Frontotemporal_Dementia

The patient is a 68 year old right handed retired air conditioner repair man who presented with impaired balance and slow walking. For about one year he had noted difficulty lifting his feet high enough when climbing the stairs. From that time on, his movements slowed and worsened so that he had difficulty dressing, using a knife and fork, and getting in and out of a chair. His walk became slow and unsteady and his balance was poor. Because of impaired balance, he needed to sit to put his trousers on. His writing became less legible and his speech slow and hesitant. When answering a question, he often had to repeat the question before giving the answer. The patient denied any visual symptoms except trouble judging space and distance. He reported having a minor car accident when he drove 100 feet in reverse without putting his foot on the brake pedal. He stopped only because he hit his garage door. He had noticed no trouble with memory, no confusion, no difficulty finding words or understanding speech. He also denied weakness, stiffness, tremors, numbness, headache or syncope. He had no visual complaints. Social History: Retired age 65. No history of alcohol abuse. Past History: Negative for cardiac disease, stroke or head trauma. In 1975 (fifteen years ago), he had an attack of the Guillian Barre Syndrome causing total paralysis and difficulty breathing. He was not on a respirator. He returned to normal activity and work in six to eight weeks. Family History: Negative for CNS disease. His family, concerned that his "slowing up" might be due to Parkinson's disease, consulted his primary care doctor. He was then referred to Dr. Raymond Adams for an opinion. On examination his speech was slow and hesitant without paraphasic errors. In following commands or answering questions, he repeated the command or question prior to proceeding (echolalia (repetition of the utterance of others)). He repeated "no ifs, ands or buts" and spelled the word ‘world' correctly but could not spell it backwards. He was fully oriented and remembered the President, Vice President and immediate past President but not who preceded Reagan. He was unable to subtract serial 7's beyond 93. He made one error in the Tom and Bill story after ten minutes. Writing was tremulous and poorly legible, with correct spelling. He was able to draw a clock and bisect a line correctly. He had some difficulty copying a complex drawing. There was no limb apraxia, but his truncal movements seemed apraxic. Tendon reflexes were 1+ and symmetric, plantars flexor. He had frontal lobe release signs. Sensation and coordination were normal. The neurovisual exam documented: Visual acuity 20/30 OU Visual field testing showed impaired localization of a visual stimulus in space on the tangent screen. He pointed lateral to the location of the stimulus target by about an inch both in the right half and left half field and in both superior and inferior quadrants. Pupils equal, normal reflexes Ocular motility: (See below for constellation of eye signs) Mild age-related ptosis Saccadic initiation deficit of unilateral horizontal gaze A complete paralysis of voluntary horizontal saccades on command to look left Inabililty to make on command a refixation saccade to a target held on the left (including his own hand) Normal voluntary horizontal saccadic eye movements to the right No saccadic intrusions No apraxia of eyelid closure No nystagmus Normal optokinetic nystagmus Normal optic discs and fundi OU Brain MRI showed global generalized central and cortical atrophy with frontal cortex and temporal lobes most affected bilaterally. Neuropsychological Testing by Dr. Janet Sherman showed a general depression of intellectual function as well as many specific cognitive deficits. The deficits which the patient displayed were suggestive of frontal lobe damage. Behavioral Observations: He was noted to have greatly diminished verbal output. His speech was slow and hesitant with normal articulation. He was noted to be echolalic consistently repeating what was said to him. During the examination he became agitated even angry at times. Tasks that were difficult for him he refused to do. General Intellectual Function: As assessed by the Wechsler Adult Intelligence Scale-Revised, his full scale IQ was 75 (in the borderline range), verbal IQ 73 and performance IQ 78. These scores are indicative of significantly depressed intellectual function. Visuospatial/Visuomotor Abilities: On the Judgment of Line Orientation Test his performance was severely defective suggesting a significant disturbance in spatial perception and orientation. His constructional abilities were also depressed. His ability to construct block designs copied from a model was in the low end of the low average range. On a number and letter sequencing task, he displayed perseverative movements, for example, going back and forth over already drawn lines. His motor speed on a peg board task was severely impaired. Attention/Memory: His mental control abilities were impaired for rapidly counting backwards from 20 to 1, for rapidly reciting the alphabet and for rapidly counting by 3's from 1 to 40. He was able to repeat only 4 digits forward and only 3 backward. The patient's memory for verbal material, particularly for stories fell in the 52 percentile for immediate memory, but only at the 7 percentile for delayed (20 minutes) recall. In a recognition task, he identified all but 2 of the 16 items on the initial list that was presented, and did not falsely confirm any of the items that were not on the list. His good recognition memory suggested that his memory problems may not be related to encoding or storage of information, but rather to retrieval of that information. Both his immediate and delayed drawings were noted to lack many of the figures' organizational features, particularly as the figures became more complex. Language: The patient's confrontation naming abilities fell 3 standard deviations below the mean for his age level. In addition to his word finding difficulties, his verbal and written fluency was significantly impaired. Abstract Reasoning: His abstract reasoning abilities were severely impaired; he was unable to state how an orange and a banana were alike, and denied that words such as a coat and a suit, a boat and automobile and an eye and ear had any similarity. Summary: The deficits exhibited by this patient were: Greatly diminished verbal output Perseverative tendencies An extreme stimulus boundedness; for example, he picked up any object placed in front of him, such as the examiner's coffee cup or pencil and had difficulty releasing the object A greatly decreased verbal fluency The Cognitive Syndrome displayed: •A general depression in intellectual function •Extreme demonstration of frontal lobe signs suggestive of circumscribed "cortical atrophy" which is often associated with Pick's Disease Dr. Sherman was of the opinion that the intellectual decline which the patient exhibited was less suggestive of Alzheimer's Disease where memory impairments are usually an early feature. She considered the memory impairments that he exhibited were suggestive of a "frontal amnesia", where patients tend to assert that they cannot remember something, but when prompted they produce some responses. The patient often stated he could remember nothing, but when encouraged to reply, or when given cues as in the recognition memory test, his recall was quite good. Dr. Adam's diagnosis was Frontotemporal Dementia - a behavioral syndrome due to degeneration of the frontal and anterior temporal lobes. In advanced cases marked frontal and anterior temporal atrophy is prominent on the medial and lateral views of the affected hemisphere. The initial presentation may be altered by the areas involved. Subjects with frontal involvement have significant apathy, whereas those with temporal involvement have significant hypomanic behavior, puerile and emotional behaviors. Depression and anxiety correlate with right-sided temporal change and irritability and aggressive outbursts with left sided temporal changes. These findings can help in the initial clinical diagnosis of this variably expressed disorder. See also: http://content.lib.utah.edu/cdm/ref/collection/ehsl-shw/id/24