Michael J. Doherty

Diffusion abnormalities in patients with Wernicke encephalopathy

Diffusion-weighted imaging (DWI) can help to diagnose acute ischemic stroke. Other nonischemic disorders may show abnormal signals with DWI. The authors report two cases of Wernicke encephalopathy with DWI signal changes in characteristic midline locations, one with reduction in apparent diffusion constant and one without. DWI abnormalities may suggest early thiamine deficiency and are useful in diagnosing Wernicke encephalopathy.

An Xp; Yq Translocation Causing a Novel Contiguous Gene Syndrome in Brothers with Generalized Epilepsy, Ichthyosis, and Attention Deficits

Summary:  Purpose: We describe two brothers with generalized epilepsy, attention deficits, congenital ichthyosis, and Leri–Weill dyschondrosteosis who harbor an unusual Xp; Yq translocation chromosome, resulting in a novel contiguous gene syndrome because of deletion of genes from the distal short arm of the X chromosome.

The experience of earthquakes by patients with epileptic and psychogenic nonepileptic seizures

Summary:  Purpose: We sought to understand better the experience of seizures by studying differences in the subjective experience of being in an earthquake between patients with epileptic (EP) and nonepileptic (NES) seizures.

Captain Cook on poison fish.

On his second voyage of discovery, Captain James Cook charted much of the South Pacific. The journey was long, from 1772 to 1775. During the exploration, the geographic, ethnographic, and scientific variety provided no shortage of work for the accompanying naturalists, astronomers, navigators, and painters. Culinary discoveries included new species of fish, many of which were sketched, dressed, and ultimately eaten. The examined journals and correspondence document clinical poisonings after ingestion of two different species of fish. The clinical findings are described and likely represent ciguatera and tetrodotoxin poisonings. Mechanisms of these toxin’s actions are discussed in light of more recent studies.

Age at focal epilepsy onset varies by sex and hemispheric lateralization

Background: Previous studies have shown that interictal epileptiform discharges favor the left hemisphere in adults but the right side in children up until age 5. This may be due to sex-influenced asymmetric brain maturation. To clarify this relationship, the authors analyzed age at epilepsy onset by sex and by lateralization of epileptiform activity. Methods: An adult epilepsy center long-term monitoring database was used to define patients with exclusively unilateral epileptiform findings. Three groups were studied: any epileptiform activity (n = 404), ictal activity (n = 287), and interictal activity (n = 265). The second and third groups were drawn from the first group and the second and third groups overlapped with each other. Side of lateralized finding and sex were analyzed via factorial two-way analysis of variance with the outcome variable being age at epilepsy onset. Comparison analysis included patients with generalized epilepsy (n = 114), nonepileptic seizures (NES, n = 232), and surgical mesial temporal sclerosis (MTS, n = 116). Results: Patients with unilateral epileptiform activity displayed bimodal epilepsy onset ages with infant and adolescent peaks. For patients with a right-sided focus, epilepsy onset was earlier in men (14.4 years) than women (20.7 years). In contrast, among patients with a left-sided focus, epilepsy began earlier in women (18.2 years) than men (19.9 years, p < 0.01). Parallel results were found in unilateral ictal (p < 0.01) and unilateral interictal activity (p = 0.01). Patients with surgical MTS, NES, or generalized seizure showed no similar patterns. Conclusions: In adult patients with focal epilepsy, sex and lateralized epileptiform abnormalities may be related to age at epilepsy onset.

Ventricular cerebrospinal fluid hypocretin-1 inversely correlates with glucose levels in cerebrospinal fluid and serum from patients with neurological injuries.

