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 |
| Â | Self mutilation (65%) | Aspiration pneumonia | Self mutilation (88%) |
| Â | Delayed development | Breech presentation (37%) | Â |
| Â | Â | Hypothermia | Â |
| Â | Â | Delayed development | Â |
Unique features | No axon flare | No axon flare | No axon flare |
| Â | Lack of fungiform papilla | Lack of fungiform papilla | Anhydrosis |
| Â | Hearing loss (30%) | Alacrima | Consanguinity 50% |
Sensory dysfunction | Â | Â | Â |
   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 | Â | Â | Â |
   Gastroesophageal Reflux | Frequent (71%) | Frequent (67%) | Uncommon (24%) |
   Postural hypotension | Uncommon (25%) | Almost consistent (99%) | Uncommon (29%) |
   Episodic hypertension | Rare | Frequent | Rare |
Ectodermal features | Â | Â | Â |
   Dry Skin | No | No | Consistent |
   Fractures | 29% | 40% | 71% |
   Scoliosis | 59% | 85% | 23% |
Intelligence | Â | Â | Â |
   IQ < 65 | Common (38%) | Uncommon (10%) | Common (33%) |
   Hyperactivity | Common (41%) | Uncommon | Common (54%) |