BACKGROUND Neurodegenerative dementias are typically characterized by an insidious onset and a relatively slowly progressive course. Less common are patients with a rapidly progressive course to death. OBJECTIVE To characterize patients with a neurodegenerative disease and a rapidly progressive course to death. DESIGN, SETTING, AND PATIENTS Using a text word search for "rapid" and "dementia...

Parkinson's disease is characterized by a triad of cardinal motor symptoms (bradykinesia, rigidity and tremor) resulting from the loss of dopaminergic neurons in the substantia nigra. This synucleinopathy is classified in the larger group of Lewy body disorders. Currently, these symptoms are relatively well alleviated by drugs that restore dopaminergic neurotransmission, andlor by deep brain st...

Peripheral glia are known to have a critical role in the initial response to axon damage and degeneration. However, little is known about the cellular responses of non-myelinating glia to nerve injury. In this study, we analyzed the transcriptomes of wild-type and mutant (lacking peripheral glia) zebrafish larvae that were treated with metronidazole. This treatment allowed us to conditionally a...

Degeneration of the sympathetic neuron can be achieved by a variety of procedures. Until 1960 the most common method for producing degeneration of postganglionic sympathetic nerve fibers was either to surgically remove the neuronal cell body, or to cut the extrinsic nerve fibers between the cell body and the effector organ. In recent years a number of chemical agents and immunological methods h...

Amyotrophic lateral sclerosis (ALS), which was described since 1869 by Jean Martin Charcot, is a devastating neurodegenerative disease characterized by the selective and progressive loss of upper and lower motor neurons of the cerebral cortex, brainstem and the spinal cord. Progressive motor neuron loss causes muscle weakness, spasticity and fasciculation, eventually paralysis and finally death...

The motor neuron diseases are a set of disorders associated with the selective degeneration of motor neurons. Amyotrophic lateral sclerosis (ALS) is the most common and confers the gravest prognosis. Although ALS occurs with known genetic causes in a small minority, other motor neuron disorders have well-defined genetic mutations. Electrodiagnostic testing is important to distinguish these vari...

Axonal degeneration is a prominent feature of many neurological disorders that are associated with mitochondrial dysfunction, including Parkinson's disease, motor neuron disease, and inherited peripheral neuropathies. Studies of the Wld(s) mutant mouse, which undergoes delayed Wallerian degeneration in response to axonal injury, suggest that axonal degeneration is an active process. Wld(s) mice...

Parkinson’s disease (PD) is a progressive neurodegenerative disease due to dopaminergic neuron degeneration within the Substantia Nigra. According to the Parkinson’s Disease Foundation, as many as one million Americans live with Parkinson’s disease and approximately sixty thousand are diagnosed with PD each year. Although there are a number of treatment options available for the symptoms that a...

Forty patients with cervical myelopathy underwent high-resolution computed tomography (CT) with intrathecal administration of metrizamide for evaluation of cervical spinal cord atrophy. Thirty of them showed evidence of either focal atrophic distortion or generalized accentuation of the anatomic surface features of the spinal cord. Patients with a Chiari malformation or syringomyelia were exclu...