AbstractIntroduction: Hypocretin-1 is a hypothalamic neuropeptide that may help regulate arousal and feeding behavior and is quantifiable in cerebrospinal fluid (CSF). In this retrospective pilot study, hypocretin-1 levels obtained from ventricular CSF of neurologically injured patients were correlated with clinical and laboratory results to test whether arousal or metabolic factors might be related to the level of hypocretin-1. Methods: CSF samples from a heterogeneous group of neurosurgical patients with externally draining intraventricular catheters were assayed in a standard manner for hypocretin-1 and other routine laboratories. Associations were sought between hypocretin-1 and clinical data such as body mass index (BMI), temperature, and Glasgow Coma Scale (GCS) score and between hypocretin-1 and laboratory data such as serum and CSF glucose, protein, and cell counts. Results: Lower levels of ventricular CSF hypocretin-1 were correlated with higher levels of serum (p=0.020) and ventricular CSF glucose (p=0.001). Clinical findings such as BMI, temperature, and GCS failed to correlate with hypocretin-1. Conclusions: In a group of neurologically injured patients, hypocretin-1 and glucose levels are inversely correlated. More studies are needed to investigate these associations, particularly in a homogenous patient sample.

The quicksilver prize: Mercury vapor poisoning aboard HMS Triumph and HMS Phipps.

In 1810, two British ships, HMS Triumph and HMS Phipps, salvaged a large load of elemental mercury from a wrecked Spanish vessel near Cadiz, Spain. The bladders containing the mercury soon ruptured. The element spread about the ships in liquid and vapor forms. The sailors presented with neurologic compromises: tremor, paralysis, and excessive salivation as well as tooth loss, skin problems, and pulmonary complaints. The events are reviewed in the context of what was known about mercury vapor inhalation.

James Glaisher's 1862 account of balloon sickness: altitude, decompression injury, and hypoxemia.

In 1862, James Glaisher and Henry Coxwell ascended to 29,000 feet in an open hot-air balloon. During the ascent, Glaisher described marked neurologic compromises: appendicular and later truncal paralysis, blindness, initially preserved cognition, and subsequent loss of consciousness. The author examines Glaisher’s account of balloon sickness by comparing it with other balloonists’ observations and discussing it in the context of altitude sickness, decompression injury, and hypoxemia.

Herpes simplex virus encephalitis complicating myxedema coma treated with corticosteroids

Herpes simplex encephalitis (HSE) may develop after high-dose corticosteroids, but has not been described after myxedema coma. We report a patient with myxedema coma who, after initial improvement, developed HSE likely due to activation of dormant herpes simplex virus (HSV) from a combination of myxedema, corticosteroid treatment, and acute illness. MRI of HSE and myxedema showed diffuse diffusion-weighted imaging abnormalities. Neighbors found an 81-year-old man unresponsive in his unheated home. His respiratory rate was irregular at 32, and his pulse was 10 to 20. Paramedics intubated him in the field. His admission temperature was 23 °C; he was slowly warmed and hydrated. Third-degree heart block necessitated transcutaneous pacing. His paced blood pressure was 90 mm Hg systolic. He had nonpitting edema around his orbits and extremities, yellow-tinged sclerae, difficult to auscultate cardiac tones, and course lung sounds. Pressure ulcerations marked his skin. He did not respond to command or question, his eyes remained closed, and he failed to localize pain. He had reactive pupils, poor extraocular movements, minimal …

How long does it take for partial epilepsy to become intractable? [1] (multiple letters)

To the Editor: Berg et al. wrote a fascinating article on epilepsy intractability.1 Their careful study documented that patients with early childhood seizure onset have long latencies before developing refractory epilepsy, whereas older patients have shorter latencies. There is a simpler question: when do patients with partial epilepsy become refractory? The answer might be clinically useful and is within the authors’ unique data. If, hypothetically, 75% of Berg et al.’s series became refractory between the ages of 18 and 25, my counseling and initiation of more intense treatments of young adults with focal epilepsy would change. Furthermore, I would be more inclined to ask about neurologic, maturational, or other events that occurred during or in recently preceding years. Plots of frequency vs age at the time of second drug initiation and frequency vs age at the time of second drug failure would show when patients become refractory. These graphs should illustrate specific ages during which patients are more (or less) likely to develop refractory focal epilepsy. This information, combined with Berg et al.’s latency analysis, might help define distinct ages during which mechanisms that ultimately lead to refractory epilepsy are most active.