PubMed Health⌕ Search

Biomedical subjects

J M Grau

Publications and source records attributed to J M Grau.

At least 19 recordsLinked to original sources
PubMed1996

Neuropsychological alterations in patients with computed tomography-detected basal ganglia calcification.

OBJECTIVE: To investigate the cognitive and mental status of patients with basal ganglia calcification on a computed tomographic scan. DESIGN: Eighteen ambulatory patients with basal ganglia calcification and without other radiological findings who were identified from the computed tomography records of a general hospital in a 2-year period and 16 control subjects who were matched for age, education, sex, and premorbid IQ estimation consented to participate. All subjects underwent a neurological evaluation, a comprehensive neuropsychological battery, and tests with psychiatric rating scales. RESULTS: Significant differences for the control group were found in tests that evaluated motor speed and executive, visuospatial, and some memory functions. Four patients (22%) met criteria for organic mood disorder (minor depression, three patients; bipolar depression, one patient) according to the Diagnostic and Statistical Manual of Mental Disorders, Revised Third Edition, whereas six other patients (33%) met diagnostic criteria for obsessive-compulsive disorder. CONCLUSIONS: These results indicate that patients with basal ganglia calcifications frequently have a subcortical pattern of neuropsychological dysfunction and behavioral changes that are known to be associated with alterations of the frontal-limbic-basal ganglia circuits. The pattern of neuropsychological impairment is consistent with basal ganglia damage. However, poor performance in other neuropsychological tests suggest additional involvement of other connected networks.

Adult↗
PubMed1996

Hodgkin's disease presenting as a cholestatic febrile illness: incidence and main characteristics in a series of 421 patients.

In order to determine the frequency and characteristics of patients with liver abnormalities as the presenting manifestation of Hodgkin's disease (HD), 421 consecutive HD patients were studied. Six patients in the series (1.4%) presented with liver abnormalities that led to of a liver biopsy and the subsequent diagnosis of HD. All had fever prior to HD diagnosis, four frank jaundice, and one hepatic failure. No patient had pruritus. Moderate hepatomegaly was present in four patients. Cholestasis was observed in all cases; in most patients a moderate increase in the transaminase activity was also seen. Two patients had a mild rise in the serum LDH, four had leukopenia, and one eosinophilia. At liver histologic study, Reed-Sternberg cells were demonstrated in four patients; in the remaining two, the presence of atypical histiocytes, lymphocytes, and eosinophils was highly suggestive of HD, the latter diagnosis being confirmed by subsequent study of bone marrow and/or retroperitoneal lymphadenopathies. In three of the six patients, HD was not demonstrated in sites other than the liver. Three patients older than 60 years died shortly after HD diagnosis. By contrast, three patients younger than 40 years showed a dramatic response to chemotherapy: two of them had a further relapse, and one is considered cured after 14 years of continuous remission. Liver disease constitutes an infrequent form of HD presentation which must be included in the differential diagnosis of any patient with fever of unknown origin.

Adult↗
PubMed1996

Lack of muscle toxicity with didanosine (ddI). Clinical and experimental studies.

Currently, 2',3'-dideoxyinosine (ddI) is used in AIDS therapy. To investigate the possible myotoxicity of ddI in patients infected with human immunodeficiency virus (HIV), we examined the effect of ddI in vitro in tissue cultures of skeletal muscles of rats exposed to ddI at doses equivalent to plasma ddI levels obtained in the treatment of HIV patients. Control cultures were exposed to normal saline and zidovudine (AZT). After 4 weeks no changes were noted in the ddI and normal saline cultures, but AZT cultures showed abnormal accumulation of mitochondria. The creatine kinase values in culture supernatants were all normal. We also reviewed the clinical, nutritional and biological parameters, AZT and ddI dosage, and histochemical findings in muscle specimens of 14 HIV patients receiving ddI therapy. All patients had previously received AZT. The mean cumulative dose of ddI was 91.6 gm. Two patients had myalgia, 9 muscle atrophy, and 13 weakness. All patients were malnourished. Five patients had mitochondrial myopathy related to AZT, 4 had ddI-associated neuropathy and 2 patients had only selective type 2 fiber atrophy. One patient had necrotizing vasculitis, one had scattered necrotic fibers and type 2 fiber atrophy and 2 had a normal muscle biopsy. On the basis of the results, we have been unable to implicate ddI as a cause of skeletal myopathy.

Adult↗
PubMed1996

Muscle involvement in rheumatoid arthritis: clinicopathological study of 21 symptomatic cases.

