Peroxidase-negative Neutrophils in Arakawa-Higashi's Syndrome, in Encephalitis Economo, Further in “Healthy Brethren”

Sato, Akira

doi:10.1620/tjem.87.94

Cited by 5 publications

(3 citation statements)

References 1 publication

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“…However, the lack of pandemic occurrence, the acute process of infection, and the abnormal behavior suggest parkinsonism associated with C H S rather than postencephalitic parkinsonism. Furthermore, the picture of neutrophils containing peroxidase-positive granules differs from that of Echonomo encephalitis, in which peroxidase-negative granules appear (46).…”

Section: Discussionmentioning

confidence: 87%

Adult Chédiak-Higashi syndrome presenting as parkinsonism and dementia

Uyama

Hirano

Ito

et al. 2009

Acta Neurologica Scandinavica

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Chédiak‐Higashi syndrome (CHS) in children can be a fatal disease. We describe the oldest known CHS patient first seen with a neurologic disorder in early adult life. From the age of 22, this 39‐year‐old woman developed mental deterioration, parkinsonism including resting tremor at the tongue, mandible, and hands, oculogyric crisis, muscular atrophy of limbs, and loss of tendon reflexes. MRI showed marked temporal dominant brain atrophy and diffuse spinal cord atrophy. Partial albinism, panleukocytic giant granules, and profoundly decreased NK‐cell activity were compatible with childhood CHS, but apparently normal neutrophil function prolonged her survival. Stimulated proliferation of lymphocytes was less than 40% that of normal controls.

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Section: Discussionmentioning

confidence: 87%

Adult Chédiak-Higashi syndrome presenting as parkinsonism and dementia

Uyama

Hirano

Ito

et al. 2009

Acta Neurologica Scandinavica

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“…Differential centrifugation studies revealed that over 90% of the organisms added to the patient's leukocytes were cell associated within 15 min. The intracellutlar location of a major fraction of the cell-associated Scrratia was confirmed by the ability of the organism to resist an antibiotic exposure (50 Family studies Leukocytes from three of the patient's four sons were assayed with the test strain of C. albicans and showed normal candidacidal activity. The peroxidase activity of homogenates of leukocytes from the four sons ranged from 22 to 38% of the mean normal level.…”

Section: Functional Studiesmentioning

confidence: 97%

“…Our data suggest that MPO may be the major determinant of candidacidal activity in the human neutrophil. Occasional reports have associated leukocyte peroxidase deficiencies with other disorders (47)(48)(49)(50). With time it should be possible to evaluate the relative significance of hereditary _MPO deficiency as a predisposing cause of systemic candidiasis or other infections in man.…”

Section: Functional Studiesmentioning

confidence: 99%

Leukocyte myeloperoxidase deficiency and disseminated candidiasis: the role of myeloperoxidase in resistance to Candida infection

Lehrer¹,

Cline²

1969

J. Clin. Invest.

666

278

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A B S T R A C T The neutrophils and monocytes of a patient with disseminated candidiasis were found to lack detectable levels of the lysosomal enzyme myeloperoxidase (MPO), although they had normal levels of other granule-associated enzymes. Leukocytes from one of the patient's sisters also lacked detectable MPO; leukocytes from his four sons contained approximately one-third of mean normal peroxidase levels. Neither the patient nor his affected relatives had experienced frequent or unusual bacterial infections.The phagocytic activity of the patient's MPO-deficient neutrophils was intact, and the cells displayed normal morphologic and metabolic responses to phagocytosis. In contrast to normal leukocytes which killed 30.5 ±7.3% of ingested Candida albicans in 1 hr, however, the patient's neutrophils killed virtually none. His leukocytes also failed to kill the strain of C. albicans recovered from his lesions, as well as other Candida. species. These MPO-deficient neutrophils killed Serratia marcescens and Staphylococcus aureus 502A at an abnormally slow rate, requiring 3-4 hr to achieve the bactericidal effect attained by normal leukocytes after 45 min. No other abnormalities in his cellular or humoral immune responses were demonstrated.These findings suggest that hereditary MPO deficiency is transmitted as an autosomal recessive characteristic, that the homozygous state conveys enhanced susceptibility to disseminated candidiasis, and that MPO is necessary for candidacidal activity in human neutrophils.This work was presented in part at the National Meeting of the American Federation for Clinical Research, Atlantic City, N. J., and published in abstract form (1).

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Antimicrobial polypeptides of human neutrophils

Lehrer¹,

Ganz²

1990

Blood

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Peroxidase-negative Neutrophils in Arakawa-Higashi's Syndrome, in Encephalitis Economo, Further in “Healthy Brethren”

Cited by 5 publications

References 1 publication

Adult Chédiak-Higashi syndrome presenting as parkinsonism and dementia

Adult Chédiak-Higashi syndrome presenting as parkinsonism and dementia

Leukocyte myeloperoxidase deficiency and disseminated candidiasis: the role of myeloperoxidase in resistance to Candida infection

Antimicrobial polypeptides of human neutrophils

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