Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model

We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS

Periodic Alternating Nystagmus

Loss of hearing AS; Declining visual acuity; Gait disturbancesA 42-year old female with abnormal eye movements. Previous history significant for positive serology for syphilis and delivery of a stillborn fetus at age 16.UTI; Infected sacral decubitiUricosuric agents; Anti-bacterial agent

Tolosa-Hunt Syndrome

Painful loss of vision OS; HeadacheA 35-year old female with left-sided acute, painful orbital apex-avernous sinus syndrome accompanied by loss of vision.VA: 20/15 OD, CF OSEMIBilateral occlusions of internal carotid arteryCorticosteroids; Surger

The "Possible" Meningioma Syndrome

Decreasing vision ODA 47-year old female with decreasing vision OD. Previous history significant for an episode a year earlier of nausea, fatigue and weight loss. Anosmia and bilateral tonic pupil noted.VA: 20/200 OD, 20/20 OS declining to 20/400 ODCTBony mass within olfactory grooveSurger

Ptosis Plus

Difficulty swallowing; Ptosis; DiplopiaA 47-year old female with complaints of difficulty swallowing, ptosis and diplopia followed by absence seizure and inappropriate behavior.VA: 20/20 OU; Nystagmus on lateral gazeRight-sided focal siezures; Cortical atrophyAnticonvulsantsVBseizuresn

Pupil Sparing Third Nerve Palsy

Diplopia; HeadacheA 72-year old female with diplopia. Previous history significant for uterine cancer and remote presumption of melanoma.VA: 20/60 OD, 20/200 OSCTMid-brain lesio

Retinal Infarcts in a Patient with an Acute Confusional Syndrome

A 46-year-old man presented with acute confusional syndrome, ataxia, dysarthria, and right hemiparesthesia. Brain MRI showed small bilateral infarcts and fluorescein angiography revealed multiple peripheral retinal infarcts bilaterally. No visual loss was present, and no other organs were involved. The diagnosis of Susac syndrome (microangiopathy of the brain, retina and cochlea) was made and immunosuppressive therapy begun