| ObjectiveTo investigate the clinical characteristics and auxiliary examination features of FTD,we analyzed the clinical data of 17 patients with frontotemporal dementia(FTD)and compared the commons and difference between two clinical subtypes of FTD-behavioral variant of frontotemporal dementia(bvFTD)and primary progressive aphasia(PPA),and two clinical subtypes of PPA--progressive non-fluent aphasia(PNFA)and semantic dementia(SD)in age at onset,gender,early symptoms,cognitive function,daily ability,imaging characteristics,electrophysiology,and cerebrospinal fluid.MethedsWith reference to the 2015 Diagnostic Guidelines for Dementia and Cognitive Impairment and the 2014 Chinese Diabetic Degeneration E'xpert Consensus,17 patients with FTD admitted to the Department of Neurology,Qilu Hospital of Shandong University from 2015.12 to 2017.12 were recruited and clinically classified.We recorded their clinical data such as basic information,clinical manifestations,imaging,electrophysiology,cerebrospinal fluid,and gene detection.Independent sample T-test and chi-square test were used to compare the differences in sex,age of onset,first symptom,clinical features,cognitive function,mental,behavioral changes,daily living ability,and brain imaging between the two clinical subtypes of the FTD.Statistical analysis was performed with SPSS 21.0.A P value of<0.05 indicates a statistical difference.Graphs were plotted using GraphPad Prism 5.0.Results1.Gender,age of onset,genetic background:7 males and 10 females were involved in 17 cases of FTD patients,with a male to female ratio of 0.7:1.12 bvFTD patients,which consisted of 7 male patients and 5 female patients.5 PPA patientswere all females which consisted of 3 PNFA patients and 2 SD patients.The gender composition of patients with bvFTD and PPA was significantly different.PPA was more common in female.The age of onset of FTD patients was 32-77 years old,and the average age of onset was 61.88±12.79 years,which was earlier than the age of onset in patients with Alzheimer's disease.The average age of onset of bvFTD patients was 59.33±13.634 years,and the average age of onset of PPA patients was 68.00±8.746 years.There was no significant difference in the age of onset of the two subtypes.None of the 17 patients with FTD had a family history of inheritance.Only one patient had a gene mutation:NEFH.2.First symptoms:Loss of motivation,apathy,and dyslexic comprehension were occurred in over 80%of patients with FTD,which may be used as the sign to identify the disease in the earliest stage.Sleep disorders,delusions,behavioral impulses,aggression,inflexibility,decreased judgment,loss of empathy,stereotyped language,illogical behavior,compulsive behaviors,preference for sweets,and overeating were more common in bvFTD patients,while words repetition errors,impaired confrontation naming,and impaired word finding are more common in patients with PPA.With the progress of the disease,the clinical symptoms of the two FTD subtypes(bvFTD and PPA)gradually present overlapping:In the second year of onset,patients with bvFTD developed language disorders such as impaired confrontation naming,sentence comprehension errors,and impaired word finding.Patients with PPA showed abnormal behaviors such as apathy and personality changes in the third year after onset of illness3.Cognitive function:There was no difference in cognitive impairment between bvFTD patients and PPA patients.(The mean scores of MMSE were 18.1016.674 and 15.80±7.981 respectively,P>0.05).Patients with SD had significantly milder cognitive impairment than patients with PNFA(The mean scores of MMSE were 22.50±2.121 and 11.33±7.095 respectively,P =0.035).The naming function of bvFTD patients was significantly stronger than that of PPA patients(The mean ACE-? scores for naming were 8.90±1.729 and 5.00±2.550 respectively,P =0.004).The recognizing function of the SD patients was significantly higher than that of the PNFA patients.