Clinical grand round: A rapidly progressive pyramidal and extrapyramidal syndrome with a supranuclear gaze palsy

The case history of a patient with a rapidly progressive spastic tetraparesis, pseudobulbar palsy, supranuclear gaze palsy, and extrapyramidal signs is presented. The patient died 17 months after symptom onset, and the differential diagnosis and pathological findings are reviewed. © 2005 Movement Disorder Society

[1]  A. Godbolt,et al.  Frontotemporal lobar degeneration with ubiquitin-only-immunoreactive neuronal changes: broadening the clinical picture to include progressive supranuclear palsy. , 2004, Brain : a journal of neurology.

[2]  Seth Love,et al.  Motor neuron disease with neurofibrillary tangles in a non-Guamanian patient , 2004, Acta Neuropathologica.

[3]  James Lowe,et al.  Parkinsonism in motor neuron disease: case report and literature review , 2004, Acta Neuropathologica.

[4]  S. Al-Sarraj,et al.  Ubiquitin‐only intraneuronal inclusion in the substantia nigra is a characteristic feature of motor neurone disease with dementia , 2002, Neuropathology and applied neurobiology.

[5]  M. Vaphiades,et al.  Motor neuron disease presenting with slow saccades and dementia , 2002, Amyotrophic lateral sclerosis and other motor neuron disorders : official publication of the World Federation of Neurology, Research Group on Motor Neuron Diseases.

[6]  J. Hardy,et al.  A clinical and pathological study of motor neurone disease on Guam. , 2001, Brain : a journal of neurology.

[7]  J. Hardy,et al.  The genetic and pathological classification of familial frontotemporal dementia. , 2001, Archives of neurology.

[8]  W. Kamphorst,et al.  Familial frontotemporal dementia with ubiquitin-positive inclusions is linked to chromosome 17q21-22. , 2001, Brain : a journal of neurology.

[9]  T D Bird,et al.  Familial frontotemporal dementia with ubiquitin-positive, tau-negative inclusions , 2000, Neurology.

[10]  R. Leigh,et al.  Slow vertical saccades in motor neuron disease: Correlation of structure and function , 1998, Annals of neurology.

[11]  G. Borasio,et al.  Dopaminergic deficit in amyotrophic lateral sclerosis assessed with [I-123] IPT single photon emission computed tomography , 1998, Journal of neurology, neurosurgery, and psychiatry.

[12]  J. Lowe,et al.  Motor neurone disease-inclusion dementia. , 1996, Neurodegeneration : a journal for neurodegenerative disorders, neuroprotection, and neuroregeneration.

[13]  J. Schneider,et al.  Motor neuron disease with parkinsonism. , 1996, Archives of neurology.

[14]  R. Defendini,et al.  The neuropathology of chromosome 17‐linked dementia , 1996, Annals of neurology.

[15]  K. Marder,et al.  Clinical characteristics of a family with chromosome 17‐linked disinhibition‐dementia‐ parkinsonism‐amyotrophy complex , 1994, Neurology.

[16]  L Lavine,et al.  Supranuclear disturbances of ocular motility in Lytico‐Bodig , 1988, Neurology.