The patient is a 76 year old Hispanic male who was transferred to our hospital for evaluation for normal pressure hydrocephalus. There is no past medical history. There is no family history of neurological disorders.
He was in his usual state of good health until about six months prior to his presentation at which time he developed subacute, progressive memory loss, and gait disturbance with imbalance. The imbalance led to a fall on the stairs in which he hit his occiput and had a contrecoup contusion and hemorrhage of the bilateral frontal lobes one month prior to his presentation. Imaging at this time was significant for T2 Fluid Attenuation Inversion Recovery (FLAIR) hyperintensities in bilateral frontal lobes as well as 2.42 mm right convexity subdural hemorrhage are seen on MRI in Fig. 1. Following an admission for his traumatic brain injury he went to inpatient rehabilitation. His rehabilitation course was complicated by progressive drowsiness. Lumbar puncture was performed, which his family reports resulted in him becoming more awake and cooperative. Hence, post traumatic hydrocephalus was considered and he was transferred to our hospital for higher level of care.
At the time of transfer to our hospital he had waxing and waning mental status with about five minute episodes of depressed mental status with closed eyes, not following verbal commands, but withdrawing to noxious stimuli and increased tone in the upper extremities bilaterally, which was bordering on catatonic. He did not have any verbal output and did not follow verbal commands during these episodes. Lifting of either extremity by the examiner led to him holding up that extremity for about three seconds before slowly dropping it back down. He was previously evaluated by the psychiatry department at the sending institution and catatonia was considered. He was given a benzodiazepine trial (Valium 5 mg single dose) with the family reporting absence of improvement. He was also noted to have chorea of both hands and feet which was moderate continuous.
He subsequently had five minute episodes of clearer mental status during which he had open eyes and he could intermittently follow one step commands such as to perform finger taps and finger to nose testing. He was also oriented to self and hospital. He was able to provide brief, up to two word, answers to verbal questions. There was paucity of spontaneous speech.
For the frontal release signs; the glabellar sign was positive, palmomental sign was negative bilaterally, and orbicularis oris (snout) reflex was negative. The rest of the examination was the same.
On cranial nerve examination, during the depressed mental status episodes he did not have horizontal or vertical pursuit but he did have normal oculocephalic response horizontally and vertically. During improved mental status episodes as well as after repeat lumbar puncture at our hospital, he had apraxia of horizontal gaze on verbal command but he could perform horizontal pursuit towards the right and left. He also would spontaneously have slow saccadic movements horizontally to a vertically moving stimulus. He could not perform vertical gaze movements on command. There was no vertical upward gaze on pursuit. There was severely diminished downward gaze on pursuit. He was also noted to have a decreased blink rate and at times an eyelid -opening apraxia.
During all levels of mental status, he had diminished, but still present horizontal optokinetic kinetic nystagmus (OKN). There was an absence of upward and downward OKN. There was horizontal nystagmus while the patient was provided upward and downward moving stripe stimulus during vertical OKN testing. There was normal oculocephalic response with vertical and horizontal head tilt in all directions (Video 1).
He received a lumbar puncture with opening pressure of 10 CM H2O, glucose normal at 51 mg/dL, 4 white blood cells per mm3, 2 red blood cells per mm3 and mild elevation in protein to 46 mg/dL (normal 15–45 mg/dL). Cerebrospinal fluid (CSF) lactate dehydrogenase was normal at 24 units / L. CSF analysis showed a normal proportion of 89% lymphocytes as well as 0% lymphocytes and low monocytes at 11% (normal 16–56%). CSF Gram stain and bacterial cultures were negative.
He underwent further extensive workup for his decline in mental status.
Copper level was normal at 120 mcg/dL and ferritin level was normal at 356.1 micrograms/L. Total iron was low at 38 mcg/dL (normal 60–160 mcg/dL), total iron binding capacity was low at 160 mcg/dL (normal 275–365 mcg/dL), and UIBC was low at 122 mcg/dL (normal 136–286 mcg/dL). Iron percent saturation was normal at 24%.
Serum IgG4 level was normal at 14.1 mg/dL. Serum carcinoembryogenic antigen was normal at 1.5 ng/mL. Serum red blood cell morphology was normal. MRI brain showed eye of the tiger sign (Fig. 2) and midbrain atrophy (Fig. 3) [3].
After this lumbar puncture, he remained in the clearer mental state continuously. He was alert and oriented to person and place with paucity of verbal output. Three word responses to verbal questions were present and he was following one step commands with eyes mostly open, but intermittently manifesting apraxia of eyelid opening and horizontal gaze apraxia.
At this time, the patient could perform only the first step of the Luria sequence with the right hand. He could not perform any steps of the Luria sequence with the left hand. He had continuous stereotypic hand wringing movements bilaterally and continuous chorea of the feet. The patient was more attentive and engaging, however chorea remained present at a moderate level after the administration of Carbidopa levodopa 25/ 100 mg tablets three times a day. He had improvement of upper extremity rigidity from moderate to mild post carbidopa / levodopa initiation.