Amyotrophic lateral sclerosis presenting with apraxia of speech.

Speech disturbance observed in patients with amyotrophic lateral sclerosis (ALS) usually arises from bulbar palsy. In those patients, atrophy and fasciculation of the tongue are observed and dysphagia might be complicated with speech disturbance in parallel. We herein describe an unusual woman with ALS whose initial symptom was apraxia of speech (AOS). A right-handed 66-year-old woman presented with a speech problem lasting for 6 months. She went to a dental clinic, departments of otolaryngology and neurosurgery at other hospitals, and a psychological clinic, but there was no abnormality detected in their examination. She was unable to speak or vocalize at all when she came to our hospital. There was no neurological disease in her family. Her parents were not consanguineous. She was alert and could walk by herself. There was no weakness of her face. Atrophy and fasciculation of the tongue was not observed. She did not complain of dysphagia. She could eat and drink anything without difficulty. There was no weakness in any of her limbs. Tendon reflexes were evoked normally. No pathological reflex was elicited. Sensory function and coordination were also normal. She could understand what we said. She could obey written commands. Although she could not speak, she could write what she wanted to say. Laryngoscopy and brain magnetic resonance imaging results were normal. Electromyography did not show any denervation potentials in muscles including the tongue. Single-photon emission computed tomography (SPECT) using I-N-isopropyl-P-iodoamphetamine revealed hypoperfusion at the middle and lower parts of the left precentral gyrus when analyzed with easy Z-score imaging system (Fig. 1) [1]. No objective examination indicated ALS at that time. One year after we had seen her for the first time, she came to our hospital requiring re-examination. There was no facial palsy, but forced laughing was observed. Atrophy and fasciculation of the tongue was seen. She did not complain of dysphagia. There was a weakness of her left lower limb. Tendon reflexes including jaw jerk were increased. Babinski sign was evoked bilaterally. The grammar of her sentences and morphology of her handwritten letters including kana (Japanese syllabograms) and kanji (Japanese ideograms) were almost correct. However, there was a phonological error in her handwriting (Fig. 2). Nerve conduction study showed decreased F wave frequency without conduction block. Electromyography revealed acute and chronic denervation potentials in her limbs. She was diagnosed as having ALS. In the next 3 years, she developed tetraplegia. Dysphagia, which developed slowly, required gastrostomy. Brain computed tomography 5 years after the onset showed atrophy of the medial temporal lobes but not the precentral gyri. Our patient’s initial symptom of speech problem was considered to be AOS. AOS is an impairment of motor planning and programming of speech articulation. Since K. Maeda (&) N. Ogawa R. Idehara Department of Neurology, National Hospital Organisation Higashi-ohmi General Medical Centre, 255 Gochi, Higashi-ohmi, Shiga 527-8505, Japan e-mail: maeda-kengo@shiga-hosp.jp