Progressive supranuclear palsy: clinical and pathological diagnosis.

Progressive supranuclear palsy (PSP) is an heterogeneous disorder, both clinically and pathologically and, in consequence, it is often difficult to diagnose. In this review we shall discuss those clinical settings in which it is difficult to make the diagnosis of PSP and review the pathology of the disorder. Clinical and pathological diagnostic criteria currently in use will also be discussed.

Striated anal sphincter denervation in patients with progressive supranuclear palsy.

Anal sphincter electromyography may contribute to the differential diagnosis between Parkinson's disease (PD) and other parkinsonisms featuring autonomic dysfunction, such as multiple system atrophy (MSA). Although patients with progressive supranuclear palsy (PSP) do not normally exhibit clinical signs of autonomic dysfunction, a few may be first seen with urinary and fecal incontinence. We performed an electromyographic study of the anal sphincter in 12 patients with clinical criteria of probable or definite PSP, two of them with clinical manifestations of vesical and anal sphincter dysfunction.

Reaction time and acoustic startle in normal human subjects.

We studied the effects of collision between a voluntary command and the startle response by interrupting a simple visual reaction time task with an acoustic startle. We observed two main effects. First, the reaction time was markedly shortened when the startle was delivered at intervals of 0-75 ms after the 'go' signal.

New and emerging strategies for improving levodopa treatment.

Soon after the successful introduction of large oral doses of levodopa or of levodopa plus a decarboxylase inhibitor, such as carbidopa or benserazide, for the treatment of Parkinson's disease, it became evident that several disturbing side effects were limiting the therapeutic efficacy of this amino acid. This paper discusses novel practical approaches for the management of these levodopa-related complications. These approaches include therapeutic strategies for controlled delivery of levodopa to the brain (controlled-release preparations), rescue treatment with subcutaneous, intranasal, or sublingual administration of the dopamine agonist apomorphine, and the administration of an atypical neuroleptic, such as clozapine.

Mid-stage parkinsonism with mild motor fluctuations.

Most patients with Parkinson's disease who undergo levodopa therapy eventually develop fluctuations in the diurnal control of their symptoms. These fluctuations, when mild, consist mostly of the "wearing-off" effect--the re-emergence of symptoms several hours after the ingestion of each dose of levodopa. In some patients, "wearing-off" in the daytime is preceded by, or associated with, the development of nocturnal and early morning akinesia.

Clinical diagnosis and diagnostic criteria of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome).

Progressive supranuclear palsy (PSP) is characterized clinically by supranuclear gaze palsy, neck dystonia, parkinsonism, pseudobulbar palsy, gait imbalance with frequent falls and frontal lobe-type dementia. In the advanced typical case, when supranuclear gaze palsy and other main features are present diagnosis is relatively easy. Diagnostic problems, though, are frequent in the early stages due to the variable clinical presentation and in those atypical cases in which gaze palsy does not develop or that present as a severe dementia disorder or as an isolated akinetic-rigid syndrome.

The expression of a neuronal nuclear antigen (Ri) recognized by the human anti-Ri autoantibody in the developing rat nervous system.

Anti-Ri is a human autoantibody that recognizes a neuronal nuclear antigen (Ri) of unknown function. To ascertain the possible role of the Ri antigen in neuronal development, we analysed the pattern of the anti-Ri immunoreactivity in the developing rat nervous system. Neurons of the retina, except the photoreceptors, and central but not peripheral nervous system were anti-Ri-positive.

Delayed appearance of anti-myelin-associated glycoprotein antibodies in a patient with chronic demyelinating polyneuropathy.

A patient who had a polyneuropathy compatible with a chronic inflammatory demyelinating polyneuropathy and was initially negative for anti-myelin-associated glycoprotein (MAG) antibodies developed a double monoclonal gammopathy, IgM kappa and IgM lambda, two years after the diagnosis. The IgM kappa, but not the IgM lambda, exhibited strong anti-MAG antibody activity. The late appearance of the anti-MAG immunoreactivity suggests that in patients with an initial diagnosis of chronic inflammatory demyelinating polyneuropathy, the search for anti-MAG antibodies should be repeated during the course of the neuropathy.

Absence of REM sleep, altered NREM sleep and supranuclear horizontal gaze palsy caused by a lesion of the pontine tegmentum.

A 27-year-old woman with a mass lesion confined to the pontine tegmentum had absence of rapid eye movement (REM) sleep and abnormal nonrapid eye movement (NREM) sleep with no sleep spindles when the sole neurologic manifestation was a bilateral supranuclear horizontal gaze palsy. This study suggests that the tegmentum nuclei associated with REM sleep generation in humans and the pathways that control horizontal eye movements are closely related. Furthermore, these findings indicate that bilateral damage of the pontine tegmentum is necessary for REM sleep abolition.

[Neurologic syndromes associated with anti-Hu antibody. Study of 24 patients].

Background: Twenty-four patients with neurologic involvement and anti-Hu antibodies were studied with the aim of defining the type of tumor associated, evaluating whether the clinico-pathologic picture agreed with the concept of paraneoplastic encephalomyelitis (PEM) and evaluating the treatments used.

Methods: The study was retrospective with the clinical histories being reviewed to define the neurologic syndromes, their evolution and response to the different treatments, time of appearance and type of tumor as well as the neuropathologic changes in the patients undergoing autopsy.

Results: In 18 patients a neoplasm was diagnosed as small cell pulmonary carcinoma (SCPC) in 89% of the cases.