Idiopathic Orthostatic Hypotension

Roessmann, Uros; Noort, Stanley van den; McFarland, Dee E.

doi:10.1001/archneur.1971.00480360037004

Cited by 56 publications

(18 citation statements)

References 11 publications

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“…As for the presence of Lewy bodies, our patient's dementia seems related to the wide spectrum of dementia with Lewy bodies, whereas, clinically, the patient exhibited progressive autonomic failure and lethargy without parkinsonism or cerebral cortical signs. This case and the other similar ones [5][6][7]16,17] suggest a specific type of Lewy body disease, and argue that Lewy body disease represents a broader clinicopathologic spectrum than believed so far.…”

Section: Brainstem-ty?e Lewy Body Diseasementioning

confidence: 57%

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Brainstem-type Lewy body disease presenting with progressive autonomic failure and lethargy

Hishikawa

Hashizume

Hirayama

et al. 2000

Clinical Autonomic Research

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The authors report an autopsy case characterized by progressive lethargy and autonomic failure with a distinctive pattern of occurrence of Lewy bodies. Autonomic dysfunction such as sleep apnea, orthostatic hypotension, dysuria, and hypohidrosis predominated with lethargy, whereas parkinsonism was not apparent. Numerous Lewy bodies were widely evident microscopically in brainstem nuclei and the intermediolateral cell columns of the spinal cord, as well as in the sympathetic ganglia, but were rare or absent in the cerebral cortex and other supratentorial structures. Marked neuronal loss was seen in the locus ceruleus, raphe nuclei, dorsal vagal nuclei, and intermediolateral cell columns, but neurons in the substantia nigra, other brain regions, and sympathetic ganglia appeared undiminished. This case represents a specific clinicopathologic form of Lewy body disease occurring predominantly in the brainstem, spinal cord, and sympathetic ganglia.

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Section: Brainstem-ty?e Lewy Body Diseasementioning

confidence: 57%

“…Lewy bodies were found in these areas. Among these patients with autonomic failure with Lewy bodies, 3 showed isolated autonomic failure without parkinsonism [5][6][7]. Subsequently, 2 cases of pure autonomic failure with similar pathologic features were reported [16,17].…”

Section: Brainstem-ty?e Lewy Body Diseasementioning

confidence: 99%

Brainstem-type Lewy body disease presenting with progressive autonomic failure and lethargy

Hishikawa

Hashizume

Hirayama

et al. 2000

Clinical Autonomic Research

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“…However, this has proved to be a heterogeneous condition, including diseases such as PAF, acute autonomic neuropathy, the early stages of Shy-Drager syndrome, and Parkinson's disease with autonomic failure. [1][2][3][4][5][6] Bannister et al 7 classified primary autonomic failure into three categories: Parkinson's disease with autonomic failure, multiple system atrophy (MSA), and pure autonomic failure. In 1996, a consensus statement was established concerning PAF, 8 but it has remained uncertain whether the autonomic failure of PAF can readily be distinguished from those of MSA and Parkinson's disease with autonomic failure.…”

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confidence: 99%

Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy

Mabuchi

Hirayama²,

Koike³

et al. 2005

Journal of Neurology, Neurosurgery & Psychiatry

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Objective: To clarify the progression of autonomic symptoms and functional deterioration in pure autonomic failure (PAF), particularly in comparison with multiple system atrophy (MSA). Methods: The investigation involved eight patients with PAF (M/F = 7/1; mean age at onset, 57 years) and 22 with probable MSA matched for age at onset (M/F = 14/8; onset 56 years). Subjects were followed up for neurological symptoms, activities of daily living, and autonomic function for more than seven years. Autonomic functional tests were carried out. Results: In PAF, fainting or sudomotor dysfunction occurred first, followed by constipation and syncope. Urinary dysfunction developed late, and respiratory dysfunction was not evident. This clinical course contrasted sharply with that in MSA, where early urinary dysfunction usually proceeded to sudomotor dysfunction or orthostatic hypotension (p = 0.004), followed by respiratory dysfunction (p = 0.0004). Results of pharmacological tests also distinguished PAF from MSA. Progression and prognosis in patients with PAF did not worsen, unlike the steady progressive autonomic dysfunction in MSA (p,0.0001, p,0.0001, p = 0.0009, and p = 0.003, for progression to modified Rankin scale grade III, IV, V, and death, respectively). Conclusions:The time course and pattern of progression of autonomic failure differed significantly between PAF and MSA. Patients with PAF had slower functional deterioration and a better prognosis.

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“…There have been two previously reported cases of this disease who developed stridor and respiratory failure requiring tracheostomy (Roessmann et al, 1971;Israel and Marino, 1977). Two patients have also been described with typical Parkinson's disease who had glottic obstruction because of vocal cord paralysis (Vas et al, 1965).…”

Section: Discussionmentioning

confidence: 99%