Noble J. David

Fatal atheromatous cerebral embolism associated with bright plaques in the retinal arterioles Report of a case

IN 1961 HOLLENHORST~ described “bright plaques” which he had observed in the retinal arterioles of patients with occlusive cerebral vascular disease, especially during and after carotid thromboendarterectomy . He speculated that the material was embolic and that i t contained crystalline cholesterol or calcium: “These plaques had a characteristically bright orange-yellow color. They reflected the light of the ophthalmoscope, often in a heliographic fashion, and tended to lodge simultaneously at several bifurcations of the same arteriole, as though they were fragments of a larger plaque. Only occasionally did they occlude the involved arteriole.” The patient described herein suffered a fatal hemorrhagic cerebral infarction following surgical examination of a large atheroma of the internal carotid artery in the neck and atheromatous embolism to the middle cerebral artery. The coincident appearance of a shower of bright plaques in the retinal artery on the affected side revealed the diagnosis and provided an opportunity for direct examination of this lesion.

Psychosis as the initial manifestation of adult-onset Niemann-Pick disease type C

Niemann-Pick disease type C (NPC) is a neurometabolic genetic dis6order that is distinguished from Niemann-Pick disease by its later onset, more insidious progression, variable visceromegaly, and abnormalities of intracellular cholesterol metabolism. We describe a patient who presented with an 8-year history of psychosis requiring chronic neuroleptic therapy for a presumed diagnosis of schizophrenia. He was subsequently diagnosed with NPC as the emerging features of dementia, ataxia, dysarthria, and vertical supranuclear ophthalmoplegia were recognized. The characteristic features of adult-onset NPC and the obstacles to early diagnosis are reviewed. NEUROLOGY 1995;45: 1739-1743

Occipital lobe infarctions Perimetry and computed tomography

Seven patients, each with a different pattern of homonymous visual loss caused by ischemic infarction of the occipital lobe, were studied with computed tomography. The retinotopic representation of the visual field on the occipital lobes, as determined by these studies, fully confirms those deduced by previous investigators.

Limbic encephalitis associated with thymoma

Brierley et all initially described 3 cases of subacute encephalitis, resembling limbic encephalitis. Two of these patients did not show any detectable neoplasm, and the authors concluded that this condition was not necessarily associated with malignancy. Subsequently, however, there have been multiple case reports of limbic encephalitis associated with neoplasm, and the entity is now commonly considered a paraneoplastic syndrome.2.3 We describe a 59-year-old man who developed limbic encephalitis in association with a benign thymoma. This represents the 1st report of limbic encephalitis associated with a benign thymoma or other benign neoplasm. Despite complete removal of the thymoma, inexorably progressive neurologic deterioration resulted in death. Case report. A 59-year-old man was evaluated for a progressive alteration in mental status of 7 months’ duration that consisted of confusion, hypersomnolence intermixed with agitation, memory deficits, and depression. Generalized malaise and weakness accompanied a 25-pound weight loss. A benign thymoma discovered by chest x-ray had been removed surgically 2 months prior to admission. General physical examination was normal. He was awake and alert, but oriented to name and date only. His speech was rambling but without anomia. He was unable to write his name, copy a figure, or do simple calculations. Digit span was 4 forward and 2 backward, and memory for recent events was poor. Cranial nerve examination, muscle tone and strength, and sensory perception were normal. Muscle stretch reflexes were hyperactive and the plantar responses were bilaterally extensor. Snout, grasp, and palmomenta1 reflexes were present. Truncal ataxia, without appendicular ataxia, was noted. CT and MRI of the brain and 4-vessel cerebral arteriogram were normal. Lumbar puncture revealed clear CSF with 13 mononuclear white cells per 1111113, protein 100 mg/dl, glucose 47 mg/dl, negative viral and bacterial cultures, and negative VDRL. All other laboratory study findings were unremarkable, including sedimentation rate, serum FTA-ABS, thyroid function, antinuclear antibody, rheumatoid factor, complement studies, heavy metal screen, carcinoembryonic antigen, folic acid, vitamin B,,, human immunodeficiency virus (HIV) antibody, and lymphocyte subsets. An EEG showed background activity of the theta frequency with frontal intermittent rhythmic delta activity. A left temporal lobe brain biopsy (figure) revealed perivascular lymphocytic infiltrates affecting both gray and white matter and occasional microglial nodules. No inclusion

