Chinese Medical Sciences Journal ›› 2020, Vol. 35 ›› Issue (3): 207-214.doi: 10.24920/003669
• Original Article • Previous Articles Next Articles
Dong Liling, Liu Caiyan, Mao Chenhui, Chu Shanshan, Li Jie, Huang Xinying, Gao Jing()
Received:
2019-10-12
Published:
2020-09-30
Online:
2020-09-25
Contact:
Gao Jing
E-mail:gj107@163.com
Dong Liling, Liu Caiyan, Mao Chenhui, Chu Shanshan, Li Jie, Huang Xinying, Gao Jing. Linguistic Characteristics of Mandarin-Speaking Huntington’s Disease Patients[J].Chinese Medical Sciences Journal, 2020, 35(3): 207-214.
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Table 1
Demographic and neuropsychological data of HD and AD patients§ (n=5)"
Items | HD | AD |
---|---|---|
Gender (M/F) | 3/2 | 3/2 |
Age on admission§ (y/o) | 61.0±6.82 | 55.4±5.32 |
Age of onset§ (y/o) | 55.6±9.21 | 50.4±5.59 |
Disease course§ (yrs) | 5.4±2.97 | 5.0±1.87 |
Educational level§ (yrs) | 10.4±3.85 | 13.4±1.95 |
MMSE§ (score) | 24.0±1.22 | 11.8±7.95 |
Moca§ (score) | 19.8±3.83 | 8.2±5.76 |
ADLs§ (score) | 23.8±2.49 | 32.0±16.05 |
HADS-A§(score) | 1.0±1.41 | 4.2±3.03 |
HADS-D§(score) | 1.8±2.49 | 4.2±4.02 |
NPI-frequency§ (score) | 5.0±5.48 | - |
NPI-severity§(score) | 4.4±5.13 | - |
NPI-distress§(score) | 5.6±6.73 | - |
DSST§ (score) | 20.2±4.87 | 12.0±19.65 |
TMT-A§ (s) | 121±23.82 | 171.4±95.78 |
Clock drawing§(score) | 2.0±0.71 | 1.4±0.89 |
Single gesture§(score) | 5.2±1.64 | 3.8±1.92 |
Serial gesture§(score) | 1.4±0.89 | 0.4±0.55 |
Figure copying§(score) | 9.8±0.45 | 6.6±4.22 |
Block design§(score) | 4.7±2.51 | 2.3±3.90 |
Rey-copy (score) | 17.0±0.00 | 13.0±7.31 |
Rey-recall§ (score) | 7.0±5.34 | 2.6±3.97 |
PAL-sum§ (score) | 5.0±1.77 | 0.8±1.79 |
PAL-N1§ (score) | 1.2±0.57 | 0.2±0.45 |
PAL-N2§ (score) | 1.9±0.42 | 0.3±0.67 |
PAL-N3§ (score) | 1.9±1.08 | 0.3±0.67 |
Episodic memory§ (score) | 4.9±1.78 | 1.7±1.72 |
AVLT-I§ (score) | 13.0±6.28 | 5.4±3.78 |
AVLT-N1§ (score) | 3.0±2.00 | 0.4±0.55 |
AVLT-N2§ (score) | 4.2±1.92 | 2.6±2.07 |
AVLT-N3§ (score) | 5.8±2.68 | 2.4±1.82 |
AVLT-N4§ (score) | 3.0±2.83 | 0.2±0.45 |
AVLT-N5§ (score) | 2.4±2.30 | 0 |
AVLT-N6§ (score) | 1.4±1.67 | 0 |
AVLT-N7§ (score) | 12.4±1.67 | 7.2±6.57 |
Similarity§ (score) | 12.2±6.38 | 3.0±3.67 |
Calculation§ (score) | 7.0±1.22 | 2.2±1.64 |
Table 2
Clinical data of HD and AD patients"
Case | Gender | Age (yrs) | Disease course (yrs) | Educational level (yrs) | CAG repeats | CSF test (pg/ml) | Brain MRI* (Grade) | ||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
Aβ | t-tau | HCN atrophy | FL atrophy | TL atrophy | PL atrophy | OL atrophy | |||||||
1 | Male | 57 | 4 | 7 | 39 | + | 2 | 2 | 2 | 2 | |||
2 | Male | 67 | 2 | 6 | 41 | 346 | 50 | + | 2 | 1 | 2 | 1 | |
3 | Female | 64 | 5 | 15 | 38 | + | 2 | 2 | 1 | 2 | |||
4 | Male | 51 | 10 | 13 | 40 | + | 2 | 1 | 2 | 1 | |||
5 | Female | 66 | 6 | 11 | 41 | + | 1 | 1 | 1 | 1 | |||
6 | Female | 47 | 4 | 14 | 213 | 306 | - | 1 | 1 | 1 | 0 | ||
7 | Male | 58 | 5 | 11 | 267 | 356 | - | 1 | 1 | 1 | 1 | ||
8 | Male | 61 | 5 | 14 | 122 | 425 | - | 1 | 2 | 1 | 0 | ||
9 | Male | 54 | 8 | 16 | 182 | 620 | - | 1 | 1 | 1 | 0 | ||
10 | Female | 57 | 3 | 12 | 140 | 437 | - | 1 | 2 | 1 | 0 |
Table 3
Neuropsychological data and drug treatment of HD and AD patients"
Case | Screening test (score) | NPI (score) | Cognitive domain* (grade) | Drug treatment | ||||||||||||
---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|---|
MMSE | Moca | ADLs | HADS-A/D | Frequency | Severity | Distress | Executive | Visuospatial | Memory | Reasoning | Dose | Duration | ||||
