Extrapontine myelinolysis (EPM) is a rare cause of parkinsonism. In this case report, we describe a 63-year-old woman with parkinsonism due to EPM after correction of hyponatremia. During a 4-year follow-up, both the clinical features of parkinsonism and the changes on magnetic resonance imaging resolved. Parkinsonism due to EPM should be recognized as it has a good prognosis

A Gocht

Bart Post

I Tomita

J Sajith

JS Kim

K Okada

M Waragai

M. A. J. Tijssen

R Salvesen

RJ Martin

S Koussa

W. A. van Gool

WD Brown

English

PubMed

Post, Bart

van Gool, W. A.

Tijssen, M. A. J.

University of Groningen Research Database

Transient parkinsonism in isolated extrapontine myelinolysis

Springer - Publisher Connector

CASE REPORT
Transient parkinsonism in isolated extrapontine myelinolysis
Bart Post Æ W. A. van Gool Æ M. A. J. Tijssen
Received: 24 April 2008 / Accepted: 15 April 2009 / Published online: 15 May 2009
 The Author(s) 2009. This article is published with open access at Springerlink.com
Abstract Extrapontine myelinolysis (EPM) is a rare
cause of parkinsonism. In this case report, we describe a
63-year-old woman with parkinsonism due to EPM after
correction of hyponatremia. During a 4-year follow-up,
both the clinical features of parkinsonism and the changes
on magnetic resonance imaging resolved. Parkinsonism
due to EPM should be recognized as it has a good
prognosis.
Keywords Parkinsonism  Extrapontine myelinolysis 
Hyponatremia
Introduction
Parkinsonism is mostly due to Parkinson’s disease, vas-
cular parkinsonism, dementia-parkinsonism or drug-
induced parkinsonism. Other than drug-induced causes of
secondary parkinsonism account for a small percentage of
cases of parkinsonism and can be due to several causes.
One of the possibilities is extrapontine myelinolysis
(EPM). Myelinolysis results from rapid correction of
hyponatremia and is generally located central in the pons,
so-called central pontine myelinolysis (CPM) but can also
be located outside the pons (EPM). Here, we describe a
case of parkinsonism due to EPM without CPM with 4-year
clinical and radiological [magnetic resonance imaging
(MRI)] follow-up and we review the recent literature on
this subject.
Case report
A 63-year-old woman was admitted to the hospital because
of nausea, vomiting, difficulty speaking and impairment of
coordination of her arms and legs. Laboratory studies
showed a severe hyponatremia of 104 mmol/l which
resulted in an acute metabolic encephalopathy. This was
corrected initially using hypertonic NaCl 1.5% and later
using isotonic NaCl 0.9% infusions. After 18 h the sodium
concentration raised to 121 mmol/l and 36 h later it was
130 mmol/l. During 1 week of hospitalisation, she expe-
rienced an improvement and was discharged from the ward
with a slight dysartria. In the past she had been diagnosed
with breast carcinoma, diverticulosis and had undergone a
cholecystectomy. Furthermore, she suffered from hyper-
tension and was on drugs for this: B-blocking agent and
diuretics (triamtereen-epitizide). The use of diuretics in
combination with the vomiting was thought to have caused
the severe hyponatremia and the drugs were discontinued.
Eight days later she was readmitted to hospital with
progressive dysartria, bradykinesia and loss of ‘‘strength’’
of her arms and legs. Within several days she deteriorated
to a bed-ridden stage. Two weeks after this deterioration,
without a clear diagnoses, she was referred to the neurol-
ogy department in our hospital.
During neurological examination the patient was alert
and well-oriented. MMSE was 28/30, where she lost two
points on memory and visuo-construction. She had a
masked face and hypo-phonic speech. Examination of the
B. Post  W. A. van Gool  M. A. J. Tijssen
Department of Neurology and Clinical Neurophysiology,
Academic Medical Center, Amsterdam, The Netherlands
B. Post
e-mail: b.post@amc.uva.nl
M. A. J. Tijssen (&)
Department of Neurology H2-222, Academic Medical Center,
P.O. Box 22660, 1100 DD Amsterdam, The Netherlands
e-mail: m.a.tijssen@amc.uva.nl
123
Neurol Sci (2009) 30:325–328
DOI 10.1007/s10072-009-0088-9
cranial nerves showed no abnormalities, with normal eye
movements. Her upper and lower limbs showed, left more
than right-sided, rigidity with cogwheeling and bradyki-
