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Biomedical subjects

George H Kraft

Publications and source records attributed to George H Kraft.

At least 19 recordsLinked to original sources
PubMed2006

Double trouble in hereditary neuropathy: concomitant mutations in the PMP-22 gene and another gene produce novel phenotypes.

BACKGROUND: Mutations in the peripheral myelin protein 22 (PMP-22) gene are the most common cause of Charcot-Marie-Tooth neuropathy and may rarely occur in combination with other neurogenetic diseases. OBJECTIVE: To characterize 3 families having a mutation in PMP-22 in addition to another neurogenetic disease mutation. DESIGN: Clinical, electrophysiologic, and genetic evaluations were made of 3 families with more than 1 genetic neuromuscular disease. SETTING AND PATIENTS: Family members were evaluated in neurogenetic and muscular dystrophy clinics in a university medical center setting. RESULTS: Three unusual families were found: (1) 2 young brothers each having a PMP-22 duplication and a missense mutation in the GJB1 (Connexin-32) gene; (2) a 32-year-old woman having a PMP-22 duplication and a 1000-fold CTG repeat expansion in the DMPK gene (DM1 myotonic dystrophy); and (3) a 39-year-old man with a PMP-22 deletion and a missense mutation in the ABCD1 gene (adrenomyeloneuropathy). The mutations were "additive," causing a more severe phenotype than expected with each individual disease and coinciding with the important impact of each gene on peripheral nerve function. CONCLUSIONS: Individuals having 2 separate mutations in neuromuscular disease-related genes may develop unusually severe phenotypes. Neurologists should be alert to this possibility.

Adult↗
PubMed2006

Satisfaction with, and the beneficial side effects of, hypnotic analgesia.

Case study research suggests that hypnosis treatment may provide benefits that are not necessarily the target of specific suggestions. To better understand satisfaction with and the beneficial "side effects" of hypnosis treatment, questions inquiring about treatment satisfaction and treatment benefits were administered to a group of 30 patients with chronic pain who had participated in a case series of hypnotic analgesia treatment. The results confirmed the authors' clinical experience and showed that most participants reported satisfaction with hypnosis treatment even when the targeted symptom (in this case, pain intensity) did not decrease substantially. Study participants also reported a variety of both symptom-related and nonsymptom-related benefits from hypnosis treatment, including decreased pain, increased perceived control over pain, increased sense of relaxation and well-being, and decreased perceived stress, although no single benefit was noted by a majority of participants.

Adult↗
PubMed2005

Fatigue and psychiatric illness in a large community sample of persons with multiple sclerosis.

BACKGROUND: Fatigue and depression are among the most common and disabling symptoms in multiple sclerosis (MS), but the nature and extent of the relationships between fatigue and psychiatric disorders in MS patients remain poorly understood. METHODS: A mail survey was completed by 739 members of the King County (WA) MS Association. Fatigue was evaluated by questions from the Modified Fatigue Impact Scale (MFIS), depressive symptoms by the Center for Epidemiologic Studies Depression Scale (CES-D), substance-use disorders from the PRIME-MD. Information on demographics, employment and characteristics of MS was also collected. Logistic regression was used to identify covariates significantly associated with disabling fatigue. Receiver operating characteristic (ROC) curve analysis evaluated the sensitivity and specificity of fatigue for depression. RESULTS: Twenty-five percent of this community-dwelling sample reported that their activities were often or almost always limited by fatigue. Seventy-six percent of subjects with disabling fatigue had significant depressive symptoms (CES-D >16), compared with 31% of those without disabling fatigue. Depression was strongly associated with fatigue, after controlling for age, gender, marital status, and severity, course and duration of illness. In logistic regression analysis, subjects with clinically significant depressive symptoms (CES-D >16) were much more likely to report disabling fatigue: OR = 6.24 (4.16, 9.35). Anxiety and substance-use disorders did not have the same strong associations with fatigue. Fatigue was highly sensitive and specific for clinically significant depressive symptoms. CONCLUSIONS: Disabling fatigue is strongly associated with clinically significant depressive symptoms. Patients who report disabling fatigue should be screened for depression.

Adult↗
PubMed2005

Exercise and rehabilitation for individuals with multiple sclerosis.

