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We report the presence of basilar invagination, an unexpected and previously undescribed abnormality of the skull base, in 7 of 38 long-term survivors of multisystem Langerhans' cell histiocytosis. The abnormality is acquired, but its pathogenesis is uncertain.
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The role of saccadic adaptive processes in recovery from the effects of various neurological disorders, such as myasthenia gravis, extraocular muscle palsies, and age-related macular degeneration, is reviewed. Studies of clinical populations (e.g. cerebellar disease, mild closed head injury, and opsoclonus) in which intrasaccadic displacement of visual targets has been used to stimulate adaptation are also reviewed. Our own data from such a study of 12 subjects with Parkinson's disease are presented, showing that visually guided adaptation is preserved in PD while memory-guided adaptation is impaired. This supports a model in which different brain regions subserve adaptation in different tasks.
BACKGROUND: Hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus. CASE DESCRIPTION: A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briefly entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst. CONCLUSION: Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed.
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Fifty-one members of four generations of a family with autosomal dominant olivopontocerebellar atrophy with decreased visual acuity were examined by an ophthalmologist and a neurologist. Twenty-two persons were affected ophthalmologically and 27 were affected neurologically. We describe the ophthalmologic findings as well as the case history of our youngest patient (age 11 months) from whom we obtained brain tissue and an ophthalmopathologic report at autopsy.
The criteria for assessing adult brain death have been already established, but those for infant brain death have not been yet established in Japan. We report auditory brainstem response (ABR) and postmortem pathology of the temporal bone and brain of a brain-dead 9-month-old female. During the comatose state, her ABR showed only waves I and II bilaterally. Autopsy revealed the presence of a left cerebellar astrocytoma, herniation and anoxic encephalopathy. The pathological examination of the temporal bone revealed the destruction of the inner ear particularly on the left side. In the auditory pathway of brain-dead patients, degeneration occurs first in the cerebrum, followed by the cochlear nerve. Thus, ABR is one of the useful means to assess brain death even in infants.
Direction-changing positional nystagmus (DCPN) is a nystagmus that changes its direction with different body and head positions. Many authors consider that it indicates the presence of a central nervous system lesion. Of 1,196 patients whose abnormal electronystagmographic (ENG) traces were reviewed, 46 (3.8 per cent) showed DCPN. Of these, ten (22 per cent) had central neurologic diseases, 14 (30 per cent) had peripheral vestibular diseases, and for 22 (48 per cent) there was no definite clinical diagnosis. Nineteen patients (41 per cent) with DCPN had ENG findings suggesting a peripheral vestibular lesion, while only five (11 per cent) had ENG findings suggesting a central vestibular lesion. Four of 44 control subjects exhibited DCPN. Thus, the presence of DCPN does not necessarily indicate disease of the vestibular system and definitely does not localize the site of a lesion in the vestibular pathways, but more often indicates a peripheral vestibular site. As with spontaneous nystagmus, lack of suppression with fixation suggests a central lesion.
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As a general trend, the diagnosis in medical clinics often depends on laboratory test results. Neurotological diagnosis, however, requires detailed neurological examinations on a patient by a neurotologist. Therefore, there are differing diagnostic skills among physicians, and there is a kind of "man-made flavor" in neurotological diagnostic procedure. In the present study, current development in the knowledge on the clinical diagnostic significance of pathological eye movement during the last 2-3 years is summarized. Acquired pendular wondering eye-movement. Fixation jerks. Spontaneous and transitory eyeball burst or seizure. Vertical rebound nystagmus. Optokinetic vertical ocular dysmetria. Divergence nystagmus. Counterolling, pure rotatory positioning nystagmus. Inversion of optokinetic after-nystagmus (OKAN). Vertical congenital nystagmus and inversion of optokinetic nystagmus (OKN). Treatment of congenital nystagmus. Vertical spontaneous nystagmus to lower eyelid or so-called "downbeat nystagmus." Downbeat nystagmus seen in bilateral labyrinthine dysfunction. The significance of bilateral vestibular lesion, or symmetric lesion in other is emphasized in the present report for reader's reference and criticism. Our experience on the clinical significance of the abnormal eye movement was reported. It is our wish that accumulation of data on important cases along with the results of experimental studies directly connected with clinical medicine may contribute to the progress of our neurotology in the right direction as "neurology of the posterior fossa."
