PubMed Health⌕ Search

Biomedical subjects

R Voltz

Publications and source records attributed to R Voltz.

At least 37 records · Page 2Linked to original sources
PubMed2002

[Paraneoplastic neurologic autoimmune diseases].

All complications of tumor disease that do not result from the tumor itself, its metastases, or vascular, infectious, metabolic, or therapy-related causes are referred to as paraneoplastic neurologic syndromes. This earlier represented an excluding diagnosis and the association was established only statistically, but since the first description of the anti-Hu antibody in 1985, we know that around 2/3 of patients with paraneoplastic disorders display highly specific antibody reactions in the serum. These reactions not only prove a paraneoplastic etiology in almost 100% of cases, but, due to their highly specific associations with certain tumor types, also aid tumor detection in patients whose tumor types are not known.

Animals↗
PubMed2001

[Neuropsychiatric symptoms in palliative care].

Up to 80% of patients in palliative care suffer from neurological and/or psychiatric symptoms. For many of those symptoms, there are effective treatment options which should be known to the treating physician. The following article reviews clinical, etiological and diagnostic aspects as well as treatment options. For this, a systematic literature search is combined with clinical experience. Pure motor restlessness often is caused by aggravated physical symptoms. Delirium paradoxically often is reversible, even in the palliative setting, but may also be irreversible and a sign of impending death. Fear and depression must not be overlooked as they may effectively be treated. Suicidal ideation may be dealt with without transfer to a psychiatric ward. Physician assisted suicide is critically discussed.

Humans↗
PubMed2001

Palliative care in amyotrophic lateral sclerosis.

Amyotrophic lateral sclerosis (ALS) is the most common degenerative motor neuron disease in adults. The clinical picture consists of generalized fasciculations, progressive atrophy and weakness of the skeletal muscles, spasticity and pyramidal tract signs, dysarthria, dysphagia, and dyspnea. Pseudobulbar affect is common. Disease-specific treatment options are still unsatisfactory. Therapeutic nihilism is not justified as a large array of palliative measures available to enhance the quality of life of patients and their families. Because of its clinical characteristics, ALS represents a paradigm for palliative care in neurological diseases. Numerous projects are being undertaken worldwide in an effort to enlarge the evidence base for palliative interventions in ALS. Palliative care in ALS is a multidisciplinary effort requiring careful coordination. An open and empathic disclosure of the diagnosis is essential. Nutritional deficiency caused by dysphagia can be relieved by a percutaneous endoscopic gastrostomy. Respiratory insufficiency can be effectively treated by non-invasive home mechanical ventilation. The terminal phase of the disease should be discussed at the latest when symptoms of dyspnea appear, in order to prevent unwarranted fears of "choking to death." Psychological and spiritual care of patients and families are important. Collaboration with hospice institutions and completion of advance directives can be of invaluable help in the terminal phase.

Amyotrophic Lateral Sclerosis↗
PubMed2000

Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients.

