Geniospasm: A systematic review on natural history, prognosis, and treatment

Recurrent Nocturnal Tongue Biting in a Case of Hereditary Geniospasm

“…Hereditary geniospasm is a rare condition with less than 500 reported cases in the literature. 2 Although geniospasm is hereditary with an autosomal dominant inheritance pattern, no causative gene has yet to be identified. 1 Within these cases of geniospasm, 9-12% also have associated RNTB 1,2 which range from mild, with small lacerations of the tongue, to severe, as in this case, with amputation of a portion of the tongue with loss of speech, weight loss, sleep fragmentation, and persistent irritability.…”

“…2 Although geniospasm is hereditary with an autosomal dominant inheritance pattern, no causative gene has yet to be identified. 1 Within these cases of geniospasm, 9-12% also have associated RNTB 1,2 which range from mild, with small lacerations of the tongue, to severe, as in this case, with amputation of a portion of the tongue with loss of speech, weight loss, sleep fragmentation, and persistent irritability. 1,[3][4][5][6] Onset of RNTB in geniospasm often occurs between 9 and 18 months of age and decreases during early childhood.…”

“…BoNT is the preferred treatment with doses ranging from 5 to 60 units to each mentalis. 2 In the case of RNTB, treatment can involve benzodiazepines, BoNT to the masseters (and potentially temporalis muscles), and in severe cases such as this, a combination. [1][2][3][4][5][6] This patient experienced several complications due to the severity of his RNTB to include decreased speech with failure to progress in language development, weight loss, irritability, and sleep fragmentation.…”

“…1 Generally, its course is believed to be benign, and treatment is often sought due to social embarrassment. 1,2 Rarely, significant complications may occur. Specifically, recurrent nocturnal tongue biting (RNTB), can be associated with geniospasm and is often the presenting complaint in children seeking treatment.…”

“…Specifically, recurrent nocturnal tongue biting (RNTB), can be associated with geniospasm and is often the presenting complaint in children seeking treatment. [1][2][3][4][5][6] The association of RNTB and hereditary geniospasm as well as urgency of treatment is often underrecognized.…”