphe which could suggest an involvement of the basal limbic system in the pathogenesis of primary mood disorders. The objective of the present study was to evaluate whether a similar alteration could be found in depressed patients with Parkinson's disease using TS. METHODS: Thirty patients with Parkinson's disease and 30 age and sex adjusted controls were examined by TS. Raphe echogenicity was rated semiquantitatively. The severity of motor symptoms and depression was rated using standard research instruments. RESULTS: Raphe echogenicity was significantly reduced in depressed patients with Parkinson's disease compared with nondepressed patients with Parkinson's disease and control subjects. Raphe echogenicity correlated negatively with degree of motor impairment, and differences in raphe echo between depressed and non-depressed patients with Parkinson's disease were upheld when motor impairment was controlled for. CONCLUSION: These preliminary findings suggest that, as in unipolar depression, a morphological alteration of the brainstem raphe might be involved in the pathogenesis of depression in Parkinson's disease. This raphe alteration may reflect involvement in the basal limbic system in the pathogenesis of secondary depression. This concept is in line with current knowledge on the pathogenesis of both depression in Parkinson's disease and primary depressive disorders.

Aged↗
PubMed1997

Mitochondrial DNA mutations in multiple symmetric lipomatosis.

Multiple symmetric lipomatosis (MSL) is a rare disorder of middle life characterized by large subcutaneous fat masses around the neck, shoulders and other parts of the trunk. Peripheral neuropathy is a common finding in these predominantly male patients. Employing electrophysiological measures, we found additional signs of central nervous system involvement in a majority of patients. Etiologically, there is an association with mitochondrial dysfunction. In muscle biopsy, we found ragged red fibers in 8 of 12 patients. Molecular genetic analysis revealed multiple deletions of mitochondrial DNA in one patient and the MERRF mutation at nucleotide 8344 in another. In this review, we summarize our clinical, electrophysiological morphological, biochemical and molecular genetic findings in 17 MSL patients, and give a survey of the literature.

Adult↗
PubMed1996

Neurotransmitters in CSF of idiopathic adult-onset dystonia: reduced 5-HIAA levels as evidence of impaired serotonergic metabolism.

While several radiological findings point towards the basal ganglia as a possible anatomical site of the lesion in dystonia patients the biochemical basis of the disorder is still unknown. 5-Hydroxyindoleacetic acid (5-HIAA) and homovanillic acid (HVA) levels-the respective metabolites of serotonin and dopamine-were measured in lumbar cerebrospinal fluid (lCSF) of 15 patients with idiopathic adult-onset focal dystonia and in lCSF of 11 controls. 100 microliters lCSF were analyzed for 5-HIAA and HVA by reversed-phase HPLC with electrochemical detection. 5-HIAA levels were significantly reduced in dystonia patients (11.4 micrograms/ml) compared to controls (18.4 ng/ml) (p < 0.02). HVA levels in dystonia patients (30.3 ng/ml) were below control values (41.6 ng/ml) but this finding did not reach statistical significance. Decreased lCSF levels of 5-HIAA suggest an impaired serotonin metabolism in patients with idiopathic adult-onset dystonia. This observation may provide a biochemical basis for a more specific pharmacotherapy in dystonia patients.

Adult↗
PubMed1996

Meningeal hyperperfusion visualized by MRI in a patient with visual hallucinations and migraine.

A 41-year-old patient with a history of migraine but with no history of seizures had intermittent prolonged and variable complex visual hallucinations and illusions lasting 9 days, accompanied by unilateral headache. Electroencephalography during these visual symptoms revealed occipital epileptic discharges. Distinction between focal migrainous attacks and ictal phenomena was difficult. Magnetic resonance imaging showed a lesion in the right visual cortex probably related to low perfusion and hyperemia of meningeal vessels, representing the rarely described transient MRI changes associated with migraine. Continued treatment with antiepileptic drugs and calcium-channel blocking agents completely resolved the headache and visual symptoms, while minor EEG changes persisted. After discontinuation of treatment, a second attack occurred with a similar and reversible pattern on EEG.

