Adult-Onset Neuronal Ceroid Lipofuscinosis in a Shikoku Inu
| Autor: | Akira Yabuki, Masaya Tsuboi, Shoko Doi, Naotami Ueoka, Kazuyuki Uchida, Shinji Tamura, Yumiko Tamura, Osamu Yamato |
|---|---|
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Cerebellum
retina Ataxia General Veterinary medicine.diagnostic_test business.industry Veterinary medicine Shikoku Inu Patellar reflex Case Report Anatomy Spinal cord medicine.disease Luxol fast blue stain medicine.anatomical_structure SF600-1100 Gait abnormality dog medicine Neuronal ceroid lipofuscinosis Brainstem neuronal ceroid lipofuscinosis medicine.symptom business MRI |
| Zdroj: | Veterinary Sciences Veterinary Sciences, Vol 8, Iss 227, p 227 (2021) |
| ISSN: | 2306-7381 |
| Popis: | A two-year-and-eleven-month-old male Shikoku Inu was referred for evaluation of progressive gait abnormality that had begun three months prior. Neurological examination revealed ventral flexion of the neck, a wide-based stance in the hindlimb, wide excursions of the head from side to side, tremor in all four limbs, hypermetria in all four limbs, proprioceptive deficits in all four limbs, reduced patellar reflex in both hindlimbs, and postural vertical nystagmus. Later, behavioral and cognitive dysfunction, ataxia, and visual deficits slowly progressed. Magnetic resonance imaging revealed symmetrical progressive atrophy of the whole brain and cervical spinal cord. Bilateral retinal degeneration was observed, and both flush and flicker electroretinograms were bilaterally non-recordable at the age of five years and eight months, and the dog was euthanized. Histopathologically, faint-to-moderate deposition of light-brown pigments was frequently observed in the cytoplasm of neurons throughout the cerebrum, cerebellum, and nuclei of the brainstem. The pigments were positive for Luxol fast blue, periodic acid–Schiff, and Sudan black B, and exhibited autofluorescence. Electron microscopic examination revealed the accumulation of membranous material deposition in the neuronal cytoplasm. Small foci of pigment-containing macrophages were frequently observed around the capillary vessels. Based on these clinical and pathological findings, the animal was diagnosed with adult-onset neuronal ceroid lipofuscinosis. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|