The aim of the current study was to analyze the frequency and characteristics of symptomatic myopathies occurring in rheumatoid arthritis (RA) patients, to correlate these findings with clinical data, and to evaluate their therapeutic implications. All RA patients from a cohort of 350 RA patients from a single institution who developed muscular symptomatology during an 8-year period were included in the study (n = 21). Clinical and laboratory data and electromyographic results were recorded in all cases, and an open muscle biopsy was performed. Weakness and muscle atrophy were the most common symptoms. Serum creatine kinase was increased in 8 cases (38%). Histopathologic study showed type 2 atrophy in 12 cases. In 13 cases, a treatable disease was diagnosed: dermatomyositis (n = 2), d-penicillamine-related dermatomyositis (n = 2), polymyositis (n = 1), muscular mononuclear cell infiltration (n = 3), polyarteritis nodosa (n = 1), glucocorticoid myopathy (n = 3), and toxic chloroquine myopathy (n = 1). In all but 1 patient, muscular clinical response to new therapy and/or drug withdrawal was satisfactory. Although symptomatic muscular involvement in RA is low (6% in the current series), we have found that nearly two thirds of cases were caused by potentially treatable conditions, mainly myositis or toxic myopathies.

Adult↗
PubMed1996

The effect of zidovudine on skeletal muscle mtDNA in HIV-1 infected patients with mild or no muscle dysfunction.

Zidovudine (ZDV), a nucleoside analogue which inhibits viral replication, is currently used in the treatment of type 1 human immunodeficiency virus (HIV-1) infection. It has been considered to be the cause of an acquired form of myopathy associated with a depletion of mitochondrial DNA (mtDNA) in muscle fibres, although the fact that the patients previously studied were clearly symptomatic, and the theoretical difficulty in differentiating on HIV-related myopathy from the effects of ZDV, led to a controversy on the possible deleterious effect of ZDV on muscle fibres. We studied the muscle biopsy taken from 42 HIV-1 infected patients, regardless of their medical complaints, and two series of controls: 12 HIV-negative patients suffering from diverse neuromuscular diseases and 10 normal patients who underwent orthopaedic surgery. Whole DNA was extracted following standard procedures and analysed by means of Southern blotting and polymerase chain reaction (PCR). We found that mtDNA was only depleted in HIV-1 infected patients treated with ZDV, but not in controls or patients untreated with this antiretroviral drug. Moreover, the depletion was more marked in patients who either presented weakness, myalgia, raised serum creatine kinase (CK), or ragged-red fibres. Mitochondrial DNA deletions were found in low proportion in all groups of patients, regardless of their HIV infection or ZDV status, but not in normal controls. We conclude that ZDV treatment in HIV-1 positive patients produces depletion of muscle mtDNA. The depletion can be demonstrated even in asymptotic patients, but is more marked in patients with clinical symptoms or abnormalities in their muscle biopsies. Other mtDNA abnormalities such as deletions seemed to be more related to other circumstances concurring in HIV-1 infected patients than to the effects of ZDV.

Adult↗
PubMed1996

Polyarteritis nodosa in human immunodeficiency virus infection: report of four cases and review of the literature.

Four patients with HIV infection were diagnosed as having panarteritis nodosa type vasculitis in muscle samples taken by biopsy. Fever, weight loss and neuromuscular symptoms were the major complaints. All but one were successfully treated with a low dose of prednisone. The other patient refused treatment, but her muscular complaints did not worsen during 9 months of follow-up. Previously reported cases in the literature are reviewed.

Adult↗
PubMed1996

[Movement disorders and AIDS].

We describe the clinical characteristics, causes and response to treatment in 6 patients with AIDS who presented with abnormal movement disorders between January 1987 and July 1993 in our hospital, 3 with hemiballismus-hemichorea, 1 with athetosis, 1 myoclonia and 1 with "rubric" tremor. Brain imaging showed lesions in the corpus striatum in all the patients. Suspected diagnoses were cerebral toxoplasmosis in 4, cerebral lymphoma in 1 and progressive multifocal leukoencephalopathy in 1. The toxoplasmosis patients showed improvement (2 cases) or disappearance (2 cases) of movements with antiparasitic therapy. Treatment provided no benefit to the patients with leukoencephalopathy and lymphoma. Hemiballismus-hemichorea was the most common movement disorder in AIDS patients. The underlying cause is usually lesions in the basal ganglia arising from toxoplasmosis. If the lesions are so caused, movements may improve with antiparasitic therapy.

Acquired Immunodeficiency Syndrome↗
PubMed1996

Interstitial lung disease related to dermatomyositis. Comparative study with patients without lung involvement.

