Table 11.

Recommended Surveillance for Individuals with Acute Infantile Sandhoff Disease

System/ConcernEvaluationFrequency
Neurologic decline By pediatric neurologist w/attention to seizure severity & response to ASMEvery 3-6 mos
Abnormal tone /
Impaired mobility
  • OT/PT assessment of ADL; need for splinting for contractures/scoliosis
  • Durable medical equipment for mobility
At each visit
Nutrition/feeding By feeding team re aspiration risk / nutrition needs
Respiratory Assess need for airway hygiene.
Family support
& resources 		Assess need for:
  • Social work involvement for parental support;
  • Palliative care referral;
  • Home nursing referral.
As needed

ADL = activities of daily living; ASM = anti-seizure medication; OT = occupational therapy; PT = physical therapy

From: Sandhoff Disease

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