Davison, Andrew S 
ORCID: 0000-0001-5501-4475, Hughes, Gin, Harrold, Joanne A ORCID: 0000-0002-0899-4586, Clarke, Pam ORCID: 0000-0002-1430-1037, Griffin, Rebecca and Ranganath, Lakshminarayan R
(2022)
Long-term low dose nitisinone therapy in adults with alkaptonuria shows no cognitive decline or increased severity of depression.
JIMD reports, 63 (3).
pp. 221-230.
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Long-term low dose nitisinone therapy in adults with alkaptonuria shows no cognitive decline or increased severity of depres.pdf - Published version Download (1MB) | Preview |
Abstract
Little is documented on whether nitisinone-induced hypertyrosinaemia alters cognitive functioning or leads to worsening depression in alkaptonuria (AKU). Wechsler Adult Intelligence Scale-IV (WAIS-IV) and Beck Depression Inventory-II (BDI-II) assessments were performed before and annually following treatment with nitisinone 2 mg daily to assess the impact on cognitive functioning and severity of depression. Serum tyrosine concentrations were also measured annually. <i>WAIS-IV:</i> 63 patients (27 females/36 males: mean age[years] [±standard deviation, range] 55.7[13.7, 26-79]; 60.3[9.6, 19-75]) were included at baseline for assessment of: verbal comprehension (VC), perceptual reasoning (PR), working memory (WM), and processing speed (PS) using separate indices. Over the 6-year period studied 43, 39, 36, 29, 26 and 15 patients had annual assessments. Using a longitudinal model (age and sex adjusted) no significant differences were observed in any of the indices over this period, apart from VC which showed a significant increase after adjustment for sex (<i>p</i> < 0.05). <i>BDI-II:</i> 74 patients (32 females/42 males: mean age[years] [±standard deviation, range] 56.1[13.2, 26-79]; 42 males, 51.5[16.3, 19-70]) were included at baseline. Over the 7-year period studied 48, 47, 38, 34, 32, 24 and 12 patients had annual assessments. No significant differences in BDI-II scores were observed when compared to baseline. Hypertyrosinaemia was observed in all patients following treatment with nitisinone (<i>p</i> < 0.001, at all annual visits). Serum tyrosine was not correlated with WAIS-IV sub-test indices or BDI-II scores pre- or post-nitisinone therapy. These findings suggest that treatment with nitisinone does not affect cognitive functioning and or lead to increased severity of depression.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | Alkaptonuria, BDI‐II, WAIS‐IV, cognitive functioning, depression, nitisinone |
| Divisions: | Faculty of Health and Life Sciences Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences Faculty of Health and Life Sciences > Institute of Population Health |
| Depositing User: | Symplectic Admin |
| Date Deposited: | 20 May 2022 13:04 |
| Last Modified: | 18 Jan 2023 21:01 |
| DOI: | 10.1002/jmd2.12272 |
| Open Access URL: | https://onlinelibrary.wiley.com/doi/10.1002/jmd2.1... |
| Related URLs: | |
| URI: | https://livrepository.liverpool.ac.uk/id/eprint/3155212 |
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