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Biomedical subjects

F E Lepore

Publications and source records attributed to F E Lepore.

At least 19 recordsLinked to original sources

Follow-up study of risk factors in progressive supranuclear palsy.

The cause of progressive supranuclear palsy (PSP) is not known and has been little studied. The one previous controlled epidemiologic survey, performed at our center in 1986, found small-town experience and greater educational attainment as PSP risks, but, in retrospect, these results may have been produced by ascertainment bias. Since that time, several anecdotal reports have implicated heredity and various environmental exposures in the cause of some cases of PSP. To clarify the results of the previous study and to evaluate the more recently implicated candidate factors in a controlled fashion, we mailed a validated 69-item questionnaire to 91 personally examined patients with PSP and 104 unmatched controls with other neurologic conditions for which they had been referred to our tertiary neurologic center. We were able to match 75 subjects from each group by year of birth, sex, and race and subjected them to a separate matched-pair analysis. We allowed surrogates to supply any or all of the responses. Questions concerned hydrocarbon, pesticide, and herbicide exposure; urban/rural living; auto repair and other occupations; head trauma; educational attainment; maternal age; and family history of PSP, parkinsonism, dementia, and other neurologic conditions. A statistically significant finding was that patients with PSP were less likely to have completed at least 12 years of school (matched odds ratio = 0.35, 95% CI = 0.12-0.95, p = 0.022; unmatched odds ratio = 0.44, 95% CI = 0.21-0.89, p = 0.020). We hypothesize that this result may be a proxy for poor early-life nutrition or for occupational or residential exposure to an as-yet unsuspected toxin. Future studies should examine these potential risk factors in PSP.

Aged

Disorders of ocular motility following head trauma.

OBJECTIVE: To determine the types and frequency of symptomatic ocular motility disturbances following head trauma and their association with severity of trauma. DESIGN: Retrospective study of patients with (1) diplopia unless visual loss is present, (2) heterotropia for far or near targets, and (3) prior head injury. SETTING: Office and in-hospital consulting practice of a university neuro-ophthalmologist. SUBJECTS: Sixty patients with posttraumatic ophthalmoplegia. MAIN OUTCOME MEASURES: Paralytic and nonparalytic heterotropias were quantitated in prism diopters or percentage limitation of ductions. Convergence insufficiency was assessed by determining the near point of convergence. RESULTS: Fifty-one patients had nuclear or infranuclear findings, ie, trochlear palsies (n = 20), oculomotor palsies (n = 17), abducens palsies (n = 7), combined palsies (n = 5), and restrictive ophthalmopathy (n = 2). Nine patients had supranuclear dysfunction, including seven patients with convergence insufficiency. Bilateral ocular motor palsies and combined palsies were significantly (by means of chi 2 test) associated with head trauma of severity sufficient to cause corticospinal tract dysfunction. Individual or combined ocular motor palsies were not significantly (by means of chi 2 test) associated with intracranial hemorrhage and/or skull fracture or loss of consciousness. CONCLUSIONS: Trochlear palsy was the most common nuclear or infranuclear basis for traumatic diplopia, and convergence insufficiency was the most common supranuclear cause of double vision. Head trauma distinguished by upper motor-neuron signs was correlated with specific subsets of disordered ocular motility.

Adolescent

Harvey Cushing, Gordon Holmes, and the neurological lessons of World War I.

As the gunshots that fatally wounded Archduke Franz Ferdinand and Countess Sophie of Austria resounded from Sarajevo across Europe to become the deafening roar of artillery on the Western Front in August 1914, Harvey Williams Cushing was the world's preeminent neurosurgeon and Gordon Morgan Holmes was arguably the foremost neurologist in the world. The 45-year-old Cushing, and Holmes just 38 years old, would strive to respond to the neurological challenges of World War I. They distinguished themselves amidst the redoubtable efforts of workers such as Walter Cannon, George Riddoch, Charles Sherrington, Henry Head, Victor Horsley, Walther Poppelreuter, and Robert Barany. Even the intense martial spirit of the time would be held in abeyance by the contributions of such men of science, as when the intercession of Prince Carl of Sweden secured the release of Barany from a Russian prisoner-of-war camp and allowed him to accept the 1916 Nobel Prize for his exposition of vestibular physiology. Such respite from the brutality of war was all too brief, and if we are to grasp the significance of the different approaches of Holmes and Cushing to the terrible problems of World War I, we must examine some of the harsh medical realities that they confronted in the "war to end all wars."

