November/December 2015 Case Challenge: A 70-Year-Old Man with a Sudden Lisp
By Mohanad Alzubaidi, MD and Aziz Shaibani, MD, FACP, FAAN, FANA
A 70-year-old man presented with a six-month history of a "lisp," noted by his friends, that got worse the more he spoke. There was no diplopia, ptosis, dysphagia, muscle wasting, or extremities weakness. Since he was stressed, it was considered psychogenic. AChR antibody titer was high and lingual dysarthria responded to steroids.
Lingual dysarthria can be a feature of:
1. Myasthenia gravis (MG)
2. Amyotrophic lateral sclerosis (ALS)
3. Hypoglossal neuropathy
5. Lambert-Eaton myasthenic syndrome (LEMS)
The answer is all of the above.
The tongue consists of four pairs of extrinsic and four pairs of intrinsic muscles. Tongue movement is served by the hypoglossal nerve.
Tongue weakness may present as dysarthria or inability to move food in the mouth properly. It is a feature of several neuromuscular disorders, including muscle diseases (polymyositis), nerve diseases (GBS), Neuromuscular junction (NMJ) disorders (MG, LEMS, Botulism), motor neuron disease (ALS, Kennedy’s disease), as well as mitochondrial disorders like Sensory Ataxia, Neuropathy Dysarthria, Ophthalmoplegia (SANDO).
Bulbar weakness without prominent ocular symptoms is not unusual in MG. As a matter of fact, it is a common manifestation of MuSK antibodies associated MG where atrophy of the tongue and face may lead to diagnostic confusion with bulbar onset ALS. Even tongue fasciculations are reported in MuSK antibodies associated MG.
Fatigability of dysarthria (worsening with speech) is more typically seen in MG.
In MG, the tip of the tongue is often affected early, leading to a “lisp,” as opposed to ALS, in which the bulk of the tongue is affected and leads to a “heavy tongue,” like the tongue of an intoxicated person.
Cerebellar dysarthria is irregular. The patient cannot count fast with regular intervals and consistent volume.