Journal of Pathology and Translational Medicine

Case Report

Ultrastructural Findings of Hereditary Sensory and Autonomic Neuropathies, Type IV and II.: Jai Hyang Go, Yeon Lim Suh; Korean J Pathol. 1998;32(7):535-539.

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Abstract: Hereditary sensory and autonomic neuropathies (HSAN) are disorders of hereditary neuropathy mainly affecting sensation and also accompanying autonomic nervous system dysfunction. They are divided into five subtypes based on inheritance pattern and clinical manifestation. Among HSAN, type II is characterized by autosomal recessive inheritance, presentation at later stage of life, slow progression and mainly sensation abnormalities. The main pathology of the peripheral nerve is the absence of myelinated nerve fibers. Type IV is very rare disorder and only a few cases have been reported. It is characterized by autosomal recessive inheritance, presentation at birth as failure to thrive, retarded motor development, unexplained pyrexia and rapidly progressive and severe clinical course. The main pathology of the peripheral nerve is a loss of unmyelinated and small myelinated nerve fibers. We report two cases of type IV and one case of type II especially focusing on ultrastructural findings, which are characteristic of and diagnostic for HSAN.

Original Article

Minocycline Attenuates the Development of Allodynia: An Immunohistochemical Study on CD11b, GFAP and c-Fos in the Spinal Dorsal Horn in SD Rat.: Gu Kang, Ki Young Choi, Min Sup Lee, Young Jun Ahn, Seong Sik Kang, Il Young Cheong, Wanjoo Chun, Sung Soo Kim; Korean J Pathol. 2004;38(5):311-318.

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Abstract PDF: BACKGROUND
Minocycline, a semisynthetic second-generation tetracycline, is an antibiotic that has excellent ability to penetration into the CNS via the brain-blood barrier. Minocycline has emerged as a potent inhibitor of microglial activation, and it is an effective neuroprotective agent in experimental brain ischemia. Glial cell activation and proliferation are known to be associated with neuropathic pain in the peripheral nerve injuries.
METHODS
The fifty percent threshold of withdrawal responses was measured in the hindpaws of SD rats following tight ligation of left fifth lumbar spinal nerve. Rats were sacrificed at 1, 3, 5, and 7 days and at 0.5, 1, 2, and 4 h post ligation (n=5/group/time point). Immunohistochemistry for GFAP, CD11b and c-Fos was done on the spinal cord at the level of the fifth lumbar nerve. Minocycline (45 mg/kg) and normal saline (300-400 microL) were administered intraperitoneally, 1 day and 1 h before the operations, and every day postoperatively until the rats were sacrificed.
RESULTS
Treatment with minocycline reduced allodynia and the expressions of CD11b at 5 days and c-Fos at 1 and 2 h post operation compared with the saline treatment (control). CONCLUSIONS: It was thought that minocycline reduced the allodynia induced by tight ligation of the fifth lumbar spinal nerve in rats through the inhibition of microglial activation and c-Fos expression.

Case Report

Chronic Intestinal Pseudoobstruction Associated with Inflammatory Visceral Neuropathy: A Case Report.: Young Kyung Bae, Joon Hyuk Choi, Mi Jin Kim, Dong Sug Kim, Won Kyu Park, Jae Hwang Kim; Korean J Pathol. 2004;38(3):191-195.

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Abstract PDF: The pathogenesis of chronic intestinal pseudoobstruction (CIP) presents a broad spectrum of etiologies. Among them, visceral neuropathy and myopathy are two major pathologic conditions. We report here on a very rare case of CIP associated with inflammatory visceral neuropathy involving the terminal ileum, appendix and entire colon in a 64-year-old woman who did not have any detectable neoplasm or family history of this disease. Microscopically, the submucosal and myenteric plexuses showed a severe and diffuse lymphoplasmacytic infiltrate with degenerative changes of the ganglion cells and nerve fibers. The pathogenesis of the inflammatory reaction is unclear for our patient, but we think that this would be a rare example of idiopathic and sporadic visceral neuropathy resulting in chronic intestinal pseudoobstruction.

In Vitro

Multiple Symmetric Lipomatosis (Cervical Lipomatosis): Two cases report.: Ro Hyun Sung, Won Suk Yang; Korean J Pathol. 1988;22(4):484-488.

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Abstract PDF: Multiple symmetric lipomatosis is a rare disease and affects almost exclusively middle aged man, usually with a background of excessive a alcohol intake. The disease is characterized by progressive growth of subcutaneous fat masses which are located symmetrically at neck, shoulders, chest, abdomen and groin, and which subsequently penetrate deeply into the surrounding spaces and structures with symptomatic compression of deep organs, such as trachea. A recent survey revealed a high incidence of sometic and autonomic neuropathy. The exact cause of the disease is not known, but a hyperplastic mechanism has been postulated, with in vitro studies demonstrating a defect in adrenergic-stimulated lipolysis of lipomatous tissue. We have experienced two cases of multiple symmetric lipomatosis. Case 1 was a 59-year-old male, complaining of slowly enlarging doughunt ring-shaped mass at his neck. He had a habit of excessive alcohol intake for many years. The subcutaneous mass at the neck was excised. The pathology report described the specimen as "normal adipose tissue".
Case
2 was a 49-year-old male, complanining of slowly enlarging multiple symmetric masses at the neck, shoulders, chest, abdomen, flank and groin over a period of 6 years. He also complained of mild muscular weakness. He had a habit of excessive alcohol intake for many years. The subcutaneous mass in the neck was excised. The specimen had a tendency to form globular masses and microscopically indistinguishable from mature adipose tissue.

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