Background: A presentation of upper motor and lower motor clinical signs without sensory impairment may suggest Amyotrophic lateral sclerosis, especially when it involves limbs and bulbar regions. However, co-existence of two or more unrelated pathologies can give rise to a clinical picture similar to Amyotrophic lateral sclerosis.
Case presentation: A 45 year old lady presented with slowly progressive right upper limb weakness for 6 months and mild left upper limb weakness for 2 months associated with muscle twitching. Her family members have noticed a change in character of her speech for few weeks. She couldn’t recall a radicular pain at any given time. On examination, her right deltoid was wasted with numerous fasciculations over right proximal muscles. Right shoulder movements were weak. Her left deltoid was slightly wasted and fasciculations were appreciated. Her both biceps and supinator jerks were normal with exaggerated triceps jerks and positive Hoffman sign. Sensory and proprioception examinations were unremarkable. Both lower limbs were hypertonic with exaggerated knee and ankle jerks. Ankle clonus was absent and plantar responses were equivocal. Her tongue was deviated to right side with fascicuations and wasting of right side. Palatal movements and Jaw jerk were normal. She was clinically diagnosed as possible amyotrophic lateral sclerosis. Electromyography showed denervation changes in deltoids and biceps with right predominance and right genioglossus muscle too showed denervation changes. Magnetic resonant imaging of brain stem was undertaken especially because her tongue wasting and fasciculations were unilateral and it showed a right sided hypoglossal schwannoma. Magnetic resonant imaging of cervical spine revealed degenerative disk disease with mild cord compression and exit foramina narrowing at multiple levels.
Conclusions: Pure motor symptoms with a mixture of upper motor and lower motor signs may suggest a clinical diagnosis of Amyotrophic lateral sclerosis. However, when there are rare manifestations of a relatively common disease, such as unilateral tongue involvement as in the above scenario, a higher degree of clinical suspicion is needed to think of a dual pathology.
Keywords: Hypoglossal schwannoma, cervical myeloradiculopathy, Amyotrophic lateral sclerosis, dual pathology.