(E) Table
Patient / 1 / 2 / 3 / 4Age in years / 39 / 54 / 34 / 69
Gender / Male / Male / Male / Male
Duration of symptoms before admission / 5 weeks / 2 weeks / 2-3 weeks / 3 days
Presentation / Blurred vision, progressive left hemianopia; recurrent photopsias in his left visual field for 2-5 minutes, every couple of hours, some were associated with severe right-sided headaches that responded to treatment for migraine. / Intermittent blurred vision associated with round colored fluttering lights seen in his left upper visual field; staring spells for 3-4 days before admission. / Brief episodes of right facial twitching, drooling, and RUE shaking, followed by confusion, worsening of pre-existing short term memory loss, bumping into objects particularly on his right side. The episodes of twitching increased in severity and were associated with right periocular pain. The week before admission his confusion increased / Multiple episodes of visual distortion in his “right eye” for three days, described as colored squiggles of light inside a green circle moving in a predominantly downward direction. The episodes occurred about every 10 minutes and lasted 1 to 3 minutes. He had left frontal headache
Weight loss / 25 lbs over 4 weeks / not known / 15 lbs over 3 weeks / 15 lbs ‘recently’
Medication / Amplodine, atorvastatin, captopril, cisapride, clonazepam, clonidine, fluoxetine, and ranitidine / Allopurinol, amlodipine, clonidine, lisinopril and metformin. / Glyburide / Atenolol, coumadin, lisinopril, metolazone, potassium chloride, prednisone 90 mg every other day for the previous three months, pyridostigmine, and ticlopidine.
Past History / Hypertension, hyperlipidemia, lazy eye, depression, panic attacks, psoriasis, renal stones / Right mastectomy for breast cancer, radiation and chemotherapy five years earlier, gout, hypertension. / Type II diabetes mellitus for six years / Generalized myasthenia, ischemic heart disease and coronary artery bypass surgery
Family History / Stroke, diabetes mellitus, “phlebitis” in two brothers, one still on coumadin. / Diabetes, thyroid and vascular disease
Physical exam / Alert, oriented. VA: 20/20 OD and 20/20-1 OS. Impaired color vision OU. Dense left homonymous hemianopia splitting fixation. Funduscopic examination: grade I atherosclerosis. Normal tone, power, coordination, sensation and flexor plantar responses. Brisk (+3) deep tendon reflexes (attributed to fluoxetine). BP: 130/90 mmHg. Pupil stimulation with a Finhoff transilluminator provoked two episodes of photopsias, in his left visual field, each lasting about 30 seconds and associated with prolonged bilateral miosis, left beating nystagmus, and left upper limb tremor. / Alert, oriented. During the exam he had three spells of tonic conjugate leftward eye deviation, each lasting 30-60 seconds, followed by a few beats of left beating nystagmus. During the spells he answered questions hesitantly and appeared distracted; he said he saw round colored fluttering lights moving up to the left. He had a dense left homonymous hemianopia splitting fixation. VA was J2 OD and J1 OS. Pupil exam was normal. His ocular movements were full, although he had a little difficulty finding targets in his left visual field. / Afebrile, thin, appeared ill. He responded to simple commands intermittently. He had no apparent localizing deficit. Initially, acute encephalitis, or cerebritis with seizures, was suspected; acylovir, benzodiazepines and rehydration were initiated. On the second hospital day, following a generalized seizure, he received a bolus of fosphenytoin, followed by maintenance phenytoin. A neurology consul was obtained. At that time he was awake but still disoriented. His speech was fluent but with little spontaneity. He had a dense right homonymous hemianopia His ocular movements were full. He had normal strength, but had drift of his RUE. / He was alert with right incomplete homonymous hemianopia, worse inferiorly. During the examination he reported a number of episodes of seeing unformed visual hallucinations - flashing colored objects and squiggly lines, moving to the right and upward, that were provoked by either attempted fixation, or visual stimulation with a target such as a pen tip. Each time he reported the hallucinations, his eyes tonically deviated to the right so he could not make leftward saccades in response to command. Immediately after the episodes, which lasted about 1 ½ to 2 minutes each, he had rightward beating nystagmus on attempted right gaze. Between episodes he had a full range of saccades but impaired leftward pursuit and impaired slow phases of optokinetic nystagmus to the left.