Brain Injuries

Uhthoff's symptom in disorders of the anterior visual pathways.

To determine the frequency and clinical characteristics of Uhthoff's symptom, I studied 100 consecutive patients with pregeniculate visual loss of diverse etiology. Eighteen patients reported Uhthoff's symptom. Although there was a significant association of Uhthoff's symptom with multiple sclerosis (10 patients), other etiologies for this symptom included tumor (3 patients), optic neuropathy or papillitis (3 patients), aneurysm, and orbital pseudotumor (1 patient each). There was no link between the severity of visual loss, as measured by diminished acuity, field loss, or binocular involvement, and Uhthoff's symptom.

Adult

Amaurotic mydriasis.

Amaurotic mydriasis is characterized by larger than normal pupils in patients with visual loss. In sought to establish whether amaurotic mydriasis can reliably identify different kinds of visual loss and whether this static measurement might prove useful in discerning "balanced" bilateral optic neuropathies where no relative afferent pupillary defect (RAPD) is detected. Patients with binocular pregeniculate visual loss, patients with balanced binocular pregeniculate loss without RAPD, and patients with monocular pregeniculate visual loss had significantly larger pupils than age-matched controls. Although pupils of patients with binocular pregeniculate visual loss (20/50 or better in one or both eyes) were significantly smaller than pupils of age-matched patients with binocular pregeniculate deficits of worse than 20/50 in one or both eyes, no such correlation between Snellen visual acuity and amaurotic mydriasis was found in patients with monocular pregeniculate visual loss. Amaurotic mydriasis is a clinically useful phenomenon that may identify pregeniculate disease in the absence of a RAPD or distinguish pregeniculate from postgeniculate visual loss.

Blindness

Unilateral and highly asymmetric papilledema in pseudotumor cerebri.

I compared six subjects with pseudotumor cerebri and unilateral or highly asymmetric papilledema with 20 such patients with bilateral papilledema. Patients with unilateral papilledema were significantly older than those with bilateral papilledema, but there were no significant differences between the two groups in disease duration, elevation of intracranial pressure, visual fields and acuity, and presence of headaches or transient visual obscurations. I hypothesize that, in patients with unilateral papilledema, one optic nerve is protected from pressure effects by optic nerve sheath anomalies or lamina cribrosa changes due to increased collagen and decreased elasticity with aging.

Adult

The origin of pain in optic neuritis. Determinants of pain in 101 eyes with optic neuritis.

In 101 eyes with either anterior or retrobulbar optic neuropathy of ischemic or inflammatory origin, ocular pain occurred significantly more often with retrobulbar optic neuropathy. The association of pain with posterior optic nerve lesions supports Whitnall's hypothesis that the pain of optic nerve inflammation is caused by traction of the origins of the superior and medial recti on the optic nerve sheath at the orbital apex. Eye pain reflected neither severity nor origin of optic neuropathy.

Adult

Spontaneous visual phenomena with visual loss: 104 patients with lesions of retinal and neural afferent pathways.

Fifty-seven percent of 104 consecutive patients with retinal or neural visual loss reported spontaneous visual phenomena (SVP). Elementary SVP occurred more commonly (51% of patients) than complex SVP (21%). SVP occur with lesions of any portion of the visual pathways. Unlike irritative hallucinations, they do not aid in localization of the lesion. SVP occur significantly more frequently with visual acuity of 20/50 or less in both eyes. These purely visual hallucinations are unlikely to herald psychiatric disease and may be release phenomena stemming from loss of inhibitory visual input.