nesia. She had difficulty raising from a chair, and when
walking her armswing was decreased. The postural reflexes
were impaired. No tremor, dystonia or dyskinesias were
noted. The remaining neurological examination was with-
out abnormalities.
Blood tests showed no abnormalities. Sodium was
142 mmol/l, potassium 3.5 mmol/l, ceruloplasmin and
copper were normal, thyroid function and lactate in blood
were normal, as was the chest X-ray. The T2-weighted
images of the MRI of the brain showed bilateral and
symmetric increased intensities in the nucleus caudatus and
the putamen with sparing of the globus pallidus (Fig. 1,
top). There was no abnormal signal in the pons (Fig. 1,
top). T1-weighted images of both regions were normal.
Based on the clinical course, history of hyponatremia
with correction and the abnormalities on the MRI-scan the
diagnosis EPM without CPM was made. The patient
recovered slowly and was discharged home with parkin-
sonism; dysartria, bradykinesia and rigidity. During follow-
up at the outpatient clinic the patient gradually recovered
from her parkinsonism. Three years and 9 months after
discharge neurological examination showed only a mild
masked face, but further neurological examination was
Fig. 1 Top T2-weighted
magnetic resonance scan image
showing bilateral symmetrical
hyperintensities in caudate
nucleus and putamen with
sparing of the pons; there is also
typical sparing of the pallidum.
Bottom T2-weighted magnetic
resonance scan after 4 years of
follow-up showing a normal
intensities of caudate nucleus,
putamen and pons
326 Neurol Sci (2009) 30:325–328
123
unremarkable. MRI at this stage showed no abnormalities
on T2-weighted and FLAIR images in the nucleus caudatus
and the putamen (Fig. 1, bottom).
Discussion
We describe a case of parkinsonism after correction of
hyponatremia with radiological evidence of EPM without
CPM with the longest follow-up reported in the literature.
EPM without CPM causing parkinsonism has been
reported in Pubmed seven times since 1990 (Table 1)
[1–7]. In all reported cases, including our own case, a
severe hyponatremia with fast correction was described,
similar to our case. In seven of these cases an initial
improvement of general symptoms due to the hyponatremia
(e.g. confusion, epilepsy) was described after correction of
the hyponatremia, with a secondary deterioration after
which symptoms of parkinsonism developed. Spontaneous
recovery of this second episode occurred in days to weeks.
Six out of eight showed good recovery, which does not
mean complete recovery, after a variable follow-up time.
EPM without CPM is described in 22% of cases with
myelinolysis [8]. These EPM lesions were most frequently
seen in the cerebellum (55%), lateral geniculate body
(41%), putamen (34%), thalamus (34%) and cerebral cor-
tex/subcortex (34%) [8]. CPM was first described in 1959
by Adams. [9] In a series of 58 cases of myelinolysis with
brain autopsy in 47% of patients myelinolysis was confined
to the pons, while in 53% of patients lesions were also
located outside of the pons; EPM. In 1976 Tomlinson
suggested rapid correction of sodium as an etiological
factor for myelinolysis [9]. Myelinolysis, however, not
only appears in adult or alcoholic patients with hypona-
tremia but can also occur with normal or elevated serum
sodium levels [10]. Nowadays it is believed that myeli-
nolysis is caused by a dysbalance between brain electro-
lytes, especially sodium and chloride, and organic brain
osmolites, e.g. creatine, taurine, glutamate. With the cor-
rection of serum hyponatremia a net shrinkage of the brain
Table 1 Cases described in literature from 1990 until now with parkinsonism due to EPM without CPM
Article Cause of hyponatremia Hyponatremia Lucent interval
between correction
hyponatremia and
start myelinolysis
Follow-up Prognosis
Post (2009)
(current
case)
Diuretics and vomiting Initial: 104 mmol/l; correction to
121 mmol/l in 18 h and to
130 mmol/l in 30 h
? 4 years Good recovery
Sajith (2006) Addison’s disease; exacerbated
by dehydration and vomiting
Initial: \100 mmol/l; correction
to 130 mmol/l over 5 days
? 2 months Good recovery
Okada (2005) History transphenoidal resection
and gamma knife treatment of