OBJECTIVE: To compare clinical and histopathological data and outcome of patients with dermatomyositis (DM) with and without interstitial lung disease (ILD). METHODS: Patients diagnosed with definite DM were prospectively evaluated. Clinical and analytical data were recorded and muscle biopsies were performed. In patients with respiratory symptoms an extensive pulmonary evaluation was done. RESULTS: 104 patients were diagnosed with idiopathic inflammatory myopathy, 63 with DM. Eight of the patients with DM (13%) also had associated ILD. Arthralgia and fever were more frequently seen in the ILD group and there was no associated malignant condition. Either atypical or nonspecific cutaneous lesions were more frequently seen in ILD patients. Anti Jo-1 antibodies were positive in 75% of the patients with ILD and in 3% of patients with DM without ILD (p < 0.001). Bronchoalveolar lavage measured for cell count showed an increased lymphocyte count in 3/5 cases in which this data was recorded. Lung biopsies showed interstitial pneumonitis in 2 cases and desquamative interstitial pneumonitis in one. All patients achieved complete remission of muscular involvement. With respect to lung disease, 4/8 obtained complete remission, and the other 4 showed partial improvement. No significant statistical differences in terms of survival were found between the 2 groups. CONCLUSION: ILD associated with DM represents a subgroup of DM with clinical and biological differences. However, such patients do not have poorer prognosis than patients without ILD if they are managed aggressively with immunosuppressive drugs.

Aged↗
PubMed1996

[Critical illness polyneuropathy].

Critical illness polyneuropathy (CIP) is a recently identified entity for which reliable data is unavailable and of which the prevalence is unknown. Although the percentage of patients suffering CIP is low, more cases, including subclinical ones, are likely to be detected through the use of electroneurography. Any aged patient may be affected by CIP. It usually occurs a long stay in the intensive care unit (ICU) and is generally associated with sepsis, severe trauma and so-called multiple organ failure. The main clinical sign is distal weakness in the lower extremeties, although finding one or more of the following signs is not uncommon: quadriparesis, quadriplegia, difficult weaning, loss of osteo-tendon reflexes and muscle atrophy. The pathophysiology of CIP is unknown, although such mechanisms as cell dehydration, increased proteolysis and the activation of certain cytokins have been suggested. Before diagnosing CIP, other neuromuscle diseases and several recently described toxic myopathies must be ruled out. Electroneuromyographic study of axonal lesions may be of great utility, whereas analyses have low specificity. Histologic examination, when possible, allows axonal lesions (but never demyelinization) to be observed along with the non specific changes of myopathy. Although no specific treatment is available, the long-term prognosis is good if the underlying disease that gave rise to ICU admission is controlled, and if rehabilitation therapy is started early.

Age Factors↗
PubMed1995

[A comparative study of the clinical and histological characteristics between classic nemaline myopathy and that associated with the human immunodeficiency virus].

BACKGROUND: Several cases of nemaline myopathy (NM) have been described in patients with human immunodeficiency virus (HIV) infection. The clinical and histological characteristics of classical NM are known, but remain to be defined in HIV positive patients. METHODS: A retrospective review of the NM over a 12-year period (1982-1993) was carried out. The clinical and histological data of the classical NM were compared with those associated to the HIV. RESULTS: Eight cases of NM were collected: five in HIV negative patients (classical NM) and three in HIV positive patients. The latter patients with NM were all males with no family history of myopathy. Motor development had been normal and was not associated with disorders in other systems and the muscular symptoms were slight. On muscle biopsy a lower percentage of fibers with nemaline bodies and type 2 muscle fiber atrophy were observed, as well as slight alterations in the oxidative reactions. A second muscle biopsy performed two years after in one of these patients was normal. Females predominated in the group of HIV negative patients with family history, motor retardation during infancy and different extramuscular alterations being frequent. The muscle symptoms were more marked and severe in the two cases with respiratory musculature involvement. Histologic study demonstrated a higher percentage of nemaline bodies, predominance of type 1 muscle fiber atrophy and frequent alterations in the oxidative reactions. CONCLUSION: Nemaline myopathy presented in patients with HIV infection differs from the classically described form. The presence of nemaline bodies on muscle biopsy in HIV positive patients may be interpreted more as an epiphenomenon in relation with HIV infection than as a primary myopathy.

Adult↗
PubMed1995

[Progressive external ophthalmoplegia and the Kearns-Sayre syndrome: a clinical and molecular study of 6 cases].

