Spastic eyelids. Failure of levator inhibition in unconscious states.

Three patients exhibited tonic uninhibited lid elevation in unconscious states. In the two cases adequately studied at autopsy, the lesions were too large for precise localization but suggest that damage to the pons (and probably midbrain as well) can interfere with normal lid relaxation in coma and sleep.

Bilateral sixth nerve palsy. Analysis of 125 cases.

Bilateral abducens nerve pareses were nearly as common as unilateral cases in an inpatient setting (125:143). Cerebrospinal fluid abnormalities were more frequent among the bilateral cases, but generally the same causes produced unilateral and bilateral sixth nerve palsy. The relative ease of diagnosis was in contrast with the large number of undiagnosed or "vascular" cases in previous studies of outpatients. The degree of lateral rectus limitation proved to be of limited help in suggesting the cause or predicting recovery of oculomotor function. The etiology was of some prognostic value, with universal recovery of pressure palsies and rare improvement with tumor involvement. Myasthenia, orbital muscle entrapment, convergence spasm, divergence palsy, and pretectal pseudoconvergence entered into the differential diagnosis, but were only occasionally difficult to exclude.

Retinal hemorrhages. Its significance in 100 patients with acute encephalopathy of unknown cause.

A total of 94 patients had subarachnoid hemorrhage and it was strongly suspected in the remaining six patients. Acute encephalopathy associated with independent ocular hemorrhage due to hypoxia, multiple emboli, or bleeding tendencies was not a diagnostic problem during this study. Aneurysms occurred in 64 patients (combined with vascular malformations in four), isolated vascular malformations in four; "spontaneous" hematomas in 13, evidence of cryptic head trauma in six, hemorrhage from a glioblastoma in one, and no cause was identified in six patients. Retinal hemorrhages were more prominent ipsilateral to the site of intracranial bleeding. No single aneurysm location predominated and multiple aneurysms were common. The high mortality of 56% supports previous conclusions that retinal hemorrhages tend to accompany severe intracranial bleeding.

Transient opsoclonus with thalamic hemorrhage.

Opsoclonus, a rare and dramatic saccadic eye movement disorder, was seen in three patients following thalamic hemorrhage. The occurrence of this presumed cerebellar-system disorder in association with pupillary and oculomotor signs of pretectal dysfunction suggests that upper brainstem damage may facilitate the release of spontaneous saccades.

Bilateral ocular motor signs after tentorial herniation in 25 patients.

Twenty-five patients with upper brain-stem damage after severe transtentorial herniation showed a variety of residual oculomotor signs. Pupillary abnormalities (19 patients), ptosis (15 patients), internuclear ophthalmoparesis (13 patients), vertical gaze paresis (14 patients), and third-nerve dysfunction (11 patients) were the principal findings.

Pretectal pseudobobbing. Five patients with 'V'-pattern convergence nystagmus.

Five patients with acute obstructive hydrocephalus experienced arrhythmic, repetitive downward and inward eye movements at a rate ranging from one per three seconds to two per second and an amplitude of one fifth to one half of the full voluntary range. Because of a faster downstroke and appropriate amplitude, these movements were usually diagnosed as ocular bobbing. However, their "V" pattern, the generally faster rate, and pretectal, rather than pontine-associated, signs distinguished them from true ocular bobbing. This pretectal pseudobobbing probably represents a variety of convergence nystagmus, and in contrast with true pontine bobbing, usually indicates a need for prompt surgical attention.

The neurological complications of spermatorrhoea.

The age-old belief that loss of sperm could be dangerous to health became a major medical concern throughout the Western world following Claude-Francois Lallemand's inflammatory warnings about the dangers of spermatorrhoea. This article investigates the leading role played by American neurologic authorities of the mid and late 1800s in their fight against the cerebral and spinal complications of spermic depletion.

Twelfth-nerve palsy. Analysis of 100 cases.

