Samuel M. Chou

Chloroquine neuromyotoxicity. Clinical and pathologic perspective.

Six cases of toxic myopathy and/or neuropathy with chloroquine and/or hydroxychloroquine therapy are described. Two patients had unique clinical and pathologic evidence of cardiomyopathy secondary to chloroquine or hydroxychloroquine therapy. One patient had polyneuropathy secondary to chloroquine toxicity. This may be the first documentation of several features of chloroquine/hydroxychloroquine toxicity: morphologic changes in human peripheral nerve in chloroquine toxicity; chloroquine/hydroxychloroquine cardiomyopathy diagnosed by endomyocardial biopsy; and hydroxychloroquine myotoxicity. Chloroquine is a neuromyotoxin that affects nerves and cardiac and skeletal muscles. Discontinuation of chloroquine and hydroxychloroquine resulted in marked improvement in most cases. The reversibility of the symptoms emphasizes the importance of recognizing potential signs of nerve, muscle, and cardiac toxicity in patients being treated with chloroquine or hydroxychloroquine.

Sudden death and paroxysmal autonomic dysfunction in stiff-man syndrome

SummaryTwo women with typical stiff-man syndrome (SMS) developed increasingly frequent attacks of muscle spasms with severe paroxysmal autonomic dysfunctions such as transient hyperpyrexia, diaphoresis, tachypnea, tachycardia, pupillary dilation, and arterial hypertension. Autoantibodies to GABA-ergic neurons were identified in the serum of both patients and in the cerebrospinal fluid of one. Both died suddenly and unexpectedly. General autopsy did not reveal the cause of death. Neuropathological studies revealed perivascular gliosis in the spinal cord and brain stem of one patient and lymphocytic perivascular infiltration in the spinal cord, brain stem, and basal ganglia of the other. The occurrence of a chronic inflammatory reaction in one of the two patients supports the idea that an autoimmune disease against GABA-ergic neurons may be involved in SMS. A review of the literature indicates that functional impairment in SMS is severe and prognosis is unpredictable because of the potential for sudden and unexpected death. Both muscular abnormalities and autonomic dysfunctions may result from autoimmunity directed against GABA-ergic neurons.

Posttraumatic intracranial meningioma: a case report and review of the literature.

A case of posttraumatic meningioma with pathological evidence of intimate association with a nidus of chronic inflammatory reaction is presented. The literature regarding the controversial association of trauma with this neoplasm is critically reviewed. Although meningiomas are not common sequelae of head trauma, it is proposed that they may arise when the injury results in chronic inflammation.

Enhanced DNA synthesis of human glial cells exposed to human leukocyte products

DNA synthesis was studied in primary glial cell cultures derived from adult human non-neoplastic and neoplastic brain tissues. Enhanced DNA synthesis occurred in 5/5 non-neoplastic astrocyte, one oligodendroglioma, and 2/5 astrocytoma cultures after exposure to medium containing 1.25-12.5% supernatant fluid (SF) from insoluble concanavalin A (Con A) stimulated unseparated or T lymphocyte-enriched human mononuclear leukocytes (MNL). Analyses of SF indicated that the presence of platelet-derived growth factor (PDGF) could not account for glial cell stimulation, and exposure to semi-purified interleukin-2 (IL-2) in amounts comparable to those in SF from Con A-stimulated MNL had no effect on glial cells. These data indicate that non-neoplastic astrocytes and other human glial cells are stimulated by products of human MNL.

Myeloradiculopathy secondary to pseudogout in the cervical ligamentum flavum: case report.

A case of cervical myeloradiculopathy secondary to deposits of calcium pyrophosphate dihydrate (Ca2P2O7 2H2O) (CPPD) crystals in the degenerating ligamentum flavum, with marked granulomatous inflammation, is presented. This uncommon clinical presentation of pseudogout (CPPD deposition disease) was confirmed after surgical removal of a compressive cervical ligamentum flavum. The diagnosis of CPPD crystal deposition was determined by polarized light microscopy and energy-dispersive x-ray microanalysis in frozen sections of the biopsy specimen. A review of seven previously reported cases along with the present case failed to reveal trauma as a causative factor.

Primary intracranial germinoma presenting as lower cranial nerve involvement: case report and review of the literature.

Primary intracranial germinomas are classically known as midline lesions, occurring most commonly in the pineal and suprasellar regions, and will usually present with symptoms related to increased intracranial pressure or hypothalmic dysfunction. Involvement of both the pineal and the suprasellar region in the same patient (double midline lesions) is relatively rare (7%). Dissemination of germinoma to both cerebellopontine angles, in addition to the double midline lesions, with the initial clinical presentation of lower cranial nerve involvement has not been previously reported. We encountered such an unusual case, which prompted this report and review of the literature.