Paraneoplastic limbic encephalitis (PLE) is a rare disorder characterized by personality changes, irritability, depression, seizures, memory loss and sometimes dementia. The diagnosis is difficult because clinical markers are often lacking, and symptoms usually precede the diagnosis of cancer or mimic other complications. The frequency of antineuronal antibodies in patients with PLE has not been investigated. We examined the neurological symptoms and the causal tumours in 50 patients with PLE to determine the utility of paraneoplastic antibodies and other tests. The diagnosis of PLE required neuropathological examination or the presence of the four following criteria: (i) a compatible clinical picture; (ii) an interval of <4 years between the development of neurological symptoms and tumour diagnosis; (iii) exclusion of other neuro-oncological complications; and (iv) at least one of the following: CSF with inflammatory changes but negative cytology; MRI demonstrating temporal lobe abnormalities; EEG showing epileptic activity in the temporal lobes. Of 1047 patients with neurological symptoms, whose sera or CSF were examined for paraneoplastic antibodies, 79 had the presumptive diagnosis of limbic encephalitis, dementia, cognitive dysfunction, or confusion. Fifty of these patients fulfilled our criteria for PLE. Pathological confirmation was obtained in 12 patients. The commonly associated neoplasms were of the lung (50%), testis (20%) and breast (8%). Neurological symptoms preceded the cancer diagnosis in 60% of patients (by a median of 3.5 months). Twenty-five of 44 (57%) patients with MRI studies had signal abnormalities in the limbic system. Thirty (60%) patients had antineuronal antibodies (18 anti-Hu, 10 anti-Ta, 2 anti-Ma), and 20 were antibody-negative or had uncharacterized antibodies (n = 4). The combination of symptoms, MRI findings and paraneoplastic antibodies established the diagnosis of PLE in 78% of the patients. Patients with anti-Hu antibodies usually had small-cell lung cancer (94%), multifocal neurological symptoms (78%) and a poor neurological outcome. Patients with anti-Ta (also called anti-Ma2) antibodies were young men with testicular tumours (100%), frequent hypothalamic involvement (70%) and a poor neurological outcome. In the group of patients without anti-Hu or anti-Ta antibodies, the tumour distribution was diverse, with cancer of the lung the most common (36%); 57% had positive MRI. Fifteen of 34 (44%) patients with a median follow-up of 8 months showed neurological improvement. Treatment of the tumour appeared to have more effect on the neurological outcome than the use of immune modulation. Improvement was observed in 38% of anti-Hu patients, 30% of anti-Ta patients and 64% of patients without these antibodies.

Adolescent↗
PubMed1999

A serologic marker of paraneoplastic limbic and brain-stem encephalitis in patients with testicular cancer.

BACKGROUND: In patients with cancer, symptoms of limbic and brain-stem dysfunction may result from a paraneoplastic disorder. Paraneoplastic limbic or brain-stem encephalitis occurs more frequently with testicular cancer than with most other cancers. We sought antineuronal antibodies that might be used in a diagnostic test for this syndrome. METHODS: Immunohistochemical and immunoblotting techniques were used to detect serum and cerebrospinal fluid antibodies. Serologic screening of a complementary DNA library and Northern blotting were used to clone the target antigen and determine which tissues expressed it. RESULTS: Of 13 patients with testicular cancer and paraneoplastic limbic or brain-stem encephalitis (or both), 10 had antibodies in serum and cerebrospinal fluid against a 40-kd neuronal protein. These antibodies were used to clone a gene that we call Ma2, which codes for a protein (Ma2) that was recognized by serum from the 10 patients, but not by serum from 344 control subjects. Ma2 was selectively expressed by normal brain tissue and by the testicular tumors of the patients. Ma2 shares homology with Ma1, a "brain-testis-cancer" gene related to other paraneoplastic syndromes and tumors. CONCLUSIONS: The serum of patients with subacute limbic and brain-stem dysfunction and testicular cancer contains antibodies against a protein found in normal brain and in testicular tumors. Detection of these antibodies supports the paraneoplastic origin of the neurologic disorder and could be of diagnostic importance.

Animals↗
PubMed1999

P/Q-type voltage-gated calcium channel antibodies in paraneoplastic disorders of the central nervous system.

Whether P/Q-type voltage-gated calcium channel (VGCC) antibodies are present in the serum of patients with paraneoplastic syndromes other than the Lambert-Eaton myasthenic syndrome (LEMS) and tumors other than small-cell lung cancer (SCLC) is controversial. Using a commercially available radioimmunoprecipitation assay kit, we examined the sera of 93 patients with paraneoplastic syndromes of the central nervous system (CNS), including 27 patients with paraneoplastic cerebellar degeneration (PCD) associated with tumors other than SCLC and 66 SCLC patients with paraneoplastic encephalomyelitis and sensory neuronopathy (PEM/SN). All PCD sera from patients with tumors other than SCLC were negative for P/Q-type VGCC antibodies. Eight of 66 (12%) SCLC patients with PEM/SN had P/Q-type VGCC antibodies; 4 had LEMS and the other 4 had no symptoms of LEMS or they were overlooked and, therefore, not examined electrophysiologically. In patients with paraneoplastic syndromes of the CNS, the detection of P/Q-type VGCC antibodies supports the diagnosis of LEMS; in our series, only 6% of patients with SCLC and PEM/SN may have had a false positive antibody result, or undiagnosed LEMS.