(The ACE-? scores for recognizing were 1.33±1.528 and 8.50±2.121 respectively,P =0.021).4.Overlap with Amyotrophic lateral sclerosis(ALS):Among the 17 patients with FTD,two FTD patients had ALS,accounting for about 12%.One patient with bvFTD carried a NEFH gene mutation which were prone to cause ALS.However,the patient had no symptoms or signs of upper and lower motor neuron lesions during the 2-year follow-up.Whether the patient suffering from FTD-ALS spectrum disease still need follow-up observations.5.Daily life ability:According to the percentage of RRS scale,daily lives of 5 PPA patients were moderately affected by disease;daily lives of 4 bvFTD patients were severely affected,7 bvFTD patients were pretty severely affected,while one patient suffered extremely severely affection,p=0.000.Patients with PPA had a smaller impact on their daily lives than patients with bvFTD.Compared with patients with bvFTD,PPA patients have less frequency and severity of psychiatric symptoms,which makes caregivers less distressed.(The mean scores for frequency and severity of NPI were 20.58±6.986 and 1.60± 1.342 respectively,P<0.05;The mean scores for distressing caregivers were 7.92±1.929 and 0.40±0.548 respectively,P<0.05).The highest frequency and the highest degree of severity are the biggest causes of the distress of caregivers.(The frequency × the average severity of anomalous behaviors is 6.58±3.029 points,which causes the caregiver distressed to be an average of 2.17±0.577 points.)6.Brain atrophy:The cranial MRI of 17 patients showed atrophy of the frontal lobe and/or temporal lobe in different degrees.There were 14 cases of asymmetrical atrophy(left hemisphere atrophy:right hemisphere atrophy ratio was 3.7:1),and 3 cases of symmetrical atrophy.Brain atrophy in FTD patients is mostly bilateral asymmetry,and the left hemisphere is usually involved.According to the GCA scale,the degree of acrosomal atrophy averaged 1.71 ±0.772 grades,the degree of atrophic atrophy averaged 1.53±0.875 grade,the atrophy degree of the parietal lobe averaged 0.30±0.470 grade,the degree of atrophic lateral fissure averaged 1.41 ±0.712 grade,the degree of atrophy of the parietal lobe Significantly lighter than frontal,temporal,and lateral fissures.The degree of atrophy of the lateral fissures of PNFA was significantly higher than bvFTD and SD(p=0.04).The MTA score of 17 patients with FTD was 0.76±0.66,and the degree of hippocampal atrophy was lighter.7.EEG:6 patients underwent electroencephalography:EEG showed normal range in 2 patients,edge state in 1 patients,abnormal electroencephalogram in 3 patients.Abnormal electroencephalograms had low-medium amplitude and slow-wave ?rhythm,mostly on the left side,suggesting that the left hemisphere was more severely damaged,which was consistent with cranial MRI.8.CSF:Of the 17 patients with FTD,4 underwent lumbar puncture.3 had lower lumbar puncture pressure,and 1 had normal pressure.Four patients had normal cerebrospinal fluid,cytology,and biochemistry.Three patients were examined for cerebrospinal fluid,serum autoimmune encephalitis,and paraneoplastic serial antibodies were all negative,further excluding cognitive disorders caused by central nervous system infectious diseases and autoimmune diseases.Two patients were examined for cognition-related markers of cerebrospinal fluid,normal or elevated A?1-42,and normal or elevated P-tau.ConclusionsThe onset age of frontotemporal dementia was earlier,with an average age of 61.88±12.79 years.FTD is common in sporadic form,with hidden symptoms,slow progress.Personality/behavioral changes and language disorders were more important than memory impairment.There was no difference in classification of cognitive impairment between bvFTD patients and PPA patients.The degree of cognitive impairment in SD patients is lighter than that in PNFA patients.The naming function of bvFTD patients was significantly higher than that of patients with PPA,and the recognition function of SD patients was significantly higher than that of PNFA patients.Loss of motivation,indifference,and dyslexic comprehension are more than 80%of the first occurrence and can be used to identify the symptoms of FTD.About 12-18%of FTD patients also sufferred ALS.MRI plain scan showed different degrees of frontal/temporal atrophy and left hemisphere atrophy.The bvFTD is mainly atrophy of the temporal lobe and the frontal pole.PNFA was mainly atrophy of the lateral cleft of the brain.SD was mainly atrophy of the frontal part of temporal lobe of the brain.FTD hippocampus did not shrink or slightly shrink.Electroencephalography and cerebrospinal fluid examination are not specific. |
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