The Kearns-Sayre syndrome (KSS) associates progressive external ophthalmoplegia initiating prior to the age of 20 years and pigmentary retinitis with a series of other heterogeneous clinical manifestations. The incomplete syndrome is usually denominated progressive external ophthalmoplegia (PEO)-plus which is a sporadically appearing mitochondrial cytopathy associated with large deletions of a variable proportion of mitochondrial DNA (mtDNA) molecules. Six patients with PEO-plus/KSS in whom muscle biopsy was performed following a complete clinical study are described. The muscle was processed by conventional histochemical techniques, electron microscopy, and genetic study (Southern transference, polymerase chain reaction, restriction cartography and both manual and automatic sequencing). The percentage of mutated mtDNA molecules for each patient was obtained by densitometry. The 6 patients presented multiorganic clinical manifestations characteristics of most mitochondrial diseases. The presence of destructured red fibers were observed in all the biopsies. All the patients presented a deletion in the mtDNA of a size between 4,861 to 7,437 base pairs (bp). All the deletions appeared flanked by direct repetitions from 4 to 13 bp and one also presented inverse repetitions from 5 to 6 bp in the zone next to the rupture point. In the 6 cases heteroplasmia was observed with a variable percentage of deleted molecules from 23 to 56%. The molecular basis of progressive external ophthalmoplegia-plus/Kearns-Sayre syndrome appears to be the existence of sole, large deletions in the mitochondrial DNA with the varying in location and percentage conditioning the appearance of different phenotypes similar among themselves. The 7,437 base pair deletion was the most frequently observed in the patients analyzed.

Adolescent↗
PubMed1995

Membrane attack complex deposits in cutaneous lesions of dermatomyositis.

BACKGROUND AND DESIGN: The presence of membrane attack complex of complement (MAC) deposits in the intramuscular vasculature of biopsy specimens taken from patients with dermatomyositis (DM) has been implicated in the pathogenesis of this myopathy. The purpose of this study was to investigate the presence of MAC deposition in the skin lesions of patients with DM. Using immunohistochemical methods, we examined 22 biopsy specimens from lesional skin, six biopsy specimens from uninvolved skin, and 12 muscle biopsy specimens from patients with DM for the presence of MAC and vitronectin and CD59, two regulatory proteins of complement. RESULTS: The deposition of MAC was demonstrated in a large percentage of biopsy specimens obtained from the lesional skin of patients with DM. Deposits were found along the dermoepidermal junction in 19 (86%) of 22 biopsy specimens. Deposits on the vessel walls of the dermis were found in 17 (77%) of 22 biopsy specimens; but only in six of these biopsy specimens (27%) were deposits present in more than 10% of blood vessels. In contrast, deposits along the dermoepidermal junction and the vessel walls of the dermis were absent in specimens from uninvolved skin. In 12 muscle biopsy specimens obtained simultaneously from these patients, MAC deposits were found on the vessel walls in nine (75%), but only in six (50%) were deposits found in more than 10% of the intramuscular vessels. The pattern of vitronectin immunoreactivity in skin and muscle biopsy specimens obtained from patients with DM was similar to MAC deposits. The expression of CD59 was normal in all skin and muscle biopsy specimens. CONCLUSIONS: The deposition of MAC was found in a high percentage of biopsy specimens from the lesional skin of patients with DM; it was absent in uninvolved skin. These findings suggest that the complement system may be involved in the pathogenesis of the skin lesions of DM.

Adolescent↗
PubMed1995

Acute axonal Guillain-Barré syndrome with IgG antibodies against motor axons following parenteral gangliosides.

We studied 7 patients with an acute motor axonal Guillain-Barré syndrome (GBS), manifested 5 to 15 days after parenteral injection of a commercial ganglioside preparation given for nonspecific pain syndromes. The serum IgG and IgM antibody response to ganglioside was studied serially and the recognition of epitopes on the peripheral nerves and motor end-plates was examined using biotinylated IgG extracted from the patient's serum. Sera from 8 patients treated with the same ganglioside preparation who did not develop neuropathy and from 25 patients with classic GBS never treated with gangliosides were studied concurrently. All patients with ganglioside-related GBS had a rather severe axonal degeneration, incomplete recovery, and high IgG, but not IgM, antiganglioside antibody titers, ranging from 1:320 to 1:10,240. Seven (28%) of the 25 GBS patients had IgG antibody titers, ranging from 1:160 to 1:10,240. None of the ganglioside-treated patients who did not develop GBS and none of the 50 disease control subjects had IgG GM1 antibodies. Purified IgG from the patients with high GM1, antibodies, but not from the others, recognized epitopes at the nodes of Ranvier and the distal motor nerve terminals at the end-plate. We conclude that exogenous ganglioside injections can be immunogenic, triggering IgG antiganglioside antibodies with specificity for motor nerve-terminals. In some patients with axonal GBS such antibodies may be markers or mediators of axonal involvement.

Antibodies↗