Autoantibodies↗
PubMed1999

Ma1, a novel neuron- and testis-specific protein, is recognized by the serum of patients with paraneoplastic neurological disorders.

The identification of antineuronal antibodies has facilitated the diagnosis of paraneoplastic neurological disorders and the early detection of the associated tumours. It has also led to the cloning of possibly important neuron-specific proteins. In this study we wanted to identify novel antineuronal antibodies in the sera of patients with paraneoplastic neurological disorders and to clone the corresponding antigens. Serological studies of 1705 sera from patients with suspected paraneoplastic neurological disorders resulted in the identification of four patients with antibodies that reacted with 37 and 40 kDa neuronal proteins (anti-Ma antibodies). Three patients had brainstem and cerebellar dysfunction, and one had dysphagia and motor weakness. Autopsy of two patients showed loss of Purkinje cells, Bergmann gliosis and deep cerebellar white matter inflammatory infiltrates. Extensive neuronal degeneration, gliosis and infiltrates mainly composed of CD8+ T cells were also found in the brainstem of one patient. In normal human and rat tissues, the anti-Ma antibodies reacted exclusively with neurons and with testicular germ cells; the reaction was mainly with subnuclear elements (including the nucleoli) and to a lesser degree the cytoplasm. Anti-Ma antibodies also reacted with the cancers (breast, colon and parotid) available from three anti-Ma patients, but not with 66 other tumours of varying histological types. Preincubation of tissues with any of the anti-Ma sera abrogated the reactivity of the other anti-Ma immunoglobulins. Probing of a human complementary DNA library with anti-Ma serum resulted in the cloning of a gene that encodes a novel 37 kDa protein (Mal). Recombinant Mal was specifically recognized by the four anti-Ma sera but not by 337 control sera, including those from 52 normal individuals, 179 cancer patients without paraneoplastic neurological symptoms, 96 patients with paraneoplastic syndromes and 10 patients with non-cancer-related neurological disorders. The expression of Mal mRNA is highly restricted to the brain and testis. Subsequent analysis suggested that Mal is likely to be a phosphoprotein. Our study demonstrates that some patients with paraneoplastic neurological disorders develop antibodies against Mal, a new member of an expanding family of 'brain/testis' proteins.

Amino Acid Sequence↗
PubMed1998

Triple dose of gadolinium-DTPA increases the sensitivity of spinal cord MRI in detecting enhancing lesions in multiple sclerosis.

We compared the sensitivity of single and triple dose Gd-DTPA magnetic resonance imaging (MRI) in detecting enhancing lesions in the spinal cord of 13 patients with multiple sclerosis (MS). We detected two enhancing lesions in two of 13 (15%) patients when the single dose of Gd-DTPA was used and 12 lesions in five of 13 (38%) patients when the triple dose of Gd-DTPA was used. These results suggest that: (1) the use of triple dose increases the sensitivity of spinal cord MRI when studying relapsing-remitting or secondary progressive MS patients, (2) triple dose Gd-DTPA enhanced of the spinal cord MRI might be useful in monitoring disease activity in patients with MS.

Adult↗
PubMed1998

Discontinuation of mechanical ventilation in patients with amyotrophic lateral sclerosis.

Mechanical ventilation, both invasive and non-invasive, may be an effective means of improving the quality of life and prolonging the survival of patients suffering from amyotrophic lateral sclerosis (ALS). However, the attitude towards this palliative measure varies greatly between different centres and countries. One of the arguments cited against this procedure is the fear that a patient might request the physician to discontinue life support. We believe that the question of withdrawal of mechanical ventilation can only be meaningfully addressed in the general context of palliative care. Here, we review possible modes of action in response to a patient's request for life support withdrawal and their medical, legal and ethical implications. We propose that the following goals should be pursued: (1) prevention of unwanted ventilation by early, open discussion with patient and relatives, (2) delivery of optimal palliative care by the caring team, (3) recognition of the patient's right to withdraw his/her consent to an invasive medical procedure. If these goals have been met, it may be medically, legally and ethically justified for the physician to take all necessary steps to ensure a peaceful death after discontinuation of life support.

Amyotrophic Lateral Sclerosis↗
PubMed1998

End-of-life decisions and advance directives in palliative care: a cross-cultural survey of patients and health-care professionals.

