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Biomedical subjects

I Steiner

Publications and source records attributed to I Steiner.

At least 55 records · Page 3Linked to original sources

Subacute painful lumbosacral polyradiculoneuropathy in immunocompromised patients.

The syndrome of inflammatory subacute lumbosacral polyradiculoneuropathy (SLP) has been reported in acquired immunodeficiency syndrome (AIDS) patients in association with cytomegalovirus infection and is only partially amenable to anti-viral therapy. We report three cases of relatively benign inflammatory painful SLP in two non-AIDS, immunosuppressed patients and one who HIV-seroconversed at the time of clinical presentation. SLP developed: (1) in association with HIV seroconversion; (2) during ECHO virus infection in a patient with common variable immune deficiency; and (3) after a severe systemic infection that induced transient immunosuppression due to Epstein-Barr virus reactivation. This report expands the spectrum of viruses associated with acute and subacute lumbosacral polyradiculoneuropathy and may shed light on its possible pathogenesis.

Adult↗

Focal, steroid responsive myositis causing dropped head syndrome.

The dropped head syndrome, which occurs in a variety of neuromuscular disorders, is usually not due to an inflammatory process and generally either self-limited or nonresponsive to therapy. We present an 80-year-old woman who developed progressive neck weakness over a few months due to a focal and restricted inflammatory process involving the neck extensor muscles. She responded dramatically to treatment with immunosuppressive therapy.

Aged↗

Features of dural sinus thrombosis simulating pseudotumor cerebri.

To characterize dural sinus thrombosis (DST) patients presenting with a syndrome simulating pseudotumor cerebri (PTC), medical records of patients initially diagnosed as having PTC between 1980 and 1995 were analysed. Inclusion criteria were intracranial hypertension (IH) and a normal initial computed tomography (CT) scan. All patients underwent angiography or magnetic resonance imaging (MRI) and magnetic resonance venography (MRV). Patients with IH secondary to a determined disorder were excluded from the study. Forty-six patients were included, 12 patients had MRI/MRV or angiography findings compatible with DST and the remaining 34 patients had no vascular pathology on neuroimaging studies and were considered to have PTC. No clinical or auxiliary findings differed between the two groups except for younger age, which delineated the PTC patients. Two patients with DST died during the study. Both were agitated, had a relatively rapid course of symptom progression, and a CSF pressure of over 40 cm water. We conclude that DST can be identified in 26% of patients presenting with symptoms and signs typical of PTC. It may be impossible to differentiate between patients with and without DST if MRI/MRV or angiography are not performed in this scenario.

Adult↗

The value of cerebrospinal fluid antiviral antibody in the diagnosis of neurologic disease produced by varicella zoster virus.

We studied four patients with subacute to chronic varicella zoster virus (VZV) infection of the central nervous system (CNS). VZV infection was verified by detecting antibody to VZV in the cerebrospinal fluid (CSF). VZV caused myelitis in two patients and encephalitis in two patients. In one of the patients with VZV encephalitis, in addition to VZV IgM antibody, VZV DNA was found in the CSF. Among the four patients with VZV infection of the CNS, CSF antibody to VZV was the crucial diagnostic laboratory test which corroborated the clinical features, and indicated that VZV caused neurologic disease. In addition to looking for amplifiable VZV DNA in the CSF of patients with neurologic disease whose clinical and radiologic features suggest VZV infection, we also recommend a search for CSF antibody to VZV, particularly in patients with intervals of weeks to months between zoster and the onset of neurologic disease, or in those patients without rash in whom the tempo of illness is unknown.

AIDS-Related Opportunistic Infections↗

Reversal of vision metamorphopsia: clinical and anatomical characteristics.

BACKGROUND: Metamorphopsia is a visual illusion that distorts the size, shape, or inclination of objects. Reversal of vision metamorphopsia (RVM) is a rare transient form of metamorphopsia described as an upside-down, 180 degrees rotation of the visual field in the coronal plane. The pathophysiological characteristics of RVM remain unclear. DESIGN: Patients with RVM had a complete neurologic examination during or shortly after an episode of metamorphopsia, with particular emphasis on gaze disorders, visual fields, visually guided hand movements, and perceptual or cognitive deficits. Workup included imaging studies, visual field examinations, and brainstem auditory and visual evoked response. SETTING: Department of Neurology, Hadassah University Hospital, Hebrew University-Hadassah Medical School, Jerusalem, Israel. PATIENTS: Six consecutive patients were evaluated from 1991 to 1996. RESULTS: Five patients had parieto-occipital brain insult sparing the primary visual cortex, and 3 also had evidence of a concomitant brainstem or cerebellar syndrome. One patient had pure brainstem syndrome underlying the RVM. Three patients had complete RVM as well as oblique RVM of less than 180 degrees. CONCLUSIONS: These cases imply a possible anatomical localization of the central integrator of visual extrapersonal orientation. Our observations suggest that a separate central mechanism of visual orientation might exist in each cerebral hemisphere and that occipital and parietal lesions that spare the optic radiations may account for the oblique and complete RVM. We postulate that failure to perceive space in an allocentric coordinate frame, particularly in the coronal roll plane, is potentially the critical event underlying RVM.

