Table 6.

Recommended Surveillance for Individuals with RVCL-S

System/ConcernEvaluationFrequency (Minimum)
Eyes Ophthalmologic evaluationAnnually starting in 4th decade or as appropriate based on symptoms
Cardiovascular Blood pressure evaluation
Renal Renal function tests 1
Endocrinologic TSH and free T4
Hematologic Complete blood count
Hepatology Liver enzymes 2 & serum albuminAnnually starting in the 4th decade
Neurologic Assessment of cognitionAnnually or as appropriate based on symptoms starting after diagnosis
Psychiatric Assessment for psychiatric symptoms
1.

Serum creatinine, BUN, and urinalysis (to include creatinine and protein content)

2.

Aspartate transaminase (AST), alanine transaminase (ALT), alkaline phosphatase, gamma-glutamyltransferase (GGT)

From: Retinal Vasculopathy with Cerebral Leukoencephalopathy and Systemic Manifestations

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