INTRODUCTION The normal startle response is a form of physiological myoclonus. Its anatomic origin is probably the brain stem. Pathologic startles are defined as reproducible exaggerated startle responses to trivial and not surprising stimuli. Symptomatic forms of an exaggerated startle response can be due to a variety of brain stem disorders. We have, however, found scant data about an exagger...

Congenital indifference to pain (pain asymbolia) is a rare condition. Since Dearborn in 1931 described the first case, 51 cases have been reported in the English literature. Congenital auditory imperception as an isolated defect is equally uncommon. It is perhaps the least uncommon of the various types of congenital aphasia. We have recently studied a family in which two siblings showed an asso...

Pleomorphic xanthoastrocytoma (PXA) is an uncommon tumor of young adults that typically occurs supratentorially. It is generally considered to be a low-grade, circumscribed tumor that when treated by surgical resection has a relatively favorable outcome. Cases of cerebellar PXA are rare, and those associated with neurofibromatosis type 1 (NF1) are even less common, with only 2 cases reported to...

The physiological mechanisms that produce involuntary movements are not well understood. The discovery of decreased levels of dopamine in the basal ganglia of patients with Parkinsonism (Ehringer and Hornykiewicz 1960) and the subsequent successful use of levodopa in the treatment of Parkinsonism (Birkmayer and Hornykiewicz 1961 ; Barbeau 1962) have resulted in renewed interest in the understan...

Multiple sclerosis (MS) is the most common permanently disabling disorder of the central nervous system in young adults. Relapsing remitting MS is the most common type, and typical symptoms include sensory disturbances, Lhermitte sign, motor weakness, optic neuritis, impaired coordination, and fatigue. The course of disease is highly variable. The diagnosis is clinical and involves two neurolog...

Controversy has surrounded the rare clinicopathologic entity of inflammatory myelinoclastic diffuse sclerosis (IMDS) since the seminal description of Schilder in 1912 [1]. Since that time several distinct myelinoclastic and dysmyelinating conditions have been grouped under this eponym. To this newly described demyelinating disease, the author planted the seeds of an enduring controversy by incl...

The Great War accelerated the development of neurological knowledge. Many neurological signs and syndromes, as well as new nosological entities such as war psychoneuroses, were described during the conflict. The period between 1914 and 1918 was the first time in which many neurologists were concentrated in wartime neurology centres and confronted with a number of neurological patients never see...

PTEN is one of the most frequently mutated tumor suppressor genes in human cancers. Germ line mutations of PTEN have been detected in three rare autosomal-dominant disorders. However, identical mutations in the PTEN gene may lead to different symptoms that have traditionally been described as different disorders, such as Cowden disease, Lhermitte-Duclos disease, and Bannayan-Zonana syndromes. T...

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OBJECTIVE To aid in the timely diagnosis of patients who present with clinically isolated syndrome (CIS). PATIENTS AND METHODS We studied 25 patients (18 women, 7 men), originally presented in our clinic with a CIS suggestive of multiple sclerosis (MS). All patients underwent the full investigation procedure including routine tests, serology, cerebrospinal fluid (CSF) examinations, evoked pot...