Aged↗

Anticardiolipin antibodies are frequently present in patients with idiopathic intracranial hypertension.

BACKGROUND: Anticardiolipin antibodies (ACL-Ab) are associated with various neurologic syndromes, but idiopathic intracranial hypertension (IIH) has only rarely been reported in this context. OBJECTIVES: To delineate the frequency and clinical and radiological features of, as well as the cause-and-effect relationship between, ACL-Ab and IIH. METHODS: We analyzed the medical records of patients with IIH hospitalized between January 1989 and September 1995. All patients underwent magnetic resonance imaging or magnetic resonance venography or angiography. Excluded were patients with intracranial hypertension due to dural sinus thrombosis or traumatic, structural, neoplastic, or infectious disorders. Patients who were found on at least 2 separate occasions to have increased IgG titers of ACL-Ab were identified and compared with patients without ACL-Ab. RESULTS: Six (43%) of 14 patients with IIH had ACL-Ab. No differences in clinical, laboratory, or radiological variables could be found between patients with and without ACL-Ab. Only 3 of the 11 ACL-Ab-positive patients had previous systemic or neurologic abnormalities associated with ACL-Ab. CONCLUSIONS: Anticardiolipin antibodies may cause IIH through mechanisms unrelated to major venous thrombosis. Idiopathic intracranial hypertension is frequently associated with ACL-Ab and can be the presenting symptom of the antiphospholipid syndrome. There are no major clinical, laboratory, or radiological features that distinguish between patients with IIH with and without ACL-Ab.

Adolescent↗

Migration of di-(2-ethylhexyl) phthalate from PVC child articles into saliva and saliva simulant.

A simple migration test and a more complicated simulation of children's sucking and biting was used to establish migration of DEHP from PVC child-use articles into saliva and compared to a mimic test. The static migration test of a film containing approximately 30% DEHP with saliva simulant gave the lowest values of DEHP; simple shaking increased the amounts of DEHP from 25 +/- 8 to 499 +/- 153 micrograms/g film. The more complicated arrangements to simulate sucking and biting are not so suitable for standard applications. When comparing the in vitro migration tests with the mimic test the factors were 25 and 29 for the static tests (3 and 6 hours) and 1.4 for the shaking test.

Chemical Phenomena↗

Herpes simplex virus type 1 latency-associated transcripts suppress viral replication and reduce immediate-early gene mRNA levels in a neuronal cell line.

During herpes simplex virus type 1 (HSV-1) latent infection in human dorsal root ganglia, limited viral transcription, which has been linked to HSV-1 reactivation ability, takes place. To study the involvement of this transcription in HSV-1 replication in neuronal cells and consequently in viral latency, we constructed stably transfected neuronal cell lines containing (i) the entire HSV-1 latency transcriptionally active DNA fragment, (ii) the same DNA sequence with deletions of the latency-associated transcript (LAT) promoters, or (iii) the DNA coding sequence of the LAT domain. Replication of HSV-1 or a LAT-negative mutant was markedly repressed in the LAT-expressing cells, a phenomenon mediated by the LATs. To study the mechanism responsible for this effect, we examined LAT influence upon expression of HSV-1 immediate-early (IE) genes ICP0, ICP4, and ICP27, by Northern blot analysis. Following infection of a LAT-expressing neuronal cell line with a LAT-negative mutant, the steady-state levels of all three IE mRNAs were reduced compared to those for control cells. Transient transfections into a neuronal cell line indicated that the LAT suppressive effect upon ICP0 mRNA was mediated directly and was not due to the LAT effect upon the ICP0 promoter. We therefore propose that the LATs may repress viral replication in neuronal cells by reducing IE gene mRNA levels and thus facilitate the establishment of HSV-1 latency in nervous tissue.

Animals↗

Tissue specific distribution of the herpes simplex virus type 1 latency-associated transcripts on polyribosomes during latent infection.