In order to explore possible differences in the scope of end-of-life decisions and attitudes toward advance directives (AD) in palliative care, we conducted a survey of 159 patients in palliative care institutions and 93 health-care professionals experienced in palliative care in the United States, Germany, and Japan. Giving an AD in this clinical setting was considered important by patients and professionals. The prevalence of a formal written AD was 79% in the United States, 18% in Germany, and 9% in Japan. In Japan, there was a high prevalence of entrusting all decisions to the family (known as omakase). More than 80% of the patients had negative feelings toward their future decisions in the United States and Germany, in contrast to only 45% in Japan. Although favored by the professionals, there were no specific instruments for obtaining ADs. In Germany and Japan, some patients had given an informal AD. As a pilot content validity step, survey results were used to derive a checklist for content and procedural aspects in end-of-life decision-making. This checklist may provide the basis for developing an instrument to guide physicians, especially non-palliative care specialists, in communication with their patients and their families in this difficult clinical situation.

Advance Directives↗
PubMed1998

T-cell receptor analysis in anti-Hu associated paraneoplastic encephalomyelitis.

OBJECTIVE: To analyze the T-cell receptor (TCR) repertoire in the inflammatory infiltrates of the nervous system and tumor of patients with anti-Hu associated paraneoplastic encephalomyelitis and sensory neuronopathy (PEM/SN). BACKGROUND: In PEM/SN, the pathogenic role of the infiltrating T cells is speculative. TCR analysis may establish whether these lymphocytes are attracted nonspecifically by a proinflammatory environment or are driven by a specific antigen or superantigen. METHODS: We examined frozen tissues of seven patients with PEM/SN using immunohistochemical and PCR analysis. Of 62 tissue blocks from seven patients, 19 blocks from five patients had >100 CD3+ cells per section infiltrating the nervous system or tumor. These infiltrates allowed screening of the TCR Vbeta family repertoire using a panel of 18 antibodies that recognize family-specific regions of most TCR Vbeta families against which antibodies have been generated. To distinguish between antigen-driven clonal and superantigen-driven family expansion, we extracted RNA from frozen tissue and performed reverse transcriptase (RT)-PCR analysis followed by subcloning and sequencing of the antigen-specific CDR3 region of the TCR Vbeta chain. RESULTS: All five patients showed a limited Vbeta repertoire. An overrepresentation (>10% of total CD3+) of certain Vbeta families was identified in three patients (as high as 45% of total CD3+), which consisted mainly of CD8 + cells. CDR3 sequences obtained from one patient revealed an in situ expansion of two clones in the amygdala (one at a frequency of 57%) and four clones in the tumor. CONCLUSION: These findings suggest that an antigen-driven oligoclonal cytotoxic T-cell response plays a role in the pathogenesis of anti-Hu associated PEM/SN.

Aged↗
PubMed1997

Palliative care in amyotrophic lateral sclerosis.

The poor prognosis of amyotrophic lateral sclerosis (ALS) makes palliative care a challenge for the neurologist. Most disabilities associated with progressive disease can be ameliorated by symptomatic treatment. Prognosis and treatment options should be openly discussed with the patient and his/her relatives. Nutritional deficiency due to pronounced dysphagia can be efficiently relieved by a percutaneous enterogastrostomy. Respiratory insufficiency can be treated by non-invasive ventilation at home, provided the familial environment is supportive. Adequate assistance and palliative treatment in the terminal phase is of paramount importance.

Advance Directives↗
PubMed1997

Palliative therapy in the terminal stage of neurological disease.

As recently pointed out by the American Academy of Neurology, providing adequate palliative care to dying patients is the duty of every neurologist. Because of a lack of relevant articles in the neurological literature, we have compiled current treatment recommendations for the most important symptoms arising in the endstage of neurological diseases. These recommendations include treatment of dyspnea, death rattle, restlessness, pain, thirst, depression, and others. A discussion of difficult decisions is included, e.g., the appropriate extent of fluid substitution or the ethical implications of sedation in the terminal phase. It is hoped that this compilation may provide a basis for future research in palliative therapy in neurology.

Adult↗