OBJECTIVE: To describe the causes and characteristics of hypoglossal nerve palsy. DESIGN: A review of 26 years of personal experience in a large public hospital. RESULTS: Twelfth-nerve palsies usually appear as signs rather than symptoms. Tumors, predominantly malignant, produced nearly half of the palsies (49 cases), while gunshot wounds made trauma (12) the second most common cause. Stroke (6), hysteria (6), multiple sclerosis (6), surgery (5), Guillain-Barré neuropathy (4), and infection (4) together accounted for about one third of the patients. CONCLUSION: Twelfth-nerve palsy proved to be an ominous sign, with only 15% of patients experiencing complete or nearly complete recovery.

Eye movement abnormalities in systemic lupus erythematosus.

OBJECTIVE: To describe eye movement abnormalities in patients with systemic lupus erythematosus (SLE). DESIGN: Between January 1, 1970, and June 30, 1995, 113 patients with a clinical diagnosis of SLE were examined. Of these, 33 had ocular motor abnormalities and a diagnosis of SLE as defined by the revised criteria of the American College of Rheumatology. SETTING: Patients hospitalized on the wards of the Los Angeles County-University of Southern California Medical Center, Los Angeles. SUBJECTS: Thirty-three patients, 82% women, ranging in age from 19 to 58 years (mean, 33.5 years). The average duration of SLE was 3.5 years, and 10 patients had been symptomatic for 5 years or longer. RESULTS: Of 55 ocular motor signs, 33 involved limitation of eye movements or abnormal eye position at rest; abnormal spontaneous eye movements occurred 12 times; ptosis was seen in eight patients and psychogenic signs in two. Sixteen patients (48%) had brain-stem infarcts; 11 had other known causes, including three with meningitis, two with hysteria, and one each with ocular myositis, pseudotumor cerebri, both ocular myositis and pseudotumor cerebri, Guillain-Barré syndrome, hyperosmolality, and hypoxic encephalopathy. In four patients with isolated sixth nerve palsies and two with isolated ptosis, the cause remained unknown. CONCLUSIONS: Ocular motor signs in SLE are uncommon and often transitory. When present, they help to ascertain the location, and often the cause, of neurologic involvement.

Cavernous sinus syndrome. Analysis of 151 cases.

OBJECTIVE: To characterize lesions causing cavernous sinus syndrome. DESIGN: Review of 26 years of personal experience in a large city hospital. RESULTS: Among 151 patients, tumors (45 patients, 30%) were the most frequent cause of cavernous sinus syndrome. However, when surgical causes (17 patients, 11%) were included, trauma (36 patients, 24%) became most common. Self-limited inflammation was the third frequent cause (34 patients, 23%), while carotid aneurysms and fistulas, infection, and other causes composed the remaining 12%. The age at onset varied with the cause, and patients with aneurysms (average age, 52 years) and patients with tumors (average age, 47 years) were older than those with self-limited inflammation (average age, 35 years) and trauma (average age, 29 years). Spontaneous remissions defined "self-limited inflammation" but were also seen following an acute onset of symptoms due to aneurysms and pituitary apoplexy. CONCLUSIONS: In an unselected series from a city hospital, tumor, trauma, and self-limited inflammation were the predominant causes of cavernous sinus syndrome, and classic causes such as aneurysm, meningioma, and bacterial infection were uncommon. Contrast-enhanced magnetic resonance imaging and watchful waiting proved the most effective diagnostic procedures.

Lid-lag in the Guillain-Barré syndrome.

Two patients with severe idiopathic polyneuritis demonstrated marked bilateral limitation of upper lid descent during the acute phase of their illness. Accompanying facial weakness does not adequately explain this phenomenon and the possibility of supranuclear levator dysfunction is raised.

Ocular skew deviation. Analysis of 100 cases.

In 100 patients with ocular skew deviation, the lesion was usually on the side of the lower eye, particularly when patients with internuclear ophthalmoplegia were excluded, but exceptions were sufficiently frequent to diminish the clinical lateralizing value of skew. Pontine damage was most commonly encountered, but midbrain-pretectal and medullary lesions were frequent. The onset of skew frequently coincided with acute brain stem damage, and while a wide variety of diseases produced skew deviation, stem strokes were present in more than two thirds of the patients. The hypothesis that skew results from damage to tonic otolith-ocular pathways is generally compatible with both the known anatomy of otolith pathways and the clinical presentation of skew. Otolith function testing in patients with skew, further anatomical definition of otolith-ocular pathways, and clinicopathological study of discrete lesions associated with skew should help resolve the causative role of the otolith organs in skew deviation.

