Abnormal Oculomotor Functions in Amyotrophic Lateral Sclerosis

BACKGROUND AND PURPOSE: Although traditionally regarded as spared, a range of oculomotor dysfunction has been recognized in amyotrophic lateral sclerosis (ALS) patients. ALS is nowadays considered as a neurodegenerative disorder of a third compartment comprising widespread areas of extra-motor brain including cerebellum. Our objective was to perform an observational study to examine for ocular motor dysfunction in patients with ALS and for any differences between bulbar-onset and spinal-onset patients. METHODS: Thirty two ALS patients (bulbar onset: 10, spinal onset: 22) underwent the standardized systemic evaluations using video-oculography. RESULTS: Oculomotor dysfunctions such as square wave jerks, saccadic dysmetria, abnormal cogwheeling smooth pursuits and head shaking and positional nystagmus of central origin have been observed in the ALS patients at a relatively early stage. Abnormal smooth pursuits and saccadic dysmetria were increased in the bulbar-onset compared to the spinal-onset (p < 0.05). CONCLUSIONS: These oculomotor abnormalities may be a marker of neuro-degeneration beyond motor neurons in ALS, especially in bulbar-onset disease. Future longitudinal studies of eye movement abnormalities have provided insights into the distribution and nature of the disease process.

The Clinical Efficacy of Vestibular Function Tests in Patients with Acute Unilateral Vestibulopathy / 대한평형의학회지

OBJECTIVES: Aim of this study is to investigate the clinical efficacy of the vestibular function tests (VFTs) and the predictability of lesion side of vestibular asymmetry parameters in acute unilateral peripheral vestibulopathy. METHODS: Medical records and results of VFTs (caloric, rotatory chair, and head impulse tests) of 57 patients with acute unilateral vestibulopathy were reviewed retrospectively. The VFTs were examined within 7 days after the clinical onset. RESULTS: For the caloric test, 74% showed significant canal paresis and the predictability of lesion side was 88%. For the sinusoidal harmonic acceleration test, 91% had low gain in at least 1 Hz, phase lead showed 70%, 89% showed phase asymmetry and the predictability of lesion side was 90%. For velocity step test, 67% had abnormal Tc asymmetry and the predictability of lesion side was 95%. In bedside head impulse test (HIT), abnormal catch up saccades were observed in 89% and the predictability of lesion side was 100%. For the video HIT, cover or overt catch-up saccades were observed in 95% and the predictability of lesion side was 100%. One hundred percent (100%) had low gain on the video HIT, but the lesion sides were uncertain because of bilateral involvements or artifacts. CONCLUSIONS: The most important things in the diagnosis of acute unilateral vestibulopathy are typical clinical symptoms and spontaneous nystagmus. A combination of rotatory, caloric, and HITs will result in a more complete examination of the vestibular system. Among them, HIT is recommended as the best tool in acute unilateral vestibulopathy.

Opsoclonus-Myoclonus Syndrome Associated with Mumps Virus Infection

BACKGROUND: Opsoclonus-myoclonus syndrome (OMS) is a rare neurological disorder that is characterized by involuntary eye movements and myoclonus. OMS exhibits various etiologies, including paraneoplastic, parainfectious, toxic-metabolic, and idiopathic causes. The exact immunopathogenesis and pathophysiology of OMS are uncertain. CASE REPORT: We report the case of a 19-year-old male who developed opsoclonus and myoclonus several days after a flu-like illness. Serological tests revealed acute mumps infection. The findings of cerebrospinal fluid examinations and brain magnetic resonance imaging were normal. During the early phase of the illness, he suffered from opsoclonus and myoclonus that was so severe as to cause acute renal failure due to rhabdomyolysis. After therapies including intravenous immunoglobulin, the patient gradually improved and had fully recovered 2 months later. CONCLUSIONS: This is the first report of OMS associated with mumps infection in Korea. Mumps infection should be considered in patients with OMS.

Decreased Retinal Thickness in Patients With Alzheimer's Disease

BACKGROUND: The loss of ganglion cells observed in Alzheimer's disease (AD) patients might be attributable to a neurodegenerative process involving the neuroretinal structure. The objective of this study was to determine the retinal thickness in patients with AD using optical coherence tomography (OCT). METHODS: Neuro-ophthalmologic examinations were performed in eight AD patients and eight age-matched control subjects. The average thicknesses of the retinal nerve fiber layer (RNFL) and the ganglion cell-inner plexiform layer (GCIPL) were measured using spectral-domain OCT in both subject groups, and the results were compared. RESULTS: The RNFL was thinner in AD patients than in the age-matched controls (p<0.05), especially at the nasal and inferior retina, and the GCIPL was also significantly thinner (p<0.05). CONCLUSIONS: The decreased retinal thickness observed in AD patients suggests that OCT can be used as a biological marker of AD. The findings of this preliminary study suggest that the extent of the reduction in GCIPL and RNFL thicknesses should be investigated further.

Bilateral Medial Temporal Hematomas in HSV-1 Encephalitis

Herpes simplex encephalitis (HSE) is the most common form of encephalitis, with predilection for the temporal lobes and insular cortex. The presence of RBCs is a characteristic finding of CSF analysis and hemorrhagic transformation of the lesions on CT or MRI is not a rare, because the hemorrhagic necrosis is an important pathologic finding of HSE. However, intracerebral hematoma is rarely reported. We report a case of PCR proven HSE with hemotomas in both the medial temporal lobes, which has not been reported.

Fulminant Guillain-Barre Syndrome Mimicking Cerebral Death Following Acute Viral Hepatitis A

A 32-year-old man was transferred to an intensive care unit due to respiratory difficulties with a 4-day history of progressive areflexic quadriparesis following acute hepatitis A. A nerve-conduction study revealed inexcitability of most nerves. The cerebrospinal fluid showed albuminocytologic dissociation, suggesting Guillain-Barre syndrome (GBS). The patient appeared brain dead on day 4, showing absent brainstem reflexes, respiratory failure, and fully dilated and fixed pupils. This case is an example of how GBS can evolve and simulate a brain-dead state from fulminant deafferentation following acute hepatitis A.