Remarkable progress has been achieved in the neurophysiological study of the cerebellum. Based on these neurophysiological data, electronystagmographic (ENG) assessment of cerebellar lesions was made to ascertain to what extent cerebellar lesions could be diagnosed. In 13 patients out of 17 whose lesions were mainly localized on one side through CT, cerebellar lesions could be diagnosed by electronystagmographic findings. In the 1 patient with amyotrophic lateral sclerosis of the remaining 4, asymptomatic cerebellar infarction was detected unexpectedly through CT. In the remaining 3 patients, differential diagnosis from brainstem lesions was difficult to evaluate on the basis of ENG findings. In 13 patients showing cerebellar signs and symptoms, pursuit eye movements, optokinetic nystagmus (OKN), and fixation-suppression of caloric nystagmus were impaired on both sides, suggesting large mass effects upon the bilateral brainstem. However, either rebound nystagmus or opposing positioning nystagmus characteristic of cerebellar lesions was detected in 13 patients (81%). The same was true in patients with bilateral cerebellar lesions. ENG test, however, could not detect any patients who showed neither cerebellar signs nor symptoms. Further investigations are needed to supplement the gap between basic and clinical research.
Positioning nystagmus accompanied by severe vertigo had been reported in patients with partial lesions of the inner ear, especially otolith lesions. Typically this type of nystagmus represents a latent period and subsequent fatiguability. We concur with this finding and have constantly emphasized the significance of this phenomenon in clinical diagnosis. Since we started to use CT-scanning, this type of nystagmus has been noted in 20 patients, all of whom had cerebellar vermis lesions. Attention should be focused on this association. A simple coincidence could not be excluded if such a combination were seen in only 1 or 2 patients, it could be attributed to simple coincidence. But its occurrence in as many as 20 patients a causal role of cerebellar vermis lesions. Its mechanism may be explained by incomplete inhibition of the vestibulo-oculomotor system including the cerebellar flocculonodular lobe or vestiburo-cerebellum.
The changes in microtubule-associated protein 5 (MAP5) expression in the cerebellum with olivocerebellar degeneration (OCD) were investigated by means of immunohistochemical method, compared with gestational age-matched controls. In controls of 24-33 postmenstrual weeks, the molecular layer was diffusely immunoreactive. However, in cases of olivocerebellar degeneration (25-35 postmenstrual weeks), MAP5 immunoreactivity was reduced in the inner half of the molecular layer, especially in a portion where Purkinje cells were absent. The ratio of the density in the outer half of the molecular layer to that in the inner half was also determined with an image analyzer, and increased significantly in Purkinje cell-negative areas. Because MAP5 was believed to be expressed mainly on growing axons in the early fetal period, the reduction of MAP5 immunoreactivity in OCD cases suggested that normal interaction of Purkinje cells and climbing fibers is vulnerable to ischemia and hypoxia in developing stage and that retrograde transynaptic degeneration of the inferior olivary nuclei is secondarily induced.
This article summarizes sleep disturbances in a variety of neuro-degenerative diseases, including Parkinson's disease, multiple system atrophy, and amyotrophic lateral sclerosis. Sleep complaints in these conditions include insomnia, hypersomnia, abnormal motor activity and behavior during sleep, sleep-related breathing problems, and circadian rhythm sleep disturbances. Clinical examination followed in selected cases by polysomnographic, multiple sleep latency, and other laboratory tests is essential for correct diagnosis and treatment of these sleep disturbances.
Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Neurologic manifestations occur usually as a part of the spectrum of the systemic disease. The aim of this retrospective study was to evaluate the role of magnetic resonance imaging (MRI) in the diagnosis of patients with neurosarcoidosis (NS). Seven patients with sarcoidosis could be included into the study. All patients had neurological symptoms and were evaluated with MRI revealing a wide spectrum of findings: periventricular and white matter lesions, multiple or solitary supra- and infratentorial brain lesions, leptomeningeal enhancement, involvement of brain nerves and intramedullar lesions. These findings are not specific for sarcoidosis and must be considered with the clinical course of the patient in arriving at the correct diagnosis.