It is the coexistence of physical and cognitive impairments, together with emotional and social issues in a disease with an uncertain course, that makes MS rehabilitation unique and challenging. Inpatient rehabilitation improves functional independence but has only limited success improving the level of neurologic impairment. Benefits are usually not long lasting. Severely disabled people derive equal or more benefit than those who are less disabled, but cognitive problems and ataxia tend to be refractory. There is now good evidence that exercise can improve fitness and function for those with mild MS and helps to maintain function for those with moderate to severe disability. Therapy can be performed over 6 to 15 weeks in outpatient or home-based settings or as a weekly day program lasting several months. Several different forms of exercise have been investigated. For most individuals, aerobic exercise that incorporates a degree of balance training and socialization is recommended. Time constraints, access, impairment level, personal preferences, motivations, and funding sources influence the prescription for exercise and other components of rehabilitation. Just as immunomodulatory drugs must be taken on a continual basis and be adjusted as the disease progresses, so should rehabilitation be viewed as an ongoing process to maintain and restore maximum function and QOL.

Persons with Disabilities↗
PubMed2005

Hypnotic analgesia for chronic pain in persons with disabilities: a case series.

Thirty-three adults with chronic pain and a disability were treated with hypnotic analgesia. Analyses showed significant pre- to posttreatment changes in average pain intensity that was maintained at 3-month follow-up. Significant changes were also found in pain unpleasantness and perceived control over pain but not in pain interference or depressive symptoms. Hypnotizability, concentration of treatment (e.g., daily vs. up to weekly), and initial response to treatment were not significantly associated with treatment outcome. However, treatment-outcome expectancy assessed after the first session showed a moderate association with treatment outcome. The findings support the use of hypnotic analgesia for the treatment of pain in persons with disabilities for some patients but not the use of pretreatment measures of hypnotizability or treatment-outcome expectancy for screening patients for treatment.

Adult↗
PubMed2004

Recovery from and consequences of severe iatrogenic lymphopenia (induced to treat autoimmune diseases).

To ascertain the consequences of severe leukopenia and the tempo of recovery, we studied the immunity of 56 adult patients treated for multiple sclerosis or systemic sclerosis with autologous CD34 cell transplantation using extremely lymphoablative conditioning. NK cell, monocyte, and neutrophil counts recovered to normal by 1 month; dendritic cell and B cell counts by 6 months; and T cell counts by 2 years posttransplant, although CD4 T cell counts remained borderline low. Initial peripheral expansion was robust for CD8 T cells but only moderate for CD4 T cells. Subsequent thymopoiesis was slow, especially in older patients. Importantly, levels of antibodies, including autoantibodies, did not drop substantially. Infections were frequent during the first 6 months, when all immune cells were deficient, and surprisingly rare (0.21 per patient year) at 7-24 months posttransplant, when only T cells (particularly CD4 T cells) were deficient. In conclusion, peripheral expansion of CD8 but not CD4 T cells is highly efficient. Prolonged CD4 lymphopenia is associated with relatively few infections, possibly due to antibodies produced by persisting pretransplant plasma cells.

Adult↗
PubMed2004

The clinical use of dermatomal somatosensory evoked potentials in lumbosacral spinal stenosis.

DSEPs provide clinicians with a safe, noninvasive technique useful in determining which patients with anatomic spinal stenosis have the added component of neurogenic compromise. Based on physiologic principles, level-by-level prolongation of DSEP latencies, reduction of amplitude, asymmetry, or a complete absence of response is associated with dysfunction in that particular afferent neurologic pathway. This dysfunction does not correspond to the exact level of stenosis noted on MRI because the rootlets in the lumbar and sacral regions pass through multiple spinal segments as they course rostrally through the spinal canal. Given that LSSS typically develops over time, the degree of abnormality likely would correspond to the physiologic slowing occurring in the multiple rootlets of the cauda equina. These recordings are not easy to perform and interpret, but when done correctly, they provide the best evidence for the type of neurophysiologic dysfunction in LSSS that responds favorably to surgical decompression. Similarly, DSEPs might provide a means of neurophysiologically monitoring clinically significant findings in a program of conservative management.

Evoked Potentials, Somatosensory↗
PubMed2003

High-dose immunosuppressive therapy and autologous peripheral blood stem cell transplantation for severe multiple sclerosis.

