James R. Keane

Bilateral seventh nerve palsy: Analysis of 43 cases and review of the literature

Among inpatients with facial diplegia, one-half (22 patients) had benign, self-limited causes, including Bells palsy (10), Guillain-Barré syndrome (5), multiple idiopathic cranial neuropathies (3), brainstem encephalitis (2), Miller Fisher syndrome (1), and association with benign intracranial hypertension (1). Nine patients had tumors: four meningeal, three prepontine, and two intrapontine. Syphilis (2 patients), Hansens disease (1), cryptococ-cal meningitis with acquired immunodeficiency syndrome (1), and tuberculous meningitis (1) constituted those with an infectious etiology, while miscellaneous causes included one patient each with diabetes, sarcoidosis, head trauma, pontine tegmental hemorrhage, undiagnosed Möbius syndrome in an adult, systemic lupus erythematosus with severe neuropathy, and slowly progressive degeneration–possibly bulbospinal neuronopathy. Bilaterality makes facial neuropathy a more ominous sign with widely varying causes that requires prompt investigation.

The pretectal syndrome 206 patients

The pretectal syndrome occurred in 2.3% of patients personally examined over an 18-year period. The symptoms were nonspecific, but the signs (abnormal pupils in 198 patients, vertical gaze limitation in 180, disjunctive horizontal eye position in 90 and vertical in 79, lid retraction in 83, and convergence-retraction nystagmus in 71) were exquisitely localizing. The etiology, skewed by the local prevalence of cysticercosis, was hydrocephalus in 80 patients, stroke in 53, and tumor in 45. The importance of timely diagnosis was underscored by the relatively good prognosis of many patients.The pretectal syndrome occurred in 2.3% of patients personally examined over an 18-year period. The symptoms were nonspecific, but the signs (abnormal pupils in 198 patients, vertical gaze limitation in 180, disjunctive horizontal eye position in 90 and vertical in 79, lid retraction in 83, and convergence-retraction nystagmus in 71) were exquisitely localizing. The etiology, skewed by the local prevalence of cysticercosis, was hydrocephalus in 80 patients, stroke in 53, and tumor in 45. The importance of timely diagnosis was underscored by the relatively good prognosis of many patients.

Hysterical gait disorders 60 cases

Simulated gait abnormalities involve weakness of 1 or both legs or ataxia and trembling. The patterns rarely duplicate those of neurologic disability and are usually promptly suspected of being functional by the experienced clinician. As with other pseudoneurologic signs, normal underlying neurologic function must be demonstrated. A dramatic cure is the best diagnostic evidence, and a bias toward organic etiology is warranted by a relatively greater risk in delayed diagnosis. Dystonia and chorea are the signs most likely to be mistaken for functional conditions. While CT and MRI now provide a welcome diagnostic safety net, the variety of hysterical gaits, and probably the effectiveness of “moral” treatment, does not appear to have changed appreciably in the past century.

Hypertensive brainstem encephalopathy : Three cases presenting with severe brainstem edema

Hypertensive encephalopathy (HTE) is manifested by headache, confusion, seizures, and visual disturbance and may progress to coma and death.1 CT or MRI scans usually show transient, predominantly posterior white matter involvement that rapidly improves with control of hypertension. The following three patients were unusual in having severe brainstem involvement without accompanying occipital lobe changes. Patient 1. A 54-year-old woman with a history of poorly controlled hypertension presented with 3 days of walking difficulty, right leg weakness, and mild headache with nausea. She denied any visual problems. Examination showed blood pressure (BP) of 210/144 mm Hg. She was confused with mild right hemiparesis. Funduscopic examination showed scattered posterior pole exudates and hemorrhages without disc edema. The T2-weighted and fluid-attenuated inversion-recovery (FLAIR) images showed hyperintense signals in the brainstem, with scattered mild signal changes in the cerebellum and periventricular white matter (figure, A and B). Diffusion-weighted MRI showed only a small left subcortical infarct. Figure. (A and B) Patient 1. MRI–fluid-attenuated …

Fourth nerve palsy Historical review and study of 215 inpatients

A review of inpatient trochlear nerve pareses diagnosed over 23 years revealed head trauma as the principal cause, with surgical injury, inflammation, and brain tumors seen occasionally. Ischemic (microvascular) neuropathies were rare. About one-half of the patients (52%) had no other neuro-ophthalmologic signs, but only 5% were truly isolated, without other neurologic or ophthalmologic signs or symptoms. Fourth nerve palsies are underdiagnosed on hospital services, where stuporous patients encounter unsuspecting physicians.

The minor symptoms of increased intracranial pressure 101 patients with benign intracranial hypertension

Of 101 patients with benign intracranial hypertension not related to vasculitis, neck stiffness occurred in 31, tinnitus in 27, distal extremity paresthesias in 22, joint pains in 13, low back pain in 5, and gait “ataxia” in 4. Symptoms resolved promptly upon lowering the intracranial pressure by lumbar puncture, and were probably directly caused by intracranial hypertension. Awareness of these “minor” symptoms of increased intracranial pressure can facilitate diagnosis and management.

Alternating skew deviation 47 patients

Of 408 patients with ocular skew deviation, 47 (12%) had hypertropia that alternated on gaze to either side. Pretectal lesions were responsible for 29 cases, and lower brainstem signs were seen in 5; the site of posterior fossa involvement was uncertain in 13. Acute hydrocephalus, tumors, strokes, and MS were the most frequent causes, followed by spinocerebellar degeneration and tentorial herniation. The mechanism of alternating skew is unknown, but probably involves pathways from both utricles to the vertical-rotatory ocular motor neurons.

Neuro‐ophthalmic signs and symptoms of hysteria

Functional eye signs and symptoms are a common and challenging part of neurologic diagnosis. Hysterical behavior and malingering may involve visual acuity and fields, eye movements, pupillary abnormalities, and ptosis.

Upbeat nystagmus Clinicopathologic study of two patients

Two patients had upward nystagmus on forward gaze before they died with acute caudal brainstem dysfunction. Bilateral dorsal paramedian damage in the rostral medulla, involving the perihypoglossal nuclei, was probably the critical lesion responsible for upbeat nystagmus.

Galloping tongue: Post‐traumatic, episodic, rhythmic movements

Two patients experienced delayed onset of episodic, rhythmic, involuntary movements of the tongue after head and neck trauma. These three-per-second waves began as posterior midline focal tongue contractions, lasted about 10 seconds in each episode, persisted for 2 to 4 months, and were not accompanied by other body movements or by EEG abnormality.