1 | 22 | 17 | 20 | 2/0 | 2 | 2 | 2 | 2 | 1 | 2 | 2 | - | - | |||
2 | 24 | 17 | 24 | 0/0 | 0 | 0 | 0 | 1 | 0 | 2 | 2 | - | - | |||
3 | 25 | 21 | 24 | 3/4 | 3 | 2 | 3 | 1 | 1 | 2 | 1 | - | - | |||
4 | 24 | 18 | 27 | 0/5 | 14 | 13 | 17 | 2 | 1 | 3 | 1 | - | - | |||
5 | 25 | 26 | 24 | 0/0 | 6 | 5 | 6 | 1 | 1 | 1 | 1 | DHT 5 mg/d | 6 years | |||
6 | 23 | 17 | 22 | 6/6 | - | - | - | 1 | 0 | 3 | 3 | DHT 10 mg/d+ MEM 20 mg/d | 1 month | |||
7 | 5 | 3 | 23 | 6/4 | - | - | - | 3 | 2 | 3 | 3 | DHT 10 mg/d | 2 months | |||
8 | 5 | 3 | 60 | 0/0 | - | - | - | 3 | 3 | 3 | 3 | DHT 5 mg/d | 1 month | |||
9 | 9 | 9 | 31 | 7/10 | - | - | - | 2 | 2 | 3 | 3 | MEM 20 mg/d | 3 months | |||
10 | 17 | 9 | 24 | 2/1 | - | - | - | 2 | 1 | 3 | 3 | MEM 15 mg/d | 1 month |
Figure 1.
Brain MRI of HD and AD patients. Pictures A-B are from a 57-year-old male HD patient, who has a 4-year disease course and a 7-year education. Brain MRI demonstrates HCN atrophy, accompanied by moderate atrophy of frontal, parietal, temporal and occipital cortices. Pictures C-D are from a 58-year-old male AD patient, who has a 5-year disease course and an 11-year education. Brain MRI shows slight atrophy of frontal, parietal, temporal and occipital cortices, with HCN unaffected."
Figure 2.
Linguistic characteristics of HD and AD patients. This is a simple scatterplot created in SPSS Statistics. It displays the linguistic scores of HD (group 1) and AD (group 2) patients in 11 aspects (Item 1-11). 1. oral comprehension; 2. repetition; 3. oral expression; 4. verbal category fluency; 5. naming; 6. reading; 7. reading comprehension; 8. copying; 9. dictation; 10. writing on picture; 11. spontaneous writing. HD patients show a poor language output with relatively spared input and conduction (both oral and written language). AD patients show a more severe form of global aphasia with written language affected the most."
Figure 3.
Writing examples of HD and AD patients. Pictures A-C are from a 67-year-old HD patient, who has a 2-year disease course and 6-year education. Pictures D-E are from a 47-year-old AD patient, who has a 4-year disease course and 14-year education. Their cognitive screening scores are close, whereas the writings are quite different. The correct answers for dictation (A, D) are COMB (梳子),KEY (钥匙),SLEEP (睡觉),RUN AWAY (跑走),FIVE-POINTED STAR (五星),THE SPRING WIND BLOWS AND THE TREES TURN GREEN (春风吹绿了树叶). The correct answers for writing on picture (B, E) are CHAIR (椅子),BED (床),FLOWER (花),TRIANGLE (三角形),CIRCLE (圆形),RED (红色),YELLOW (黄色),SMOKING (抽烟),DRINKING WATER (喝水),FALLING DOWN (摔倒). In spontaneous writing (C), the HD patient meant to write “I HAVE HYPERACTIVITY DISORDER. AFTER SEEING A DOCTOR IN THE HOSPITAL, MY CONDITION IMPROVED GREATLY. I AM VERY GRATEFUL TO THE DOCTOR IN THE HOSPITAL”. (我有多动病,在医院看后,病情大大好转,我很感谢医院医生). The basic structure of the sentences remains. Strictly speaking, the last two sentences are lack of subjects. Additionally, he shows plenty of character-formation errors and character substitutions. Some characters exhibit stroke omission (角, 院) or substitution (匙, 睡, 在, 感). Some are replaced by others with similar phoneme (形→行), grapheme (钥→钉, 匙→钼, 大→太, 谢→请) or semanteme (绿→蓝, 病→医). The AD patient can only write her name in spontaneous writing. She shows some character substitutions by those with relevant semanteme [床(BED)→睡觉(SLEEP), 花(FLOWER)→书(BOOK), 抽烟(SMOKING)→南京(NANJING) (a cigarette brand in China)]."
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