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Biomedical subjects

J Towfighi

Publications and source records attributed to J Towfighi.

At least 91 records · Page 5Linked to original sources
PubMed1983

Churg-Strauss syndrome (allergic granulomatous angiitis). Neuro-ophthalmologic manifestations.

Two patients with severe bronchial asthma, hypereosinophilia, and peripheral neuropathy were initially observed with neuro-ophthalmologic signs and symptoms that included amaurosis fugax, superior oblique palsy, ischemic optic neuropathy, and scattered areas of retinal infarction. Clinical investigation led to the diagnosis of Churg-Strauss syndrome (allergic granulomatous angiitis). As in other collagen vascular diseases, ocular signs or symptoms may occasionally be the most prominent manifestation of the disease. Recognition of this systemic disorder by the ophthalmologist may minimize systemic and ocular complications.

Asthma↗
PubMed1983

Alexander's disease: further light-, and electron-microscopic observations.

The neuropathologic and ophthalmopathologic findings in a 5 3/4-year-old boy with Alexander's disease are reported. Light- and electron-microscopic and immunohistochemical studies revealed that (1) the granular osmiophilic deposits (GOD) in Alexander's disease accumulate mainly in astrocytic processes to form Rosenthal fibers, (2) the Bergmann glia are different in this regard and accumulate the deposits primarily in their perikarya, (3) the Müller cells of retina (which closely resemble astrocytes) do not accumulate GOD, (4) the deposits are also not present in other glial cells and glial-like cells such as pituicytes and pineocytes, (5) the deposits are sparse in the retrobulbar optic nerves, and (6) the peroxidase-antiperoxidase and immunofluorescence studies do not demonstrate glial fibrillary acidic protein (GFAP), albumin, immunoglobulins, or fibrinogen in the astrocytic deposits. The different deposition of GOD in various cytoplasmic regions of astrocytes in different areas of central nervous system (CNS) suggests that astrocyte metabolism may not be uniform throughout the brain. Attention to this point may prove helpful in understanding the pathogenesis of the deposits in Alexander's disease. The absence of immunohistochemically demonstrable plasma proteins and GFAP in the astrocytic GOD indicates that the latter have an origin different from plasma proteins and glial filaments. Alternatively, the deposits may be derived from these proteins, but their antigenicity has since been altered.

Albumins↗
PubMed1983

Systemic and neuropathologic effects of E. coli endotoxin in neonatal dogs.

The acute systemic and neuropathologic effects of E. coli endotoxin were determined in neonatal dogs. Administration of sublethal (LD0), moderate (LD50), or lethal (LD100) doses of endotoxin produced significant arterial hypotension, metabolic (lactic) acidosis, and hypoglycemia. Neuropathologic changes consisted of widespread inflammation in both grey and white matter; however, necrotic lesions were found only in forebrain white matter.

Acidosis↗
PubMed1983

Cis-platinum ototoxicity: clinical experience and temporal bone histopathology.

A clinical and pathologic study of cis-platinum ototoxicity was performed. Twenty-four patients treated with cis-platinum for head and neck cancer were studied prospectively. Post cis-platinum hearing losses were subclinical, detected in 25% of patients, isolated to 4 and 8 kHz, and did not exceed 25 dB at any frequency. Temporal bones from a 9-year-old with frontal lobe astrocytoma and with cis-platinum ototoxicity were studied with routine and special techniques to elucidate oto and neurotoxicity. Degenerative changes in the outer hair cells in the lower turns of the cochlea, in the spiral ganglion, and cochlear nerve were the most striking findings.

Adult↗
PubMed1983

Mucin and fat emboli in mucinous carcinomas. Cause of hemorrhagic cerebral infarcts.

A patient with a mucin-producing carcinoma of the breast died after three weeks of progressive neurologic deterioration. At autopsy, numerous small hemorrhagic cerebral infarcts of varying ages were found. The infarcts were primarily located in arterial boundary zones of cerebral and cerebellar hemispheres. They were associated with numerous mucin and fat emboli. In any unexplained cerebral infarct in patients with mucin-producing carcinoma, mucin and fat emboli should be considered and searched for.

Adenocarcinoma, Mucinous↗
PubMed1982

Optic neuropathy and paratrigeminal syndrome due to Aspergillus fumigatus.

Chronic Aspergillus meningitis and cerebral vasculitis occurred in a 67-year-old man. He experienced periorbital pain that increased in severity during a ten-month period. Although no focal neurologic deficits were initially present, oculosympathetic paresis, corneal hypesthesia, and optic neuropathy developed. This is the first report, to our knowledge, of paratrigeminal syndrome with optic neuropathy due to aspergillosis. The case was also unusual because it was chronic and there was no extracerebral infection or predisposing factors, such as underlying malignancy or collagen vascular disease.

Aged↗
PubMed1982

Status marmoratus and Bielschowsky bodies. A report of two cases and review of the literature.