Transcription of herpes simplex virus type 1 (HSV-1) during latency produces two abundant latency-associated transcripts (LATs). We have recently shown, that during HSV-1 latency in mice trigeminal ganglia (TG) LATs are bound to polyribosomes (J Virol, 1997, 71, 2897-2904). In order to study the possible role of this binding in the latency process, we now extend the polyribosomal analysis to brainstem tissues of latently infected mice, that unlike TG do not support viral reactivation. We report here that the relative amounts of the LATs associated with polyribosomes in the brainstems of mice are significantly lower than those present in TG. We therefore show that binding of the 1.5 and 2.0 kilobases LATs to polyribosomes is tissue specific and hypothesize that this association may have a role in the reactivation function of HSV-1.

Animals↗

[A case of malignant carcinoma in submandibular salivary gland in a 13-year old girl].

Of all head and neck neoplasms, 3% are malignant salivary neoplasms. Only 20% of them affect submandibular glands. These tumours vary histologically, which results from the complex embryogenesis of the glands. Malignant submandibular gland tumours are twice as frequent as parotid gland tumours. Simultaneous occurrence of quite different malignant tumours in the same salivary gland is extremely rare. The age range of patients affected with salivary gland neoplasms is wide. However, the occurrence of these neoplasms in children is exceptionally rare. The authors describe a case of a 13-year-old girl with acinose adenoid carcinoma and cystiscarcinoma coexisting in one submandibular salivary gland.

Adolescent↗

Progressive dystonia with optic atrophy in a Jewish-Iraqi family.

The combination of progressive dystonia and optic atrophy is extremely rare and its morphological, metabolic and genetic basis is unknown. In a family of 9 children (8 males) born to consanguineous Israeli-Jewish-Iraqi parents, we identified four brothers who developed the syndrome at the end of the first decade. Patients had hemi or bilateral dystonia associated with striatal, mainly putaminal, atrophy on CT and MRI, various degrees of optic atrophy, minimal corticospinal tract involvement, normal intelligence and no peripheral nervous system or systemic abnormalities. No causative metabolic defect was identified. None of the several known mitochondrial DNA mutations associated with Leber's hereditary optic neuropathy (LHON) or with LHON with dystonia were detected. Likewise, linkage to the idiopathic torsion dystonia region on chromosome 9q34 was excluded. It is suggested that this in our patients might be due to a yet unidentified genomic, autosomal recessive mutation.

Adolescent↗

The abundant latency-associated transcripts of herpes simplex virus type 1 are bound to polyribosomes in cultured neuronal cells and during latent infection in mouse trigeminal ganglia.

During herpes simplex virus type 1 (HSV-1) latency, limited viral transcription takes place. This transcription has been linked to the ability of the HSV-1 genome to reactivate and consists of abundant 2.0- and 1.5-kb collinear latency-associated transcripts (LATs), spanned by minor hybridizing RNA (mLAT). The 1.5-kb LAT is derived from the 2.0-kb LAT by splicing, and both transcripts contain two large overlapping open reading frames. The molecular action mechanisms of the latency-associated gene expression are unknown, and no HSV-1 latency-encoded proteins have been convincingly demonstrated. We have cloned the entire latency-associated transcriptionally active HSV-1 DNA fragment (10.4 kb) under control of a constitutive promoter and generated a neuronal cell line (NA4) stably transfected with the viral LAT's region. NA4 cells produced the 2.0- and the 1.5-kb LATs. Northern blotting and reverse transcription-PCR analysis of RNA from NA4 cells and from trigeminal ganglia of mice latently infected with HSV-1 revealed that the two abundant LAT species were present in the polyribosomal RNA fractions. After addition of EDTA, which causes dissociation of mRNA-ribosome complexes, both LATs could be detected only in subpolyribosomal, but not in polyribosomal fractions. These results show that (i) HSV-1 LATs are bound to polyribosomes during latency in vivo, as well as in neuronal cells in vitro, and therefore might be translated, and that (ii) the NA4 cell line is a suitable tool with which to look for HSV-1 latency-encoded gene products.

Animals↗

[Myofibroblastic proliferative lesions in the paratesticular area].

Myofibroblastic pseudotumorous proliferations have been recognized at a variety of sites. Six cases of paratesticular myofibroblastic lesions with emphasis on the spectrum of morphologic features were described. The microscopic features changed from abundant cellular spindle cell proliferation resembling nodular (pseudosarcomatous) fasciitis to hypocellular and hyalin fibrovascular lesion with extensive dystrophic calcification. The age of the patients ranged from 14 to 77 years. In four cases sarcoma was suspected histologically. Differential diagnosis and histogenesis of the lesion were discussed.

Adolescent↗