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Documentation Required
No explicit documentation requirements are stated in this excerpt. The policy text references commonly used clinical assessments and tests frequently used in trials and practice, which are typically documented when evaluating treatment effect or safety, including:
Baseline and follow-up Unified Huntington's Disease Rating Scale (UHDRS) motor and chorea subscores (used as primary motor/chorea outcome measures in multiple studies).
Cognitive assessments: Mini-Mental State Examination (MMSE), Alzheimer Disease Assessment Scale-cognitive subscale (ADAS-cog), Symbol Digit Modalities Test (SDMT), Stroop tests, verbal fluency tests (used as trial endpoints).
CSF sampling for measurement of mutant huntingtin concentration (as used in HTTRx trials) and/or neurofilament light (NF-L) measurement in plasma/CSF (investigational biomarkers described).
Key Coverage Criteria
None explicitly stated. This Clinical Policy Bulletin addresses selected interventions for Huntington's disease and identifies numerous interventions and tests that Aetna considers experimental and investigational (see Limitations).
If HD chorea requires treatment, clinicians should prescribe tetrabenazine (up to 100 mg/day), amantadine (300 to 400 mg/day), or riluzole (200 mg/day) (Level B).
Clinicians may also prescribe nabilone for modest decreases (1- to less than 2-point changes on the UHDRS chorea score) in HD chorea (Level C), but information is insufficient to recommend long-term use.
Deutetrabenazine (a deuterated form of tetrabenazine) was recently approved for the treatment of chorea in HD; administered twice-daily up to a maximum daily dose of 48 mg.
Electroconvulsive therapy (ECT) has been reported to be well-tolerated and to produce improvement in psychiatric and behavioral symptoms (depression, psychosis) in HD in a small case series.
Percutaneous endoscopic gastrostomy (PEG) tube placement in advanced HD: one retrospective review reported increased length of life (in deceased patients insertion of a PEG tube increased length of life with HD by 3.6 years), although nutritional and other outcomes were mixed.
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Neuroimaging reports when relevant: MRI (volumetrics, MRS in research settings), PET (e.g., 11C-UCB-J or 18F-FDG) as used in research and biomarker assessment; MRI guidance for DBS implantation procedures where performed.
Safety monitoring documentation where noted in referenced guidance or trials: for example, monitoring for depression/suicidality and parkinsonism with tetrabenazine; monitoring liver enzymes with riluzole; adverse event reporting for investigational therapies.