Branch retinal artery occlusions as the presenting feature of primary central nervous system vasculitis.

A 39-year-old woman presented with multiple branch retinal artery occlusions almost three years before developing a mass lesion containing calcium in the left frontal lobe. Brain biopsy revealed a small vessel vasculitis and ischemic necrosis of brain with dystrophic calcification. We believe this to be the first case of primary CNS vasculitis with branch retinal artery occlusions and brain calcification.

MRI findings in Susac's syndrome.

BACKGROUND: Susac syndrome (SS) is a self-limited syndrome, presumably autoimmune, consisting of a clinical triad of encephalopathy, branch retinal artery occlusions, and hearing loss. All three elements of the triad may not be present or recognized, and MR imaging is often necessary to establish the diagnosis. OBJECTIVE: To determine the spectrum of abnormalities on MRI in SS. METHODS: The authors reviewed the MR images of 27 previously unreported patients with the clinical SS triad, and 51 patients from published articles in which the MR images were depicted or reported. RESULTS: All 27 patients had multifocal supratentorial white matter lesions including the corpus callosum. The deep gray nuclei (basal ganglia and thalamus) were involved in 19 (70%). Nineteen (70%) also had parenchymal enhancement and 9 (33%) had leptomeningeal enhancement. Of the 51 cases from the literature, at least 32 had callosal lesions. The authors could not determine the presence of callosal lesions in 18 of these patients, and only one was reported to have a normal MRI at the onset of encephalopathy. CONCLUSIONS: The MR scans in SS show a rather distinctive pattern of supratentorial white matter lesions that always involve the corpus callosum. There is often deep gray matter, posterior fossa involvement, and frequent parenchymal with occasional leptomeningeal enhancement. The central callosal lesions differ from those in demyelinating disease, and should support the diagnosis of SS in patients with at least two of the three features of the clinical triad.

Microangiopathy of the brain, retina, and cochlea (Susac syndrome). A report of five cases and a review of the literature.

OBJECTIVE: This study reports five new cases of microangiopathy of the brain, retina, and cochlea (Susac syndrome) and reviews the world's literature. DESIGN: Five cases were systematically studied by the authors. The cases in the literature were identified through Medline searches for Susac syndrome; microangiopathy of the brain, retina, or ear; and cross-referencing the indexes of each retrieved article. PARTICIPANTS: The number of new patients studied in this report was five. An additional 41 patients were culled from the literature. INTERVENTION: Patients were treated with corticosteroids, antineoplastic agents, and other methods in a noncontrolled, nonrandomized fashion. MAIN OUTCOME MEASURES: With respect to therapeutic intervention, the main clinical outcome measures were return of vision, improvement of neurologic and psychiatric manifestations, and recovery of auditory function. Alterations of abnormalities observed by cranial magnetic resonance imaging also were monitored. RESULTS: Of 46 identified patients, 39 were women. The mean age of the patients was 30 years. Forty-one patients (89%) had arterial occlusions, which were bilateral in 60%. Thirty-one patients (67%) reported hearing loss. Twenty patients (44%) had a global encephalopathy, but other neurologic manifestations were common. The mean duration of the illness was 46.7 months. CONCLUSION: This rare syndrome is more common than previously thought, has a strong female preponderance, and often can be identified at an early stage with a careful history and physical examination. Magnetic resonance imaging of the brain often shows lesions suggestive of multiple sclerosis. Fluorescein angiography may show arteriolar wall hyperfluorescence. Early treatment with corticosteroids often is, although not invariably, associated with a good prognosis. The disease appears to be self-limited in most patients.

Sudden visual field constriction associated with optic disc drusen.

We report two patients with optic disc drusen who suffered sudden, concentric constriction of the visual field. Visual acuity remained normal. The involved discs showed no swelling, hemorrhage, or other evidence of anterior ischemic optic neuropathy. We are unable to explain the mechanism or the pattern of visual field loss in these unusual cases.

Optic atrophy following jejunoileal bypass.