Contralateral gaze deviation with supratentorial hemorrhage.

Three patients with spontaneous supratentorial intracerebral hemorrhage had contralateral sustained conjugate gaze deviation. The autopsies disclosed large thalamic-basal ganglia hemorrhages whose caudal extension was limited to the midbrain. Current knowledge of oculomotor localization does not adequately explain this "wrong-side" gaze deviation, and the mechanism remains obscure. The present cases suggest that thalamic hemorrhage can produce contralateral gaze deviation without involving postdecussation horizontal oculomotor pathways. Contralateral gaze deviation is not a rare occurrence with deep supratentorial hemorrhages, and awareness of this confusing sign should assist in localizing intracerebral hematomas.

Oculosympathetic paresis. Analysis of 100 hospitalized patients.

Of 100 consecutive, adult, hospitalized inpatients with acquired unilateral oculosympathetic palsy, in 63 the central neuron was involved-usually due to strokes; in 21, tumor or trauma affected the preganglionic neuron; and in 13, postganglionic damage was sustained from a variety of causes. The preponderance of first neuron involvement by stroke is in part a reflection of patient sampling, but emphasizes the fact that central causes of Horner's syndrome are common.

Blinking to sudden illumination. A brain stem reflex present in neocortical death.

Further evidence that the blink reflex to light is a brain stem reflex was provided by a patient who continued to blink to sudden illumination despite anoxic neocortical death with electrocerebral silence and absent summated cortical visual evoked responses.

Intermittent see-saw eye movements. Report of a patient in coma after hyperextension head injury.

For 24 hours after an automobile accident, a 19-year-old comatose man exhibited spontaneous, intermittent, see-saw, rotatory, and downward eye movements with synchronous lid elevation. These unusual movements, which appear most closely related to nonpendular see-saw nystagmus and atypical ocular bobbing, probably resulted from the severe pontomedullary tegmental damage found at autopsy.

Alternating proptosis. A case report of acute orbital myositis defined by the computerized tomographic scan.

A 27-year-old woman had a two-week course of acute painful right proptosis with ptosis, medial conjunctival injection, and restriction of eye movements--particularly abduction. One month later, a similar remitting painful left proptosis with complete limitation of abduction developed. Computerized tomographic x-ray scanning showed marked contrast enhancement of both medial rectus muscles, documenting the presumptive diagnosis of acute orbital myositis without recourse to invasive diagnostic techniques. Myositis is a common component of idiopathic orbital inflammatory disease (orbital pseudotumor), but awareness of the rare patient with acute inflammation clinically localized to the extraocular muscles will decrease confusion with cranial nerve and cavernous sinus disease.

Strabismus surgery for neurological illness. The Stevens Commission 1887-1889.

In 1887, the New York Neurological Society appointed a committee of distinguished neurologists to investigate the claim of ophthalmic surgeon George Thomas Stevens that he could cure chorea and epilepsy through correction of refractive errors and strabismus. After 2 1/2 years, the supervised therapeutic trial collapsed amid mutual recriminations. The commission issued a bland statement to the effect that the treatment was not curative and was insufficiently helpful to recommend. The idea of ocular reflex causation and surgical cure of "neuroses" persisted for several more decades in the face of increasing skepticism and resistance.

Isolated brain-stem third nerve palsy.

Midbrain hemorrhage from a presumed vascular anomaly caused a severe third nerve palsy without other ocular motor or neurologic signs or symptoms.

Neurologic eye signs following motorcycle accidents.

A review of 96 consecutive patients who suffered neuro-ophthalmologic injuries in motorcycle accidents revealed a wide range of findings. These findings were similar to signs associated with other forms of closed-head trauma, aside from a higher incidence of trochlear nerve palsies.