Adult↗
PubMed1996

Eyeball pressure testing in the evaluation of serious bradyarrhythmias in Guillain-Barré syndrome.

OBJECTIVE: To investigate the usefulness of eyeball pressure testing (EP) as an indicator for impending serious bradyarrhythmias in patients with Guillain-Barré syndrome (GBS) and its relationship to motor disability. BACKGROUND: Autonomic dysfunction is a common complication in GBS and accounts for a significant number of deaths. Serious bradyarrhythmias are thought to occur only in severe cases but are difficult to predict. METHODS/DESIGN: In 13 consecutive patients with GBS aged 29 to 70 years, 156 EP (6 to 19 per patient) were done serially for up to 1 year. Bilateral moderate pressure was manually applied and sustained for 25 seconds or until abnormal bradycardia developed, defined as heart rate below 40 beats per minute. Disability was graded by a score from 0 to 5 (DS). RESULTS: Four of 13 patients (DS 2/2/3-4/5) showed abnormal sensitivity to EP at least once. In two of them, vagal overreactivity could be demonstrated repeatedly, which gradually resolved within 4 and 10 days. In one patient with a rapid progressive course requiring early cardiopulmonary resuscitation, a highly abnormal EP could be recorded until 1 day after heart arrest. Another patient (DS 3-4) with abnormal EP required cardiac pacing twice because of significant bradycardia. The only other event necessitating pacing occurred in a severely disabled patient (DS 5-4) who never showed abnormal EP. CONCLUSIONS: Vagal overreactivity could be demonstrated in approximately 30% of our patients. It was not restricted to severe motor impairment and was also present in mild-to-moderately disabled patients. In this regard, EP may be a simple and useful bedside test to indicate an increased risk of developing serious bradyarrhythmias in patients with GBS.

Adult↗
PubMed1996

Lenticular nucleus lesion in idiopathic dystonia detected by transcranial sonography.

We report the transcranial sonography (TCS) findings of the basal ganglia in 86 patients with dystonic disorders including idiopathic dystonia (facial, cervical, upper limb, and generalized dystonia), drug-induced tardive dystonia, dopa-responsive dystonia, and kinesigenic dystonia. The TCS was focused on alterations of the lenticular, caudate, raphe nuclei, and the thalamus. Seventy-five percent of patient with idiopathic cervical and 83% of those with idiopathic upper limb dystonia had a hyperechogenic lesion of the middle segment of the lenticular nucleus on the side opposite to the clinical dystonic symptoms. The ipsilateral side was also affected in 20%. In facial dystonia, only one-third of the patients revealed lenticular nucleus lesions. The mean area of the lenticular nucleus lesion opposite to the clinically affected side was 30 mm2 in cervical dystonia, 17 mm2 in upper limb dystonia, and 7.5 mm2 in facial dystonia. These lenticular abnormalities were significantly more frequent (p < 0.001) and their areas were significantly greater (p < 0.001) compared with a control group of 50 patients afflicted with radiculopathy. There was a significant correlation of the severity of symptoms with the intensity of lenticular nucleus hyperechogenicity in patients with cervical and upper limb dystonia (p < 0.05). Increased caudate nucleus echogenicity was present in 20% of patients with cervical and upper limb dystonia, mostly contralateral to the clinically affected side and raphe abnormalities were present in 7% of all patients with idiopathic dystonia. In contrast, there were no abnormalities of the lenticular nucleus or thalamus in nonidiopathic dystonias. We conclude that idiopathic dystonia is associated with lesions in the basal ganglia, particularly the lenticular nucleus, that can be visualized by TCS. An alteration of the basal ganglia matrix may be the pathologic basis of idiopathic dystonia with secondary affliction of striatopallidothalamic pathways.

Adult↗
PubMed1996

Median corpectomy in cervical spondylotic multisegmental stenosis.

