Treatment
Table 5 lists symptomatic treatments tested in FTD trials. Prior etiologic treatments have either proven toxic or nonefficacious. For more details on FTD therapies, see also the review in the current issue by Tsai and Boxer, Clinical Trials: Past, Current, and Future for Atypical Parkinsonian Syndromes. In general, selective serotonin reuptake inhibitors are mildly beneficial for compulsions and eating disorders. Dopaminergic medications have no definite behavioral benefit. Recent trials do not support the use of memantine, and cholinesterase inhibitors seem to worsen behavior. Atypical antipsychotics should be used with caution only in cases of severe agitation given their extrapyramidal side effects. Levodopa may be considered in parkinsonism, especially where tau pathology or MAPT mutations are suspected, but a sustained response is rarely present.
A promising etiologic therapy focuses on halting tau spread using anti-tau antibodies, which in animal models decrease protein accumulation and improve behavior. Antisense oligonucleotides are being studied in C9Orf72 mutations. There is a single report of steroid treatment improving symptoms in svPPA, highlighting its association to autoimmunity. Finally, treatments that raise progranulin levels are in development for GRN mutations.
Nonpharmacological management of FTLD is as important as are pharmacological therapies. Family education and respite, a regular sleep schedule, social worker involvement, driving evaluation, exercise, and speech therapy can improve patients' and families' quality of life. Thus, a multidisciplinary dementia clinic is the optimal setting for management of FTLD. Table 6 contains information on foundations and support groups for FTLD.