Patrick Lavin

Fulminant idiopathic intracranial hypertension

Objective: To describe the incidence and characteristics of acute and rapidly progressive visual loss in idiopathic intracranial hypertension (IIH). Methods: We reviewed the medical records of all patients with IIH seen at two institutions. “Fulminant IIH” was defined as the acute onset of symptoms and signs of intracranial hypertension (less than 4 weeks between onset of initial symptoms and severe visual loss), rapid worsening of visual loss over a few days, and normal brain MRI and MR venography (or CT venogram). Results: Sixteen cases with “fulminant IIH” were included (16 women, mean age 23.8 years [range 14 to 39 years]). All were obese. One patient had iron-deficiency anemia, four had systemic hypertension, and none had known sleep apnea syndrome. Acute or subacute headache, nausea and vomiting, and visual loss were present in all patients. The first lumbar puncture performed for the diagnosis showed a mean CSF opening pressure of 54.1 cm H2O (range 29 to 60 cm H2O). In addition to the initial lumbar puncture, medical treatment included acetazolamide (1 to 2 g/day) in all patients, and IV methylprednisolone in four patients. Repeat lumbar punctures were performed in 11 of the 16 patients. Surgical treatment (optic nerve sheath fenestration in five cases, lumboperitoneal CSF shunting procedure in nine cases, and ventriculoperitoneal shunting procedure in two cases) was performed because of ongoing visual loss in all cases. The median delay between evaluation in neuro-ophthalmology and surgery was 3 days (range a few hours to 37 days). All patients reported dramatic improvement of headaches and vomiting following surgery. Visual function improved in 14 cases, although 8 patients (50%) remained legally blind. Visual fields remained severely altered in all cases. Conclusion: Severe and rapidly progressive visual loss suggests “fulminant idiopathic intracranial hypertension” and should prompt aggressive management. Urgent surgery may be required in these patients, and temporizing measures such as repeat lumbar punctures, lumbar drainage, and IV steroids considered.

Visual field defects after temporal lobe resection: a prospective quantitative analysis.

Objective: To evaluate and quantify prospectively visual field changes in patients undergoing temporal lobe resections for intractable epilepsy. Background: Visual field abnormalities occur after temporal lobe resections for epilepsy; however, we have not encountered published reports using automated static visual field analysis. Methods: Humphrey visual fields (program 30-2) were obtained before and after partial temporal lobe resection in 32 consecutive patients with intractable epilepsy. A quantitative point-by-point analysis was made in the affected superior quadrant, and the defects were averaged for the whole patient group. Results: Thirty-one patients developed a visual field defect, but none was aware of the defect. The points nearest fixation were relatively spared. The defects were greatest in the sector closest to the vertical meridian in the eye ipsilateral to the resection. The ipsilateral and contralateral mean field defects also differed in both topography and depth. A significant correlation was found between the extent of lateral temporal lobe resection and the degree of the defect in the contralateral eye. Conclusions: There are differences in the shape and depth of the ipsilateral and the contralateral field defects not previously reported. These findings demonstrate that certain fibers from the ipsilateral eye travel more anteriorly and laterally in Meyer’s loop, and support the hypothesis that visual field defects due to anterior retrogeniculate lesions are relatively incongruous because of anatomic differences in the afferent pathways. Automated perimetry is a sensitive method of evaluating and quantifying visual field defects.

Spontaneous visual recovery from traumatic optic neuropathy after blunt head injury

Four patients who developed immediate blindness (no light perception) after indirect traumatic optic neuropathy caused by blunt head injury recovered vision without surgical intervention. In one patient, whose affected eye recovered to a visual acuity of 20/50 + 2, corticosteroids were not used. In two of the other patients, visual recovery began before corticosteroids were instituted. One patient recovered a visual acuity of R.E.: 20/15, one recovered a visual acuity of L.E.: 20/25-2, and one recovered a visual acuity of R.E.: 20/200 but with useful temporal field vision. Many investigators advocate aggressive surgical therapy for indirect neuropathy, particularly when corticosteroids fail. Significant recovery may occur despite no light perception, however, with medical therapy or even without therapy.

