Case History #181
Date of Consultation: 5-15-1989
HISTORY OF PRESENT ILLNESS:
This 64-year-old right-handed white male retired engineer was referred for neurologic evaluation due to his mental status changes and difficulties with movement. The patient was well until four years prior to consultation (1985), when he and his family noted the gradual onset of difficulties with memory and an inability to function on the job. Specific symptoms included the inability to recall where papers had been filed and difficulty playing chess. The patient was previously a competitive chess player. Around the same time, the patient noted problems with sleeping, feeling nervous, anxious, and restless, and was somewhat agitated. At this point his physicians felt that he was suffering from an agitated depression, and he was tried on a variety of antidepressant and neuroleptic medications over the subsequent two to three years without any lasting benefit.
Approximately one year after the onset of the above symptoms (December 1986), members of his family noticed a gradual change in his voice in that it became higher pitched, more monotonous, and of low volume. In early 1987, his wife noted that his movements in general began to slow down and that his dexterity for fine movements with his hands, such as buttoning his shirt or manipulating fine objects, also diminished. Approximately 9 months prior to consultation (August 1988), he began having difficulty with frequent loss of balance and even falling. He specifically denied any dizziness, ataxia, weakness, or tendency to trip over objects when discussing these falling episodes. He stated that all of a sudden he loses his balance for no apparent reason and falls. He also noted that he seems to fall to the left and somewhat forward. At about this same time, his gait seemed to slow down, and he began to experience difficulty with turning rapidly. There was also a festinating quality to his gait, especially if he was walking downhill. In the six months prior to consultation, the patient felt that his arms and legs were becoming stiff and somewhat rigid. He denied any tremor of head and neck, upper limbs, or lower limbs.
Approximately one year prior to consultation (May 1988), the patient’s wife noticed that he would suddenly begin to laugh for no reason at all. The patient indicated that he had no control over this sudden laughter. Other symptoms present over the past year included difficulty with mathematics, a tendency to maintain unusual postures with his upper limbs, an inability to keep track of his feet when he was walking, and not cleaning up the bottom half of his plate when he was eating.
The overall course of this problem was a slow, gradual progression from late 1985 up to the present time. During the four to five years prior to consultation, the patient underwent a number of neurological and laboratory studies including neuroimaging studies and EEGs, all of which were unremarkable.
PHYSICAL EXAMINATION:
Vital signs were stable. Upon general inspection, the patient appeared to have a masked facies with a paucity of facial movements. When sitting quietly, he did not have much in the way of normal associated movements such as eye blinking and spontaneous facial expressions. His eyes tended to be held widely open, and he exhibited a blank stare on his face.
NEUROLOGICAL EXAMINATION:
Mental Status Exam: The patient was oriented x3. His fund of general information and long term memory were adequate. Short term memory testing revealed the ability to recall two out of three simple items after three minutes and three out of three items after ten minutes. He was able to perform serial sevens without difficulty. There was no evidence of aphasia, apraxia, or agnosia. Simple bedside tests for judgement were answered appropriately. The patient tended to abstract somewhat concretely. In general, the patient’s mood was felt to be normal and appropriate. However, on three or four occasions during the interview and examination, he had a rather dramatic outburst of pathologic laughing.
Cranial Nerves: Sensation of smell was intact bilaterally. Visual acuity was J7 in the right eye and J5 in the left eye without his glasses. Visual field and funduscopic examinations were unremarkable. External ocular movements were full in horizontal gaze and downgaze with pursuit movements. There was definite limitation of upgaze with smooth pursuit movements. In addition, smooth pursuit movements were performed with so-called “saccadic pursuit” or “cogwheeling”. Saccadic eye movements were performed fully and with normal velocity in the horizontal direction. Upgaze saccades were severely limited, and downgaze saccades were also slowed. With oculocephalic maneuver, one could demonstrate a full range of vertical and horizontal eye movements. Pupils were equal, round, and reacted normally to light and accommodation. Sensory and motor functions of the Vth cranial nerve were normal. There was no facial weakness. Hearing was intact bilaterally. XIth cranial nerve functions were normal. Tongue and palate appeared normal.
Motor System: There was no evidence of focal or generalized muscular atrophy. There were no fasciculations. Muscle tone was increased in all four limbs with the left side being slightly worse than the right. This increased tone was of the lead pipe or plastic rigidity variety. Muscle power was normal in all four limbs. There was no tremor present at rest or with purposeful movement.
Reflexes: Deep tendon reflexes were slightly brisker in the left upper limb than the right. At the knees, the reflexes were symmetric, and ankle jerks were absent bilaterally. Plantar reflexes were flexor bilaterally. There was a positive glabellar tap reflex, snout reflex, left-sided palmomental reflex, and grasp reflex bilaterally. His jaw jerk was 2+ and not pathologic.
Sensory System: Intact.
Cerebellar Function: Intact.
Gait and Stance: Regular gait was performed on a slightly widened base. The patient walked fairly briskly and did not have a stooped or shuffling gait. His arm swing was diminished but was by no means absent. Tandem gait, however, was very difficult for the patient, and he tended to fall both to the right and the left. The Romberg test was negative
Head and Neck Exam: Neck range of motion was somewhat limited in all directions but was exceedingly limited in neck extension. There was increased muscle tone in all directions.
Questions:
1. Considering the patient’s history and examination, how precisely can the neurologic lesion be localized in this case?
2. This patient’s signs and symptoms constitute a classical neurologic syndrome. What is the name of that syndrome? This syndrome is one of a few other syndromes, all of which are included in a larger group of syndromes. What is the name of that group of syndromes?
3. Indicate the level of the neurologic lesion in this case as precisely as possible and the structures involved by the pathologic process. If precise localization is not possible, list as many levels or structures as you can that might produce the patient’s clinical signs and symptoms.
4. This patient’s sudden outbursts of laughter are characteristic of a classic neurologic symptom complex. What is the name of that symptom complex? What does it usually signify? In this patient, what other examination findings are indicative of this symptom complex?
5. In general, what type of pathologic process do you think is involved in this case?
6. What diagnostic procedure(s) would you undertake at this point?