Adolescent

Eyelid movement abnormalities in progressive supranuclear palsy.

We systematically videotaped eyelid movements in a community-based series of 38 patients with progressive supranuclear palsy (PSP). Ten patients (26%) had blepharospasm, "apraxia" of lid opening and/or "apraxia" of lid closing. These patients as a group had more severe upgaze paresis but no greater disease duration than the patients without supranuclear lid dysfunction. Patients used a variety of synkinetic movements to overcome lid-movement abnormalities. One patient displayed "slow blinks," a phenomenon not previously described in PSP. Blink rate in PSP, 3.0/min, was markedly lower than that in patients with Parkinson's disease (PD), 12.5/min, and patients with PSP but not PD increased their blink rate during command versional eye movements.

Aged

Supranuclear disturbances of ocular motility in Lytico-Bodig.

We found abnormal supranuclear ocular or lid motility in all of 37 patients with Lytico-Bodig (amyotrophic lateral sclerosis/parkinsonism-dementia complex). Twenty-one patients had pursuit paresis, 18 abnormal vestibulo-ocular reflex (VOR) cancellation, 15 abnormal convergence, 13 abnormal optokinetic nystagmus (OKN), 12 conjugate gaze limitation, nine nystagmus, nine saccadic paresis, and six abnormal fixation. Lid abnormalities included glabellar hyperreflexia in 21, involuntary levator inhibition in three, and blepharospasm in two. Earlier reports have indicated infrequent ocular disturbances in Lytico-Bodig, but we now find supranuclear eye and lid deficits are universal and sometimes very prominent.

Aged

Unilateral ptosis and Hering's law.

In 21 patients with unilateral ptosis, only 1 was found to have lid retraction of the fellow eye--a sign regarded as evidence of Hering's law of equal innervation governing levator function. Hering's law may influence levator function in select cases of ptosis due to disease at or distal to the neuromuscular junction. Alternatively, the relatively autonomous contraction of orbicularis oculi or inhibition of frontalis may mask unilateral lid retraction.

Aged

Bilateral cerebral ptosis.

We studied 13 patients with bilateral ptosis after acute right frontotemporoparietal lobe lesions. All patients had conjugate gaze deviation to the right. The ptosis persisted for about 5 days in five patients, or was still evident at the time of discharge or death. Other cranial nerve findings included lower facial weakness in five patients and both upper and lower facial weakness in eight patients. The transience of cerebral ptosis and conjugate gaze disturbance may imply ability of the intact hemisphere to assume control.

Aged

Progressive supranuclear palsy.

Progressive supranuclear palsy (PSP) was first recognized as a distinct morbid entity by Richardson, Steele and Olszewski a quarter century ago. Subsequent experience has confirmed and extended their original observations. PSP has become familiar as a chronic progressive disorder with extrapyramidal rigidity, bradykinesia, gait impairment, bulbar palsy, dementia and a characteristic supranuclear ophthalmoplegia. It is an important cause of parkinsonism. Its etiology remains obscure. Familial concentrations have not been observed. Some cases exhibit no oculomotor dysfunction. Dementia is usually mild. Recent neuropsychological studies have defined features consistent with frontal lobe cortical dysfunction. Seizures and paroxysmal EEG activity may occur. CT and MRI scans show midbrain atrophy early and later atrophy of the pontine and midbrain tegmentum and the frontal and temporal lobes. PET scans have shown frontal hypometabolism and loss of striatal D-2 dopamine receptors. Postmortem studies have documented involvement of both dopaminergic and cholinergic systems. Treatment remains palliative and unsatisfactory.

Brain

Monocular diplopia of retinal origin.

A 68-year-old man experienced monocular diplopia related to a subretinal choroidal neovascular membrane. His symptom resolved with laser photocoagulation of the choroidal membrane. Retinal disease is an uncommon cause of this uncommon symptom.

Aged