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要 約
8カ月齢の雄の柴犬が，起立困難を主訴に来院した．来院時は失調性麻痺，測定障害，頭部企図振戦，両眼の角膜混濁がみられ，視力障害が認められたが，一般 神経学的検査の成績，聴覚および嗅覚に異常は認められなかった．血清および脳脊髄液検査で犬ジステンパーウイルスの感染は否定され，X線検査および頭部X線CT検査でも異常は認められなかった．一般
血液生化学検査値は正常範囲内であったが，血液塗抹においてリンパ球の細胞質内に複数の大きな空胞が認められたため，リソソーム病を疑い，白血球リソソーム酵素活性の測定を行った．その結果 ，白血球βhガラクトシダーゼ活性が欠損（正常値の0.3％）しており，本症例はGM1ガングリオシドーシスと診断された．
―キーワード：GM1ガングリオシドーシス，リソソーム病，柴犬．
--------------------日獣会誌 54，625〜627（2001）
GM1 Gangliosidosis Diagnosed on the Basis of Assay of Leukocytic
Lysosomal-enzyme Activity in a Shiba-breed Dog
Hiroshi TANAKA＊†, Masanari NAKAYAMA,
Yuko SUGIYAMA and Osamu YAMATO
＊Nakayama Veterinary Hospital, 6-1 Minamifukuro-cho,
Nara 630-8342, Japan
SUMMARY
A Shiba-breed dog (8 months old) referred to our hospital for progressive
neurological symptoms exhibited ataxia, dysmetria, head tremor, corneal clouding, and visual defects.
No severe abnormalities in hearing or olfactory functions were observed. Serum and cerebrospinal fluid
analysis ruled out infection with the canine distemper virus. Radiographic evaluations were normal. Computed
topography revealed no brain abnormalities. Although routine blood biochemistry values were within normal
limits, in a blood smear, multiple large vacuoles were observed in many lymphocytes. On the basis of
this finding, suspected lysosomal disease was diagnosed. Measurements of leukocytic lysosomal enzyme
activities showed a deficiency of β-galactosidase (0.3% of the normal level). The dog was therefore diagnosed
as having GM1 gangliosidosis.
―Key words : GM1 gangliosidosis, lysosomal disease, Shiba-breed dog.
| † Correspondence to : |
Hiroshi TANAKA (Nakayama Veterinary Hospital) 6-1 Minamifukuro-cho, Nara 630-8342, Japan
TEL 0742-25-0007
FAX 0742-25-0005
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-------------------- J. Jpn. Vet. Med. Assoc., 54, 625〜627 (2001) |
