Table 2 Major clinical features of HSAN II, HSAN III, and HSAN IV
From: Hereditary sensory and autonomic neuropathies: types II, III, and IV
| Clinical features | HSAN type II | HSAN type III | HSAN type IV |
|---|---|---|---|
| Onset | Birth | Birth | Birth |
| Initial symptoms (From birth to age 3 years) | Swallowing problems | Swallowing problems | Fevers |
| Self mutilation (65%) | Aspiration pneumonia | Self mutilation (88%) | |
| Delayed development | Breech presentation (37%) | ||
| Hypothermia | |||
| Delayed development | |||
| Unique features | No axon flare | No axon flare | No axon flare |
| Lack of fungiform papilla | Lack of fungiform papilla | Anhydrosis | |
| Hearing loss (30%) | Alacrima | Consanguinity 50% | |
| Sensory dysfunction | |||
| Depressed DTR | Frequent (71%) | Almost consistent (99%) | Infrequent (9%) |
| Pain perception | Absent | Mild to moderate decrease | Absent |
| Temperature perception | Severe decrease | Mild to moderate decrease | Absent |
| Vibration sense | Normal | Normal | Normal to moderate decrease |
| Autonomic | |||
| Gastroesophageal Reflux | Frequent (71%) | Frequent (67%) | Uncommon (24%) |
| Postural hypotension | Uncommon (25%) | Almost consistent (99%) | Uncommon (29%) |
| Episodic hypertension | Rare | Frequent | Rare |
| Ectodermal features | |||
| Dry Skin | No | No | Consistent |
| Fractures | 29% | 40% | 71% |
| Scoliosis | 59% | 85% | 23% |
| Intelligence | |||
| IQ < 65 | Common (38%) | Uncommon (10%) | Common (33%) |
| Hyperactivity | Common (41%) | Uncommon | Common (54%) |