Stereotactic Ventriculoperitoneal Shunt for Idiopathic Intracranial Hypertension: Technical Note

OBJECTIVE Lumboperitoneal shunting is the bastion of neurosurgical management for idiopathic intracranial hypertension (IIH). However, recent studies document a high failure rate for this procedure. The present study was designed to explore the feasibility of placing ventriculoperitoneal shunts under stereotactic control into patients with IIH as an alternative to lumboperitoneal shunting. METHODS Seven patients with IIH for whom medical management had failed underwent stereotactic implantation of ventriculoperitoneal shunts. RESULTS Shunt placement was successful and uncomplicated in each case. Five of seven patients experienced complete resolution of papilledema. The remaining two patients showed resolving papilledema. Six of seven patients experienced resolution of headache. The remaining patient continued to have headaches despite a radionuclide study demonstrating normal shunt function. CONCLUSION Our results suggest that stereotactic ventriculoperitoneal shunting may be a reasonable alternative to lumboperitoneal shunting in those patients with IIH who require surgical intervention.

Oculopalatal Myoclonus after the One-and-a-half Syndrome with Facial Nerve Palsy

PURPOSE The one-and-a-half syndrome is an eye movement disorder characterized by a unilateral gaze palsy and an ipsilateral internuclear ophthalmoplegia. The authors describe a previously unrecognized association between the one-and-a-half syndrome and oculopalatal myoclonus (OPM). METHODS Five clinical cases are presented, with pertinent physical findings and radiologic studies. RESULTS A previously unrecognized association of the one-and-a-half syndrome with subsequent development of OPM appears to exist. Involvement of the facial nerve in patients with the one-and-a-half syndrome may be a predictor of the subsequent development of OPM. CONCLUSION Patients with the one-and-a-half syndrome and facial nerve palsy should be followed closely for possible future development of OPM.

Pseudo one‐and‐a‐half syndrome with ocular myasthenia

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Simultaneous Traumatic Central Retinal Artery Occlusion and Optic Neuropathy

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Homonymous hemianopia in nonketotic hyperglycemia is an ictal phenomenon

Extended video-EEG or 18F-fluorodeoxyglucose PET (FDG-PET) was obtained in 3 adult patients with hemianopia secondary to nonketotic hyperglycemia. Two male patients presented with left hemianopia and episodic left gaze deviation and one male patient presented with right hemianopia and visual hallucinations. None of the 3 patients had a history of seizures or known epilepsy risk factors. All 3 patients were found to have elevated serum glucose (267 mg/dL, 320 mg/dL, and 487 mg/dL) without acidosis or urine ketones. In all 3 patients, video-EEG recorded recurrent ictal discharges originating from the posterior quadrant contralateral to their hemianopia. In 2 patients, FDG-PET demonstrated corresponding focal areas of hypermetabolism. Resolution of visual symptoms was achieved with antiepileptic drugs, hydration, and tight glycemic control.

Pupillary oscillations synchronous with ictal nystagmus

Transient nystagmus and simultaneous pupillary oscillations were observed during partial complex seizures, in a diabetic patient with a mild hyperosmolar state. Cerebral influences on pupillary size and the association of pupillary changes with saccadic eye movements are discussed. The contralateral middle frontal gyrus is implicated in the production of these simultaneous synchronous ictal phenomena.

Myasthenia Gravis Presenting as Lutz Posterior Internuclear Ophthalmoplegia

ABSTRACT Myasthenia gravis can mimic central neurological disorders and should be considered in the differential diagnosis of any form of pupil-sparing ophthalmoplegia. We report an unusual manifestation of myasthenia gravis presenting as bilateral internuclear ophthalmoplegia (INO) of abduction, sometimes referred to as Lutz posterior INO (or reverse INO).