Vital signs on admission / BP: 140/90
Pulse: 76/minute / BP: 180/96
Pulse: 140/minute / BP: 107/70
Pulse: 115/minute / BP: 125/91
Pulse: 57/minute
Precipitant / None identified / Two weeks earlier started metformin which caused diarrhoea / Poor control of diabetes / Steroid induced hyperglycemia during treatment for myasthenia
Known DM / No / Yes / Yes / No
Initial diagnosis / Occipital tumor or AVM / Seizure disorder / Encephalitis, cerebritis or stroke / Cryptogenic seizures
EEG / Twelve hours after initiating treatment: decreased voltage and slowing in the right occipital lobe, but no ictal discharge / Twelve hours after initiating treatment: right anterior- midtemporal and right posterior quadrant spikes / Seven days after initiating treatment: intermittent irregular theta-delta activity left temporal region, but no ictal discharges / Just before treatment:: left occipital discharges, spreading to the right,
MRI of head / Two weeks after the onset of symptoms: decreased T2 white matter signal with effacement of the sulci, in the right occipital lobe, that was iso-intense on the uncontrasted T1 images. Contrasted T1 sequence with cortical swelling and gyral enhancement suggestive of luxury perfusion. / Two weeks after onset of symptoms: decreased T2 white matter signal in the right parieto-occipital and temporal lobes, effacement of the sulci in the right parieto-occipital region, enhancement of the overlying leptomeninges and effacement of the sulci in the right parieto-occipital region indicating luxury perfusion;. Also, there was edema, and contrast enhancement of the cortex of the left inferior and medial temporal lobe, including the uncus and amygdala, the medial occipital lobe cortex and the medial and posterior parietal lobe cortex. Diffusion was restricted in the right occipital lobe. An MRA was normal. / Three weeks after the onset of symptoms: decreased T2 white matter signal. Enhancement of the cortex in the inferior medial left temporal lobe, uncus, amygdala and posterior occipital lobe. / Three days after the onset of symptoms: subtle decreased T2 white matter signal in the left occipito-temporal lobe
Glucose mg/dl / 503 / 426 / 427 / 487
Na meq/L / 129 / 125 / 135 / 132
K meq/L / 4.1 / 3.5 / 4.1 / 3.2
Cl meq/L / 94 / 87 / 93 / 89
HCO3 meq/L / 12 / 18 / 31 / 30
BUN mg/Dl / 16 / 9 / 17 / 40
Osmolality mos/L / 313 ( C) / 282 ( C)
290 (M) on day 4 / 300 ( C) / 305 ( C); 315 (M)
Hgb A1C (normal 4-6.5%) / 11.8 / 12.1 / Not tested / Not tested
Urine / ketones +3 / ketones negative / ketones moderate / ketones negative
pH / 7.41 / Not tested / 7.43 / Not tested
Cerebrospinal fluid / WBCs 1/cmm, RBCs 3/cmm, protein 55 mg/Dl, glucose 246 mg/Dl, cryptococcal antigen and VDRL negative / CSF protein 43 mg/dL (25-55), glucose 83 mg/dL (45-75) , WBCs 2 /uL, RBC 1450 /uL, mononuclear cells 1/uL / WBCs 0/cmm, RBCs 0/cmm, protein 55 mg/Dl, glucose 175 mg/Dl, cryptococcal antigen negative, VDRL non-reactive, HSV 1 and 2 PCR not detected / Not tested
Repeat MRI / 5 months late - r resolved / 4 weeks later - resolved / 3 days after admission, improved / 6 weeks later - resolved
Follow-up Goldmann visual fields / 16 days later: normal / 22 days later: normal / 3 months later: normal / Not tested formally
(Confrontation fields full two days after treatment)
Table. Details of the four patients reported here.
BP = blood pressure; C = calculated; DM = diabetes mellitus; M = measured osmolality; OD = right eye; OS = left eye; OU = each eye; RUE = right upper extremity; VA = visual acuity.
Serum osmolality was calculated using the formula :
[2 x (Na + K)] + [BUN/2.8] + [Glucose/18] = mOsm/kg (normal range 280-290 mOsm/kg).
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