TABLE 5.
Prevalence and Timing of Neurologic Signs and Symptoms of Infantile GM2 Gangliosidosis
| Symptomsa | Patients, n (%) | All Patients (n = 92), Age at Onset, Mean ± SD, mo | Tay Sachs Disease (n = 72), Age at Onset, Mean ± SD, mo | Sandhoff Disease (n = 20), Age at Onset, Mean ± SD, mo |
|---|---|---|---|---|
| Exaggerated startle response | 90 (98) | 7.9 ± 6.6 | 8.1 ± 6.4 | 7.2 ± 7.4 |
| Seizures | 90 (98) | 17.4 ± 5.9 | 16.8 ± 5.5 | 19.5 ± 7.0 |
| Diminished eyesight | 78 (85) | 17.9 ± 11.3 | 18 ± 11.6 | 17.6 ± 11.0 |
| Hypotonia | 63 (68) | 10.9 ± 3.9 | 10.8 ± 3.6 | 11.3 ± 5.0 |
| No eyesight | 63 (68) | 28.4 ± 14.7 | 30 ± 14.8 | 16 ± 7.2 |
| Spasticity | 56 (61) | 16.3 ± 11.4 | 17.4 ± 13.2 | 13.4 ± 4.5 |
| Decreased hearing | 45 (49) | 22.8 ± 15.1 | 24.1 ± 13.7 | 19 ± 21.9 |
| Loss of hearing | 31 (34) | 23.8 ± 17.3 | 23.8 ± 17.3 | — |
| Screaming episodes | 31 (34) | 13.9 ± 8.6 | 13.7 ± 8.9 | 14.6 ± 8.4 |
| Dyskinesia | 21 (23) | 18.6 ± 11.5 | 18.3 ± 10.0 | 19.5 ± 17.2 |
| Ataxia | 11 (12) | 16.3 ± 10.1 | 13.8 ± 6.9 | 37 |
a
In decreasing order of prevalence.