Aneurysm of the extracranial internal carotid artery: Report of a case and discussion

ANEURYSMS of the cervical segment of the internal carotid artery have been primarily of interest to otorhinolaryngologists and vascular surgeons. Only rarely have neurologic symptoms or signs been attributed to such lesions, except following ligation or attempted reconstruction of the carotid vessels. The patient described in this paper suffered transient hemiparesis associated with a n arteriographically demonstrated aneurysm of the internal carotid artery situated just below the base of the skull.

Amaurosis fugax. Funduscopic and photographic observations during an attack.

TRANSIENT MONOCULAR BLINDNESS is regarded as a characteristic manifestation of carotid insufficiency.1 Despite its frequent occurrence in patients with atherosclerotic disease of the carotid artery, reports of direct observations of the ocular fundus during attacks are scarce and the recorded descriptions are vaned. This report stems from an unusual opportunity to study ophthalmoscopically a patient in an attack of amaurosis fugax and to photograph the fundus oculi in color during a subsequent episode.

Simultaneous determinations of retinal and carotid artery pressures: Comparison of per cent reductions after occlusion of the common carotid artery

DETERMINATION of retinal artery pressures by ophthalmodynamometry has proved useful in the diagnosis of thrombosis of the internal carotid artery since a lower pressure in the eye on the side of the occlusion is usually observed. A significant reduction in the ipsilateral retinal artery pressure also occurs after ligation of the common or internal carotid arteries, and it has been suggested that ophthalmodynamometry be used to evaluate the effectiveness of carotid artery ligation in reducing the distal intravascular pressure.14 Whether the degree of reduction in retinal and distal carotid artery pressures is similar immediately after occlusion of the carotid artery is unknown, as simultaneous retinal and carotid artery pressure measurements have been determined in only a few cases.2 The present study was undertaken to compare the pressure changes in the retinal artery with those in the carotid artery distal to the site of occlusion. Simultaneous intracarotid and retinal artery pressure remrdings were obtained and the per cent reductions in each determined after occlusion of the common carotid artery. The results indicate that, although in most cases the per cent reductions in the 2 measurements are similar, the technic of ophthalmodynamometry alone cannot be relied upon in an individual case to determine the intravascular pressure changes distal to occlusion of the carotid artery.

Contrast radiography of the optic nerve sheath

To OUH KNOWLEDGE, contrast radiography of the pia-arachnoidal sheath of the optic nerve has not been reported. No illustrations or mention of such visualization has been encountered in reviewing standard references of neuroradiology,l-s and conversations with neuroradiologists have not stirred their memories to recollect similar demonstrations. For this reason, the following description seemed of interest.

Bilateral sixth nerve palsies in a patient with massive pituitary adenoma

A middle-aged man developed bilateral abducens weakness as the only symptom of a huge chromophobe adenoma of the pituitary gland. This unique presentation delayed diagnosis until exploration and biopsy. The patient, who recovered completely, was studied prior to the era of computerized tomography.

Unruptured aneurysm of the supraclinoid carotid artery A treatable cause of blindness

ONE OF THE MORE INSIDIOUS and potentially treatable causes of progressive visual loss is unruptured aneurysm of the supraclinoid portion of the internal carotid artery. Over the past six years we have encountered 5 intriguing examples of this syndrome and were constrained to review previously published descriptions, in view of our meager experience. Because of its relative rarity as compared to more common types of intracranial aneurysm, in which rupture causes classic subarachnoid hemorrhage, this survey of the problem seemed profitable. Each of the reported cases was seen by one of us. All were characterized by progressive visual loss in association with giant suprasellar aneurysms arising from the internal carotid artery.