There were 26 patients enrolled in a pilot study of high-dose immunosuppressive therapy (HDIT) for severe multiple sclerosis (MS). Median baseline expanded disability status scale (EDSS) was 7.0 (range, 5.0-8.0). HDIT consisted of total body irradiation, cyclophosphamide, and antithymocyte globulin (ATG) and was followed by transplantation of autologous, granulocyte colony-stimulating factor (G-CSF)-mobilized CD34-selected stem cells. Regimen-related toxicities were mild. Because of bladder dysfunction, there were 8 infectious events of the lower urinary tract. One patient died from Epstein-Barr virus (EBV)-related posttransplantation lymphoproliferative disorder (PTLD) associated with a change from horse-derived to rabbit-derived ATG in the HDIT regimen. An engraftment syndrome characterized by noninfectious fever with or without rash developed in 13 of the first 18 patients and was associated in some cases with transient worsening of neurologic symptoms. There were 2 significant adverse neurologic events that occurred, including a flare of MS during mobilization and an episode of irreversible neurologic deterioration after HDIT associated with fever. With a median follow-up of 24 (range, 3-36) months, the Kaplan-Meier estimate of progression (>/= 1.0 point EDSS) at 3 years was 27%. Of 12 patients who had oligoclonal bands in the cerebrospinal fluid at baseline, 9 had persistence after HDIT. After HDIT, 4 patients developed new enhancing lesions on magnetic resonance imaging of the brain. The estimate of survival at 3 years was 91%. Important clinical issues in the use of HDIT and stem cell transplantation for MS were identified; however, modifications of the initial approaches appear to reduce treatment risks. This was a heterogeneous high-risk group, and a phase 3 study is planned to fully assess efficacy.

Adult↗
PubMed2003

Dermatomal somatosensory-evoked potentials in the evaluation of lumbosacral spinal stenosis.

DSEPs are very useful in determining which patients with degenerative lumbar spine disease have the added component of neurogenic compromise. Based on sound physiologic principles, level-by-level prolongation of DSEP latencies, reduction of amplitude, asymmetry, or a complete absence of response is associated with dysfunction in that particular neurologic pathway. This result does not necessarily correspond to the exact level of stenosis noted on MRI, because the rootlets in the lumbar and sacral regions pass through multiple spinal segments as they course through the spinal canal. Given that LSSS develops typically over time, it is not unlikely that the degree of abnormality will be variable between different levels. The pattern of abnormality will correspond to the physiologic slowing occurring in the multiple rootlets of the cauda equina. These recordings are not easy to perform and interpret; but, when done correctly, they provide the best evidence for the type of neurophysiologic dysfunction in LSSS that responds favorably to surgical decompression.

Electromyography↗
PubMed2003

Electrodiagnosis of neck pain.

The past 3 decades have witnessed tremendous advances in the field of electrodiagnostic medicine. The high-performance electronics and microprocessors available in contemporary electrodiagnostic instruments have improved the ability to detect, record, measure, and interpret the action potentials arising from the nerves and muscle fibers. With their increased ease of use and effectiveness in both diagnosis and prognosis, electrodiagnostic tests have become valuable tools in evaluation of patients with neck pain. As with any laboratory measure, the utility of electrodiagnostic testing can be increased when it is used in appropriate clinical contexts and when its limitations are understood.

Diagnosis, Differential↗
PubMed2003

Epstein-Barr virus-associated posttransplantation lymphoproliferative disorder after high-dose immunosuppressive therapy and autologous CD34-selected hematopoietic stem cell transplantation for severe autoimmune diseases.

High-dose immunosuppressive therapy followed by autologous hematopoietic stem cell transplantation (HSCT) is currently being evaluated for the control of severe autoimmune diseases. The addition of antithymocyte globulin (ATG) to high-dose chemoradiotherapy in the high-dose immunosuppressive therapy regimen and CD34 selection of the autologous graft may induce a higher degree of immunosuppression compared with conventional autologous HSCT for malignant diseases. Patients may be at higher risk of transplant-related complications secondary to the immunosuppressed state, including Epstein-Barr virus (EBV)-associated posttransplantation lymphoproliferative disorder (PTLD), but this is an unusual complication after autologous HSCT. Fifty-six patients (median age, 42 years; range, 23-61 years) with either multiple sclerosis (n = 26) or systemic sclerosis (n = 30) have been treated. The median follow-up has been 24 months (range, 2-60 months). Two patients (multiple sclerosis, n = 1; systemic sclerosis, n = 1) had significant reactivations of herpesvirus infections early after HSCT and then developed aggressive EBV-PTLD and died on days +53 and +64. Multiorgan clonal B-cell infiltrates that were EBV positive by molecular studies or immunohistology were identified at both autopsies. Both patients had positive screening skin tests for equine ATG (Atgam) and had been converted to rabbit ATG (Thymoglobulin) from the first dose. Of the other 54 patients, 2 of whom had partial courses of rabbit ATG because of a reaction to the intravenous infusion of equine ATG, only 1 patient had a significant clinical reactivation of a herpesvirus infection (herpes simplex virus 2) early after HSCT, and none developed EBV-PTLD. The T-cell count in the peripheral blood on day 28 was 0/microL in all 4 patients who received rabbit ATG; this was significantly less than in patients who received equine ATG (median, 174/microL; P =.001; Mann-Whitney ranked sum test). Although the numbers are limited, the time course and similarity of the 2 cases of EBV-PTLD and the effect on day 28 T-cell counts support a relationship between the development of EBV-PTLD and the administration of rabbit ATG. The differences between equine and rabbit ATG are not yet clearly defined, and they should not be considered interchangeable in this regimen without further study.