A 38-year-old woman presented with a bilateral, chronic, progressive optic neuropathy following jejunoileal bypass surgery for morbid obesity. Standardized A-scan orbital echography revealed grossly swollen optic nerve sheaths with a positive 30 degrees test, indicating increased subarachnoid fluid. Review of reported complications of jejunoileal bypass surgery revealed no similar case of what might be termed the "optic nerve ascites syndrome."

Sarcoid optic neuropathy.

Three patients presented with unilateral progressive optic neuropathy. None of these patients had signs of symptoms referable to the chiasm or eye, thus confining their decline in vision to the optic nerve. Clinical and neuroradiographic evidence suggested a meningioma involving the optic nerve at the orbital-canalicular junction in one patient and the intracranial optic nerve in another patient. Surgical exploration in both patients, however, revealed a noncaseating granuloma. Decline in vision from granulomatous invasion of the retrobulbar optic nerve is an uncommon manifestation of sarcoidosis. Review of our patients' findings suggests that a nonsurgical diagnosis of sarcoid optic neuropathy may have been tenable.

Hoquet diabolique: intractable hiccups as a manifestation of multiple sclerosis.

In three patients, intractable hiccups occurred as part of the symptomatology of multiple sclerosis. In one patient intractable hiccups were the presenting complaint, and in another patient exacerbations of symptoms were almost always heralded by intractable hiccups. Intractable hiccups occur in a variety of diseases, including many that affect the brainstem and cervical cord, but have not been reported in multiple sclerosis. The hiccup may be a "primitive" gastrointestinal reflex that is disinhibited by lesions such as multiple sclerosis plaques. Carbamazepine was successful in arresting the hiccups in one of the cases presented.

Microangiopathy of the brain and retina.

Two women (26 and 40 years old) developed an unusual microangiopathy that affected the brain and retina. Psychiatric symptoms initially overshadowed the subacute features of the progressive neurologic disorder. Ophthalmoscopic findings of multifocal branch retinal artery occlusions provided clinical evidence of vasculopathy. Laboratory data did not reveal evidence of the known vasculitides, including systemic lupus erythematosus (SLE) and syphilis. Cerebral angiography suggested vasculitis in the younger patient. Brain biopsy in the older patient (after 3 months of steroid therapy) revealed a sclerosis of the small pial and cortical vessels that was consistent with a "healed" angitis. Both patients seemed to respond to steroid therapy.

Inferior branch palsy of the oculomotor nerve.

Three patients with inferior branch palsies of the oculomotor nerve are described. Two were under 10 years of age and the third was 30 years old at the onset. All 3 presented with painless diplopia. The onset was sudden in 2 patients and progressive in the third. The palsy cleared within a short time in the 2 patients with sudden onset, and a possible viral origin was considered. In the third patient, with progressive palsy, there was no change eight years later.

Intracanalicular meningioma with normal tomography. Case report.

A middle-aged woman presented with the typical symptoms and signs of a compressive optic nerve lesion. Plain skull films, tomography of the optic canals, and selective angiography with magnification and subtraction were normal. Computerized transaxial tomography clearly showed the tumor, which was a meningioma of the optic nerve sheath. At surgery the tumor was found within the optic canal with intracranial and orbital extensions. It was strictly intradural and did not involve the surrounding bone at any point. This case documents the fact that an intracanalicular meningioma may be associated with normal tomograms of the optic canal.

False diagnosis of orbital apex tumor by CAT scan in thyroid eye disease.

Computerized transaxial tomography is now the procedure of choice in diagnosing exophthalmos. Caution must be exercised, however, in interpreting the results. The patient reported here had a swollen inferior rectus muscle that simulated an orbital apex tumor on CAT scan.

The impossible meningioma.

Experience in three cases has shown that a small meningioma in the extreme posterior portion of the orbit just anterior to the optic foramen may occur with progressive visual loss in the absence of orbital signs. Such lesions cannot be detected by plain skull roentgenography, polytomography, or selective angiography with subtraction and magnification. In one patient, even computerized axial tomography gave normal results, and the meningioma went undetected until exploratory craniotomy.