Intraneuronal inclusions, consisting of polyglucosan and having histochemical and ultrastructural features identical to Lafora body of familial myoclonic epilepsy (Unverricht-Lafora disease), have been found restricted to the lateral pallidum in five patients. Two of these patients were also found to have status marmoratus of the basal ganglia. These lateral pallidal inclusions have been named after Bielschowsky, their original discoverer. We report two additional patients with status marmoratus and Bielschowsky bodies and suggest that these two conditions are frequent concomitant phenomena arising independently from a common cause.

Adolescent↗
PubMed1982

Unifocal eosinophilic granuloma of the temporal lobe.

The clinical features, treatment, and two-year postoperative course of a 14-year-old boy with unifocal eosinophilic granuloma of the temporal lobe are presented. A literature review of unifocal eosinophilic granuloma of the brain revealed 11 additional cases, 9 of which involved the hypothalamus and 1 each the frontal and temporal lobes. Four of the 9 patients with involvement of the hypothalamus developed multifocal disease, whereas none of the 3 who had frontal or temporal lobe involvement developed multifocal lesions. Effective therapy for eosinophilic granuloma in a single resectable site in the brain appears to be total excision. Low-dose radiation has been used to eradicate locally recurrent disease.

Adolescent↗
PubMed1981

Congenital hypomyelination neuropathy: glial bundles in cranial and spinal nerve roots.

Autopsy examination of a 3 1/4-year-old child with a severe congenital hypomyelination neuropathy showed the anterior spinal nerve roots and motor cranial nerves to be almost devoid of myelin in their subarachnoid course. The posterior spinal nerve roots and peripheral nerves were less severely affected. Onion bulb formation was minimal and was present only in the sural nerve. There was extensive glial overgrowth in cranial nerves and spinal nerve roots adjacent to the brainstem and spinal cord. The extent and severity of glial overgrowth were similar to that described in Werdnig-Hoffmann disease and morphologically appeared as glial bundles. These glial bundles are most likely secondary to chronic myelin and axonal damage.

Axons↗
PubMed1981

Effects of chronic vitamin E deficiency on the nervous system of the rat.

Light- and electron-microscopic studies were carried out on the central nervous system (CNS) and the peripheral nervous system (PNS) of vitamin E-deficient rats. Extensive axonal degeneration and dystrophic changes were observed in posterior columns and their medullary relay nuclei, respectively. The changes were more prominent in gracile tracts and nuclei than cuneate tracts and nuclei. Alteration in the PNS were less severe than those in the CNS. The posterior roots and sciatic nerves showed only a mild degree of axonal degeneration, while more distal segments of axons in s.c. nerves, in cutaneous sensory corpuscles, and in muscle spindles of hind paws were more severely affected. The neurons in the dorsal root ganglia showed only accumulation of lipofuscin. The above findings in chronic vitamin E deficiency indicate that (a) in addition to the degeneration of central extensions of sensory neurons, their peripheral axons are also affected, (b) the distribution of lesions is similar to those seen in distal axonopathies or a "dying back" process.

Animals↗
PubMed1980

Crystalline ribosomes are present in brains from senile humans.

Paracrystalline inclusions known as Hirano bodies characteristically appear in the hippocampal region of the brains of humans exhibiting senile and presenile dementias as well as several other neurodegenerative diseases. We present evidence that the currently accepted model for those structures based on alternating filament sheets is not correct, but that Hirano bodies are stacked sheets of membrane-bound ribosomal particles derived from partially degraded rough endoplasmic reticulum or Nissl substance. Using fluorescence staining with acridine orange and ethidium bromide, were have shown that the bodies contain RNA. Spatial filtering of electron micrographs by Fourier techniques shows that the individual particles that make up the arrays have a characteristic shape previously reported for the large subunit of eukaryotic ribosomes. The storage of these ribosomal particles in inclusion bodies may indicate a quiescent state of protein synthesis in the cells. This withdrawal of synthetic mechanisms in the hippocampus may have significant consequences in the loss of ability to consolidate short-term to long-term memory.

Alzheimer Disease↗
PubMed1979

Möbius syndrome. Neuropathologic observations.

Neuropathologic findings in an infant with congenital right facial and bilateral lateral rectus palsy (Möbius syndrome) are presented. Multiple microscopic foci of necrosis were found in the lower pontine tegmentum, involving the region of the intramedullary course of 6th and 7th cranial nerves. The significance of pathologic findings in this infant and 14 additional published cases of Möbius syndrome is discussed.

Abducens Nerve↗
PubMed1978

Neurologic complications of acute myelomonoblastic leukemia of four years' duration.

An adult with acute nonlymphoblastic leukemia involving the central nervous system is presented. Unusual features included: (1) Focal signs and radiographic evidence of sagittal sinus occlusion early in the course of disease; (2) progressive meningeal, cranial nerve, and spinal nerve involvement despite a 4-year bone marrow remission; (3) intracerebral tumor formation, and (4) retrobulbar optic neuritis associated with microscopic findings of herpeslike viral particles. The incidence of clinically overt neurologic disease in adults with acute nonlymphoblastic leukemia seems to have increased in tandem with improved chemotherapy. The prophylactic treatment of the central nervous system during prolonged remission of adult acute nonlymphoblastic leukemia may prove of benefit to these patients.

Adult↗