| Description |
Clinical Signs: saccadic oscillations about primary @ 5 hertz, 10-15 degrees; head and arms show myoclonic activity; blurred vision. 1-23-81: 19 year old student who is being studied for a profound neutropenia. Has had lib ulcers and treated with penicillin. He also had a splenectomy. A few days ago had oscillopsia which is now improved. Also had a fever for 1-2 days. Now shows profound unsteadiness considerable myoclonus, and truncal ataxia. Eyes show occasional opsoclonic movements horizontal and vertical and blinking of lids. These were not shown well at time of video. Tongue is markedly tremulous but palate unclear. Is this benign encephalitis or related to neutropenia? 1/23/81: 19 year old white male, student/summer bricklayer with neutropenia with serum inhibitor (WBC 10.9, Lymphocytes 47, Monocytes 47, Eocinophil 6) and recurring infections. Now has opsoclonus and truncal ataxia. Initially anemia in 10/79, but by January 80 he had granulocytopenia and thrombocytopenia. Had positive ANA and rheumatoid factor. Rx high dose IV antibiotic. Platelets resolved by 3/80. Splenectomy in 4/80 and Prednisone 100 mg, but persistent granulocytopenia. Then to NIH 9/80. Demonstrated anti-PMN antibodies (10/80). Marrow: (9/80 and 12/80) many mature cells (ess. Normal) except for poor granulocyte maturation. Marrow in vitro no inhibition of maturation. Most recent episode: struck in mouth January 4, 1981, febrile, readmitted on Ampicillin. Pneumonia/coarse tremulousness (episodic jerks of hands, legs and body esp. on standing (1/10/81). Decided to stop penicillin 2 days later (for presumed toxicity.) Pat Moore: Encephalopathy (confusion) tremulousness and possibly myoclonus, horiz. Gaze nystagmus. CSF: OP150; prot. 50, glu 58; Ig G 9.8 (0-3.5); lymphs 3. Plasmaphoresis started 1/21/81 (c/o blurring, increased tremulusness). EEG: diffuse slowing. Head CT scan WNL (1/22 and 1/24/81). They are looking for an infection focus. At nite temperatures up to 38° or 39°. For the last few days dancing images with infrequent blurring and diplopia. No seizures, HA, tinnitus. Plan Cefazolin and/or cytoxin. Vision good (est) VF full to confrontation. Lids normal. At time steady fixation and then irregular trains of conjugate, saccadic eye movements of low amplitude (2-5 degrees) and about 2-5 Hz. May be elicited by change of fixation. Infrequently downbeat nystagmus and gaze evoked nystagmus horizontally. DVM normal. Pupils normal. Tongue- irregular vermiform movments. Much body ataxia/tremulousnous in standing or FTN. Imp: benign encephalitis (truncal ataxia, opsoclonus). Note: Pat Moore says this is not truncal ataxia. 1/24/81: Two hours after a LP. may have witnessed a "severe" episode. Much startle myoclonus; tremulousness only effort. Initially down beating 1-2 Hz at 5 degrees amplitude. Then Oculogyral crises superimposed at 30-40 sec. intervals lasting 5 seconds. Next wildly chaotic bidirectional saccadic oscillations (3 to 5 Hz and 10-20 degrees amplitude), mostly vertical; few upward crises; also convergence spasm. Lastly subsidence. 1/28/81: Witnessed the beginning of approximately 48 hours of seizing. One typical grand episode witnessed as follows: 9:35 AM 1. Right eye intense blinking (like a left facial): 2. Gaze left; 3. slight arm activity; 9:45 AM (4) both pupils dilated; (5) mass motor activity foaming; (6) eyes centered, then intermittent, conjugate beating rightwards; (7) eyes in upgaze, many incomplete blinks with eyelid fasciculations (quietly); 10:10 AM then continuous as in # (7). Pupils mid-position, disks flat. Today LE prep, ANA, Rheumatoid factors positive. It's felt the patient has lupus cerebritis. 1/30/81: Saw Mike today for first time in 48hrs. Not seizing Comatose. PERLA. Eyes straight, quiet, no blinking. Doll's H/V. Disks flat. 2/2/81: AER/VER normal. Diffuse EEG changes, no seizure activity. Exam unchanged. Dolls and calorics (slow phase only.) Eyes open- no blinking. 2/3/81: Interesting episode of intense miosis today (2mm) OU, face turn right, eyes right. When I straightened head (against resistence) the pupils dilated to mid-position and reacted to light sluggishly OU. Dolls: no movement. Disks flat. Eyes closed, incomplete to complete blinks infrequently (once per 30 seconds). Eyes up 30 degrees. 2/5/81: Unconscious still. Perla. Eyes ahead or drift slowly downwards 10 degrees. Rarely groups small conjugate upward beating movements (< 5 degrees/2 Hz) intermittently. Disks flat; no cotton-wool. 2/9/81: Unconscious. Intermittent, slow 5 to 20 degrees drifts in no particular direction (at times vertically or laterally). Blinks about once or twice per minute. PERLA. Disks flat. 2/13/81: Intermittent inverse ocular bobbing (103Hz) from 30 degrees to 45 degrees below midline: slow down, fast up. Infrequent complete, generally incomplete blinks. PERL. DVM normal. Cold calorics each ear: brisk nystagmus (fast opposite). Cold both ears (twice tried): right beating only. (On video). Gentle unidirectional head rotation: nystagmus appropriate that fades after 5 seconds. Vertically only can get slow phase contraversion. Anatomy: Posterior cranial fossa. Pathology: Lesions of posterior cranial fossa. Disease/ Diagnosis: Posterior Fossa Lesions. Imaging: cranial CT scan w/contrast. |
