Paraneoplastic Ocular Flutter

Ocular FlutterPowerPoint Presentation: Paraneoplastic Ocular Flutter: http://library.med.utah.edu/NOVEL/Wray/PPT/Paraneoplastic_Ocular_Flutter.ppt Shirley H. Wray, M.D., Ph.D., FRCP, Harvard Medical SchoolUnsteady walkingThe patient is a 58 year old woman with known hypertension. In 1994, two weeks prior to admission she had a dramatic change in behavior with insomnia, agitation and depression. This was accompanied by "ringing of hands and anxiety for no apparent reason". She became anorexic, lost 15 pounds in weight and developed increasing headache, dizziness, head shaking, unsteadiness walking, and a feeling of generalized weakness. She denied nausea, vomiting, lethargy or syncope. After consulting her doctor she came to the Emergency Room. She was seen by Medicine, Psychiatry and Neurology and admitted to Psychiatry with a chief complaint, "I cannot stand up". Psychiatry considered her unsteady gait was histrionic and related to depression and her perception that she was unable to walk. On examination she was extremely agitated and cooperated poorly with the examiner. She was only able to walk with the use of a walker and two nursing assistants. At that time, she also complained of difficulty focusing and an abnormality of her eye movements were noted. A neuro-ophthalmology consultation led to her transfer from Psychiatry to Neurology. Past History: Significant for a previous episode of depression treated with antidepressant medication and alcohol abuse in the past. She was a heavy smoker, smoking a pack per day for over 30 years. Family History: Significant for alcohol abuse in both parents. Social History: Eldest of six siblings, unmarried and living alone. Worked as a secretary until 10 years prior to admission when she lost her job. Neurological examination: She was oriented in time, place and person but failed to obey three-step commands She had: • Fluctuating level of alertness and attentiveness • Motor impersistance and perseveration. • Impaired short term memory A constellation of cerebellar signs: • Dysarthria • Titubation • Fine tremor of the face and chin • Ocular flutter with occasional spontaneous obtrusive saccades (no continuous opsoclonus) • Marked trunkal ataxia sitting and standing • Gait ataxia with inability to walk without support The motor system was intact. Reflexes symmetric, slightly brisk, with flexor plantar responses. Coordination intact in the limbs Sensory examination showed no abnormality The Electronystagmogram showed: 1. Spontaneous and gaze nystagmus - Intermittent ocular flutter epochs (5Hz), 1-2 cycle sinusoids in duration throughout the record. Ocular flutter occurred without any rhythmic pattern, with highest frequency on saccades to the left and on eye closure. She had normal vertical excursions and normal vertical vestibulo-ocular reflex. 2. Saccades - Normal velocity and latency. With gaze-evoked nystagmus with large saccades in either direction. 3. Pursuit (tracking) 0.2 Hertz: Sinusoidal. Ocular flutter intrusions 0.3 Hertz: Saccadic breakdown to the right and left Vertical pursuit gain was negligible. 4. Optokinetic nystagmus - Poor gain in each direction 5. Positional Testing: a. Hallpike's; eyes open in the light - Dizziness with brief burst of right beating nystagmus on the first positioning. b. Static; eyes open in the dark - No nystagmus or dizziness. c. Static; eyes closed - No nystagmus or dizziness. The study showed: • Ocular flutter epochs • Loss of vertical pursuit tracking • Lateral gaze-evoked nystagmus • Saccadic breakdown of pursuit tracking at higher frequencies • A brief burst of right beating nystagmus with accompanying dizziness upon Hallpike positioning to a head hanging right position. These findings were consistent with brainstem and cerebellar dysfunction affecting fixation and saccadic and pursuit pathways. Diagnosis: Paraneoplastic Cerebellar Syndrome Ocular Flutter. Investigations: The workup was directed towards detection of an occult neoplasm breast, ovary or lung. Serum electrolytes were abnormal; Sodium 125 mmol/L (135-145) Potassium 2.8 mmol/L (3.4 - 4.8) Osmolal 262 mosm/kgw (280-296) Hyponatremia was secondary to the syndrome of inappropriate ADH Secretion (SIADH). The diagnosis of SIADH is made by finding a concentrated urine sodium (> 20 mmol/L) in the presence of hyponatremia or low plasma osmolality (