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Rechilda Rhea Pastor Reyes, MD, RND


Department of Neurosciences
Section of Neurology
Makati Medical Center

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ðntroduction

‡ Olivopontoccerebellar atrophy is listed as


a rare disease by the Office of Rare
Diseases (ORD) of the National ðnstitute of
Health (NðH).

‡ Clinicopathological study of 35 cases of multiple system atrophy.


G K Wenning, Y Ben-Shlomo, M Magalhães, S E Daniel, and N P Quinn
University Department of Clinical Neurology, ðnstitute of Neurology, London, UK.
J Neurol Neurosurg Psychiatry. 1995 February; 58(2): 160±166.
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ðntroduction

‡ Olivopontocerebellar Atrophy affects less


than 200,000 people in the US population.

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Case Presentation

‡ 5 years PTC, patient¶s condition worsened


by noticing that she has been holding onto
something to keep her stable when
walking
‡ 3 years PTC, involuntarily shaking of
head more prominent when the patient is
at rest

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Case Presentation

‡ 2 years PTC, patient worked at a company


as a janitress but she only stayed for a
month because she keeps on bumping to
things around her due to the way she
walks.
‡ Persistence of symptoms prompted
consultation.

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Case Presentation

‡ Patient has no previous illnesses, no


previous hospitalization
‡ Patient is non-smoker but drinks Ginebra
San Miguel 750 ml twice a week for 12
years

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Case Presentation

‡ Patient was employed at Valor electronics


at Cabuyao, Laguna as a production
operator from June 1994 to 2000.
‡ Patient was exposed to the following
chemials: lead, naphtha (petroleum), N-
hexane.
‡ Since then patient has been unemployed
and did not had any chance to work again

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Case Presentation

‡ On Cranial nerve testing, weak gag was


noted.
‡ There was no note of nystagmus

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Case Presentation

‡ On cerebellar testing, patient was noted to


have intention tremor, overshoot,
dysdiadochokinesia, dysmetria, bilateral
truncal Ataxia, titubation of the head, wide
based gait, has tendency to fall on either
side, and unable to do tandem walk

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T2

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T2

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SWAN

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T1
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FiberTrak

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Discussion

‡ The median age of onset was 55 (range


33.3-75.8) years and median survival was
7.3 (range 2.1-11.5) years.

‡ Clinicopathological study of 35 cases of multiple system atrophy.


G K Wenning, Y Ben-Shlomo, M Magalhães, S E Daniel, and N P Quinn
University Department of Clinical Neurology, ðnstitute of Neurology, London, UK.
J Neurol Neurosurg Psychiatry. 1995 February; 58(2): 160±166.

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Discussion

± Long term intake of moderate doses of


alcohol daily for 20-30 years may damage the
cerebellum before the onset of macroscopical
atrophy.

± Despite distinct individual differences an all


year average daily alcohol intake of 41-80 g
results in a risk of significant loss of Purkinje
cells.

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Discussion

‡ Lead in nervous system concentrates


in gray matter and specific nuclei

‡ Highest concentration in
Hippocampus > cerebellum> Cerebral
cortex and Medulla

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Conclusion

‡ The industrial solvents can greatly cause a


loss of Purkinje cells and result to a severe
cerebellar degeneration.

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Thank you

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