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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%)
  1. Rare < 1%
  2. Infrequent < 10%
  3. Uncommon < 30%
  4. Common 30–65%
  5. Frequent > 65%
  6. DTR = deep tendon reflexes