Adult↗
PubMed2003

Chronic pain in a large community sample of persons with multiple sclerosis.

INTRODUCTION: This study examined the prevalence, intensity, interference, and biopsychosocial correlates of pain in a large community-based sample of persons with multiple sclerosis (MS). METHODS: Mail surveys were returned by 442 members of the King County (WA) MS Association. Average pain intensity, pain-related activity interference, depressive symptoms and severity of MS were assessed. RESULTS: Forty-four percent reported persistent, bothersome pain in the three months prior to completing the survey. Participants with pain reported an average pain intensity rating of 5.2 (SD = 2.3) on the 0 (no pain) to 10 (pain as bad as could be) scale. Twenty-seven percent reported severe pain (score of 7-10), while 51% of those with pain rated the interference of their pain with daily activities as none to minimal. Twenty percent reported severe interference in activities as a result of pain. In multivariate modeling, MS illness severity, marital status, and self-ratings of overall health were significantly associated with pain-related interference with activities. CONCLUSIONS: Approximately a fourth of this sample described having a chronic pain problem characterized by severe pain intensity and significant pain-related interference with activities. Disability due to pain may be more important than previously recognized for the MS population.

Activities of Daily Living↗
PubMed2002

Intravascular lymphomatosis.

Intravascular lymphomatosis, also known as endovascular lymphoma or angiotropic large cell lymphoma, is a rare malignancy typically diagnosed at autopsy. We describe a case in which the diagnosis was made at an early stage. Specific electrodiagnostic findings suggested concomitant polyneuropathy and myopathy. Suspicion of a paraneoplastic syndrome during electrodiagnostic testing prompted muscle biopsy, which revealed myopathy due to the direct infiltration of neoplastic cells. Resultant treatment with a standard chemotherapy regimen not only increased the patient's survival, but also improved neurological function.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed2002

Depressive symptoms and severity of illness in multiple sclerosis: epidemiologic study of a large community sample.

OBJECTIVE: Previous research has shown high prevalence rates of depression in multiple sclerosis patients seen in specialty clinics. The relationships among depressive symptoms and severity, duration, and course of multiple sclerosis are controversial. METHOD: A survey was mailed to members of the Multiple Sclerosis Association of King County (Wash.). Of the 1,374 eligible participants, 739 returned the survey, a response rate of 53.8%. Data about demographic characteristics, employment, and duration and course of multiple sclerosis were collected. Severity of multiple sclerosis was determined by the Expanded Disability Status Scale, self-report version. Severity of depressive symptoms was evaluated with the Center for Epidemiologic Studies Depression Scale (CES-D Scale). Analysis of covariance was used to compare mean CES-D Scale scores across categories of multiple sclerosis, and logistic regression was used to identify variables associated with clinically significant depression. RESULTS: Clinically significant depressive symptoms (CES-D Scale score > or =16) were found in 41.8% of the subjects, and 29.1% of the subjects had moderate to severe depression (score > or =21). Subjects with advanced multiple sclerosis were much more likely to experience clinically significant depressive symptoms than subjects with minimal disease. Shorter duration of multiple sclerosis was associated with a greater likelihood of significant depressive symptoms, but the pattern of illness progression was not. CONCLUSIONS: In this large community sample, the severity of multiple sclerosis was more strongly associated with depressive symptoms than was pattern of illness. Clinicians should evaluate depression in patients with recent diagnoses of multiple sclerosis, major changes in functioning, or limited social support.

Activities of Daily Living↗
PubMedDate not supplied

Amphiphysin paraneoplastic syndrome: a delayed diagnosis of breast carcinoma.

We present a rare case of the paraneoplastic disorder, amphiphysin autoimmunity, which is associated with breast cancer. Amphiphysin is a presynaptic protein to which the body can form antibodies usually in cases of carcinoma, particularly of the breast and lung. The diagnosis of malignancy was delayed because of the complexity of the presenting neurologic symptoms related to the autoimmunity. The confusing picture and the rarity of the entity can result in delays in routine investigations, such as screening mammography. We advocate routine screening and physical examination in all patients, even in the presence of perplexing clinical symptoms.

Breast Neoplasms↗