A Case of Frontotemporal Dementia with Amyotrophic Lateral Sclerosis Presenting with Pathological Gambling
A 53-year-old male retired government employee with a highest education level of graduation from primary school was admitted to the psychiatric outpatient clinic due to excessive gambling, personality changes (lying, selfishness, and not caring for family members), and large financial debts to both other individuals and several banks in October 2007. He had retired 5 years previously, since when he had spent most of his time betting on horse racing. He had gradually become moody, nervous, and childish, and exhibited verbally and physically aggressive behavior toward his wife. He did not care about his personal hygiene. He was first referred to our clinic in May 2007, with symptoms of irritability, anger, and washing his hands frequently. He did not report his gambling behaviors and he was diagnosed as "anxiety disorder, not otherwise specified". He initially received citalopram (40 mg/day), to which olanzapine (2.5 mg/day) was later added. The patient was concurrently admitted to the neurology outpatient clinic due to difficulty in walking resulting from muscle cramps and weakness in his legs. His neurological symptoms had been present for approximately 1 year. He had a nasal voice, bilateral distal upper limb paresis (Grade IV), bilateral positive Hoffmann and Babinski reflexes, and bilaterally increased deep tendon reflexes (both in upper and lower limbs) in a neurological examination. The electromyogram (EMG) revealed normal neuronal conduction, but in needle EMGs there were frequent fasciculations, fibrillations, and high-amplitude polyphasic motor-unit potentials in the upper and lower extremities (biceps, triceps, I.dorsal interosseous, abductor pollicis brevis, vastus medialis, tibialis anterior muscles, and bilateral) and the thoracic paraspinal muscles (T-5, -8, and -10 paraspinals). These findings were considered compatible with the common motor neuron involvement. The results of blood and urine tests were normal, so the case was diagnosed with "clinically probable-laboratory supported ALS" according to the El Escorial Criteria for the Diagnosis of ALS.\nHis medical history and his family history were not significant.\nA mental status examination performed in October 2007 was characterized by apathy, irritability, decreased amount of speech, inappropriate affect, and psychomotor retardation. A neurological examination revealed nasal voice, bilaterally decreased gag reflex, increased deep tendon reflexes, bilaterally positive Hoffmann, palmomental, Babinski, and glabellar reflexes, postural instability, and a positive Romberg test. A detailed neuropsychological assessment was performed using the Standardized Mini Mental Test, Clock Drawing Test (CDT), Frontal Assessment Battery (FAB), Alzheimer Disease Assessment Scale-cognitive (ADAS-COG), Trail-Making Test A (TMT-A) and B (TMT-B), WCST, Wechsler Adult Intelligence Test-Revised Form (WAIS-R), and Iowa Gambling Task (IGT).- The scores in the neuropsychological tests are presented in . He had severe deficiency in frontal lobe functions such as judgement, decision making, planning, concept formation, attention, similarities, verbal fluency (as assessed on the subscales of the WAIS-R, FAB, TMT-A/B, WCST, IGT, and CDT), and mild deficiency in temporal lobe functions such as verbal recall and recognition (as assessed on ADAS-COG subscales). These results were indicative of severe dysfunction of frontal brain regions and mild dysfunction of temporal brain regions. Cranial MRI revealed frontotemporal atrophy with several ischemic gliotic lesions in the posterior periventricular and subcortical white matter. T2-weighted coronal and horizontal MRI images of the patient are shown in .\nThe case was diagnosed as FTD with ALS. Citalopram and olanzapine were stopped during follow-up since they were not providing any benefit, and were replaced by paroxetine (30 mg/day) for PG and other disinhibition behaviors, and by riluzole (100 mg/day) and baclofen (30 mg/day) for ALS. His inappropriate affect and irritability decreased and he stopped gambling. However, his fasciculations (both in upper and lower limbs), weakness (in lower limbs), and difficulties in walking and talking worsened within 2 years. At his last visit in November 2010 he could not walk without assistance and his score on the ALS Functional Rating Scale was 29 out of 40.