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Biomedical subjects

J Towfighi

Publications and source records attributed to J Towfighi.

At least 109 records · Page 6Linked to original sources

Spongy degeneration of the central nervous system (van Bogaert-Bertrand type?) in a newborn infant. A light and electron microscopic study.

Autopsy findings on a 5 day old infant with hypotonia from birth showed extensive spongy changes of the myelinating tracts within the cerebrum, cerebellum and brain stem. The spongy changes, similar to Van Bogaert-Bertrand disease, resulted from intramyelinic edema. However, unlike the typical forms of this disease, swollen astrocytes with abnormal mitochondria were not found. The relationship of this case to typical forms of Van Bogaert-Bertrand disease is discussed. This case may represent a very early form of Van Bogaert-Bertrand disease or a new pathological entity.

Astrocytes↗

The distribution of peroxidases in the sciatic nerves of normal and hexachlorophene intoxicated developing rats.

Horseradish peroxidase (mol. wt. 40 000) or microperoxidase (mol. wt. 1900) were injected over the sciatic nerve of normal or hexachlorophene (HCP) intoxicated developing rats (3,7,14 and 21 days). Light and electron microscopic studies of nerves after histochemical staining for peroxidase revealed: a) the perineurial barrier to the two peroxidases was established in 21 day normal and HCP intoxicated rats; b) in animals 3-14 days, the perineurial barrier to both peroxidases was not formed and peroxidase staining was observed in the periaxonal space and in the space between paranodal loops of myelin; c) intramyelinic vacuoles, induced by HCP in animals 7-14 days did not show peroxidase staining. HCP-induced intramyelinic vacuolation is due to the separation of the myelin lamellae at the intraperiod line; although these vacuoles are potential extensions of the extracellular space, they are not stained with the extracellular markers horseradish or microperoxidase.

Age Factors↗

Glue sniffer's neuropathy.

Progressive sensorimotor neuropathy developed in two patients exposed to prolonged (chronic) inhalation of n-hexane (glue sniffing). Sural nerve biopsies showed loss of axons; remaining axons were either normal or showed accumulation of filaments of 90 to 100 A thick, widened nodes of Ranvier, and focal enlargements. The muscle biopsy revealed neurogenic atrophy. Intramuscular nerve twigs and end-plates, studied in one patient, showed loss of axons and nerve terminals. Unmyelinated axons also showed accululation of 60 to 100 A thick filaments. The similarities between the pathologic findings in the peripheral nerve of these patients and those with acrylamide neuropathy suggest that the n-hexane inhalation produces a dying back neuropathy.

Adult↗

Sarcoidosis of the spinal cord. Case report.

A case of intramedullary sarcoidosis simulating a tumor of the cervical spinal cord is presented. Autopsy showed that the disease was limited to the cervical cord and hilar lymph nodes. The literature is reviewed and six cases of histologically documented spinal cord sarcoidosis are discussed.

Adult↗

Mental retardation, hypotonia, and generalized seizures associated with astrocytic "residual" bodies. An ultrastructural study.

Two siblings suffering since birth from convulsions, hypotonia, and mental retardation are presented. In the older sibling (eight and one-half years of age) frontal lobe biopsy revealed abnormal cytosomes with lamellar profiles in astrocytes, macrophages, and to a lesser degree in neurons. Similar cytosomes have not yet been reported in cases of sphingolipidoses or in late infantile-juvenile amaurotic idiocy. These cytosomes stained intensely with silver proteinate, an ultrastructural cytochemical stain for carbohydrate moieties. In contrast, lipofuscin did not stain with silver proteinate. Multilamellar (crescentic curvilinear) cytosomes from a reported case of late infantile amaurotic idocy (Batten-Vogt-Spielmeyer disease) did not stain with silver proteinate. Abnormal cytosomes were not found in blood cells, liver, and peripheral nerve. In the younger sibling (14 months old) postmortem ultrastructural studies of cerebral tissue showed very few abnormal cytosomes. On the basis of the clinical and ultrastructural findings, we conclude that these two cases can be distinguished from those with multilamellar (crescentic-curvilinear) inclusions and from cases of the so-called "neuronal ceroid-lipofuscinosis" syndrome.

Astrocytes↗

Hexachlorophene retinopathy in rats.

The primary effect of hexachlorophene on rat retina is disruption of the disc membranes of photoreceptor outer segments and irreversible destruction of their cell bodies. The neurons of the ganglion cell layer of the retina also become affected, probably secondary to optic nerve lesions present in hexachlorophene intoxication. The early and probably reversible changes of the discs are vacuolation and vesiculotubular degeneration. Vacuolation in the discs results from separation of their inner aspects. This lesion is analogous to the separation of myelin lamellae at the intraperiod line, also produced by hexachlorophene.

Animals↗

Sural nerve conduction study in the rat: a new technique for studying experimental neuropathies.

An electrophysiologic technique for studying sensory fibers in the rat sural nerve is described. The conduction velocity of the sural nerve in rats is much slower than in humans, probably because of lack of large myelinated nerve fibers in rats. An experimental neuropathy (induced by hexachlorophene) that predominantly causes a myelin disorder produced a slowing of sural nerve conduction velocity and a potential of normal amplitude and duration. An experimental neuropathy (induced by zinc pyrithione) that primarily causes axonal degeneration produced a sensory potential of lower amplitude and shorter duration that had a normal conduction velocity.

Animals↗

Ocular invasion in mucormycosis.

The histopathologic findings in a case of ocular invasion in rhinocerebral mucormycosis are described. The findings of hyphae in the inner sclera and marked involvement of the posterior ciliary arteries suggested an arterial route of ocular invasion by fungus. Only five other cases of rhinocerebral mucormycosis with ocular fungal invasion have been reported to our knowledge. All six patients died from the infection. As a group, these cases suggest that the presence of ocular infiltration by fungus may indicate poor prognosis in rhinocerebral mucormycosis.

Aneurysm, Infected↗