Cervical median corpectomy as an alternative to laminoplasty and laminectomy has been suggested as an effective treatment for cervical spondylotic myelopathy (CSM) in cases of multisegmental spondylotic stenosis. We report on our experience with this procedure with particular reference to neurological outcome and complications. Median corpectomy was performed in 17 cases (3 female, 14 male; mean age 59 yrs, (41-80 yrs.) with cervical myelopathy (CM) and radiologically diagnosed multisegmental spondylotic stenosis and spinal cord compression seen on MRI. The degree of stenosis was determined by means of the modified Pavlov's index (ratio between spinal canal width at the level of the intervertebral disc and the diameter of the vertebral body itself). 3/17 patients suffered from acute, 4/17 from subacute and 10/17 from chronic CM. Single level corpectomy was performed in 9 cases, one and a half vertebrae were removed in 2 cases and dual level corpectomy was performed in the remaining 6 cases. All patients received an autologous bone graft and AO - anterior plate stabilization or were stabilized as described by Morscher. Postoperative follow - up was possible in 16/17 cases over a mean time of 13.5 months. Myelopathy was graded according to Nurick's scale. Postoperatively, 12% with chronic CM improved by two grades, 38% (2 pts, with acute, 3 with subacute and 1 with chronic CM) improved by one grade. The other patients remained stable, none showed worsening of their myelopathy. Paresis improved in 92%, sensory deficits in 69%, spasticity in 73%, pain in 60%, and vegetative disturbances in 100% of all patients presenting these preoperative symptoms respectively. One patient died due to esophageal perforation and subsequent lethal mediastinitis caused by screw loosening 4 months following surgery and after initial neurological improvement. 4 other patients experienced screw loosening, three with acataposis, one remained clinically asymptomatic with concomitant graft displacement in two of these. One patient had to be re-operated due to a hematoma at the iliac crest and 2 suffered from a pelvic fracture of the spina iliaca at the site of graft removal. With respect to the neurological improvement, especially to the motor function and spasticity, median corpectomy can be regarded as an effective procedure in selected cases with cervical myelopathy, even when treatment related complications are taken into consideration.

Adult↗
PubMed1995

Proximal myotonic myopathy. Clinical features of a multisystem disorder similar to myotonic dystrophy.

BACKGROUND: Previous investigations in three families have shown that proximal myotonic myopathy (PROMM) is not linked to the gene loci for myotonic dystrophy (DM) or to the loci of the genes of the muscle sodium and chloride channels associated with other myotonic disorders. It is important to extend our clinical knowledge of this interesting new disorder by studying other families. PATIENTS: Thirty-five patients in 14 new families; 27 patients were examined. METHODS: Clinical examination, electromyography, muscle biopsy, DNA analysis. RESULTS: The following findings were noted: proximal without distal weakness of the legs (n = 21); myotonia on electromyograms (n = 23); intermittent clinical myotonia (n = 17); cataracts (n = 24) and a number of the cataracts were identical to the type in DM (n = 11); and peculiar muscle pain (n = 14). A few patients had cardiac arrhythmias, and others had elevations in the concentrations of serum gamma-glutamyltransferase. None of the patients had significant muscle atrophy. Muscle biopsy specimens showed mild myopathic changes. All patients had normal trinucleotide (cytosine, thymine, and guanine) repeat size of the DM gene in leukocyte DNA. Muscle DNA probes from three patients showed findings identical to those of their leukocyte DNA probes. CONCLUSIONS: Proximal myotonic myopathy is a new genetic disorder similar to, but distinct from, DM. Patients suspected of having DM but with negative DNA studies may have PROMM. The gene defect for PROMM awaits discovery. Because of the similarities between PROMM and DM, this discovery will not only shed light on the pathomechanism of PROMM, but it may also increase our understanding of DM.

Adult↗
PubMed1995

Circulating adhesion molecules and tumor necrosis factor receptor in multiple sclerosis: correlation with magnetic resonance imaging.

Adhesion molecules are important in T-cell trafficking to sites of inflammation. We determined levels of circulating vascular cell adhesion molecule-1 (VCAM-1), L-selectin, and E-selectin in the serum of 147 patients with definite multiple sclerosis of the remitting-relapsing or secondary progressive type. Soluble VCAM-1 and L-selectin concentrations were increased compared to levels in a large group of control subjects. Levels were highest in patients with gadolinium-enhancing lesions on magnetic resonance imaging (VCAM-1: 1,011 +/- 276 vs 626 +/- 87 ng/ml; L-selectin: 1,130 +/- 272 vs 793 +/- 207 ng/ml [mean +/- standard deviation]; p < 0.0001 vs patients without enhancing lesions). Serum levels of soluble tumor necrosis factor receptor (60 kd) were also raised (2.64 +/- 1.23 vs 2.17 +/- 0.69 ng/ml in subjects with other neurological diseases and 2.1 +/- 0.77 ng/ml in healthy control subjects; p < 0.05). Soluble VCAM-1 and L-selectin levels were correlated to concentrations of soluble tumor necrosis factor receptor. In 13 patients with viral encephalitis, similar observations were made. Raised levels of soluble VCAM-1 and L-selectin probably reflect cytokine-induced endothelial cell and T-lymphocyte/monocyte activation occurring in the process of T-cell migration into the central nervous system. Tumor necrosis factor-alpha may be critically involved.

Cell Adhesion Molecules↗
PubMed1995

Neurological multisystem manifestation in multiple symmetric lipomatosis: a clinical and electrophysiological study.

Multiple symmetric lipomatosis (MSL) is characterized by a typical neck and shoulder distribution of subcutaneous lipomata and is often associated with polyneuropathy. Occasionally, the central nervous system (CNS) can be involved. Twelve of 14 patients in this retrospective study had clinical or electrophysiological evidence of a predominantly axonal polyneuropathy. Among those were 10 with alcohol abuse, but 2 patients without alcohol abuse also showed clinical or electrophysiological polyneuropathy. Clinical CNS involvement was present in 4 patients. CNS dysfunction was documented by evoked potentials in 8 subjects [prolonged latency or low amplitude of the motor response following cortical magnetic stimulation (4 patients), abnormal visually evoked potentials (4 patients) or somatosensory evoked potentials (SEP) (4 patients)]. These findings were compared to 10 chronic alcoholics without clinical signs of MSL. Five of these showed mild sensory neuropathy. Additionally, 2 also had delayed SEP latencies. Motor evoked potentials were normal in all controls. We propose that the multisystem involvement in MSL demonstrated here cannot be attributed to alcohol abuse alone. Biochemical studies have suggested mitochondrial dysfunction as the basis of the widespread neurological pathology in MSL.

Adult↗
PubMed1995

Mitochondrial dysfunction in adult-onset myopathies with structural abnormalities.

Three patients with chronic progressive external ophthalmoplegia of adult-onset, generalized muscle atrophy and myalgia are described. Two patients fulfilled the histological criteria for centronuclear myopathy, the third those for fiber-type disproportion. Additionally, typical ragged red fibers were found in all muscle specimens, and several muscle fibers were cytochrome c oxidase negative. NADH and succinate dehydrogenase stains showed increased subsarcolemmal accumulation of mitochondria. To determine whether these findings are coincidental or whether they indicated an additional mitochondrial disorder, all patients were investigated using biochemical analysis of the respiratory chain, molecular genetics, magnetic resonance spectroscopy of quadriceps muscle and ergometry. These tests suggested an additional mitochondrial dysfunction. Mitochondrial dysfunction seems to be more common in this group of myopathies than previously estimated, and may be of importance in the pathogenesis of these disorders.

Adult↗
PubMed1995

Degeneration of substantia nigra in chronic Parkinson's disease visualized by transcranial color-coded real-time sonography.

To detect morphologic abnormalities in Parkinson's disease (PD), we examined 30 patients with PD and 30 age- and sex-matched nonparkinsonian controls by transcranial color-coded real-time sonography (TCCS). In 12 severely affected PD patients, the echogenicity of the substantia nigra was distinctly increased. In the remaining 18 PD patients and in all controls, the substantia nigra was poorly visualized or nondetectable by TCCS. The degree of hyperechogenicity of the substantia nigra closely correlated with the severity and duration of PD (p < 0.001). The increased echogenicity of the substantia nigra notably results from nigral gliosis and reflects the stage of degeneration.

Aged↗