prolactinoma; now episode of
pneumonia and general sickness
Initial: 109 mmol/l; correction to
120 mmol/l in 28 h
? 3 years Good recovery
Koussa (2003) Diuretics Initial: 99 meq/l; correction to
127 meq/l \ 24 h
? 4 months Good recovery
Kim (2003) Cis-platinum and vomiting Initial: 98 meq/l; correction to
128 meq/l in 20 h
? 4 months Good recovery
Salvesen [5] Transphenoidal resection of
pituarity adenoma. On hormonal
substitution therapy; episode of
water poisoning
Initial: 110 mmol/l; correction to
123 mmol/l within the first
12 h and to 139 mmol/l after
28 h
? 6 months Good recovery
Waragi (1998) Germinoma of the pineal gland
and radiation with
panhypopituitarism.
On hormonal substitution
therapy; adrenergic crisis
following cold
Initial: 105 meq/l andere EH?;
correction to 148 meq/l in
7 days
? Not stated Not stated
Tomita (1993) History of hypophysectomy after
apoplexy with concurrent
diabetes insipidus
Initial: 103 meq/l andere EH?;
correction 126 meq/l in 3 days
– 10 months Progressive
parkinsonism
without reaction to
dopaminergic
therapy
Neurol Sci (2009) 30:325–328 327
123
will occur. Oligodendroglial cells form a network around
large neurons and appear to be vulnerable to physical stress
related to shrinkage [10] leading to myelinolysis in the
typical predeliction sites for CPM and EPM. This dysbal-
ance hypothesis has led to the recommendation that cor-
rection of hyponatremia should not excess 8 mmol/l per
day [9].
We conclude that EPM is a rare cause of parkinsonism
that occurs after an initial period of recovery after rapid
correction of hyponatremia. The prognosis based, also on
the long term seems favourable, although the possibility of
publication bias can lead to overestimation of this good
prognosis.
Open Access This article is distributed under the terms of the
Creative Commons Attribution Noncommercial License which per-
mits any noncommercial use, distribution, and reproduction in any
medium, provided the original author(s) and source are credited.
References
1. Sajith J, Ditchfield A, Katifi HA (2006) Extrapontine myelinol-
ysis presenting as acute parkinsonism. BMC Neurol 6:33
2. Okada K, Nomura M, Furusyo N, Otaguro S, Nabeshima S,
Hayashi J (2005) Amelioration of extrapontine myelinolysis and
reversible parkinsonism in a patient with asymptomatic hypopi-
tuitarism. Intern Med 44(7):739–742
3. Koussa S, Nasnas R (2003) Catatonia and Parkinsonism due to
extrapontine myelinolysis following rapid correction of hypona-
tremia: a case report. J Neurol 250(1):103–105
4. Kim JS, Lee KS, Han SR, Chung YA (2003) Decreased striatal
dopamine transporter binding in a patient with extrapontine
myelinolysis. Mov Disord 18(3):342–345
5. Salvesen R (1998) Extrapontine myelinolysis after surgical
removal of a pituitary tumour. Acta Neurol Scand 98(3):213–215
6. Waragai M, Satoh T (1998) Serial MRI of extrapontine myeli-
nolysis of the basal ganglia: a case report. J Neurol Sci
161(2):173–175
7. Tomita I, Satoh H, Satoh A, Seto M, Tsujihata M, Yoshimura T
(1997) Extrapontine myelinolysis presenting with parkinsonism
as a sequel of rapid correction of hyponatraemia. J Neurol Neu-
rosurg Psychiatry 62(4):422–423
8. Gocht A, Colmant HJ (1987) Central pontine and extrapontine
myelinolysis: a report of 58 cases. Clin Neuropathol 6(6):262–
270
9. Martin RJ (2004) Central pontine and extrapontine myelinolysis:
the osmotic demyelination syndromes. J Neurol Neurosurg Psy-
chiatry 75(Suppl 3):iii22–iii28
10. Brown WD (2000) Osmotic demyelination disorders: central
pontine and extrapontine myelinolysis. Curr Opin Neurol
13(6):691–697
328 Neurol Sci (2009) 30:325–328
123


Extrapontine myelinolysis (EPM) is a rare cause of parkinsonism. In this case report, we describe a 63-year-old woman with parkinsonism due to EPM after correction of hyponatremia. During a 4-year follow-up, both the clinical features of parkinsonism and the changes on magnetic resonance imaging resolved. Parkinsonism due to EPM should be recognized as it has a good prognosis.</p

University of Groningen

ARTS repository - University of Groningen

Extrapontine myelinolysis (EPM) is a rare cause of parkinsonism. In this case report, we describe a 63-year-old woman with parkinsonism due to EPM after correction of hyponatremia. During a 4-year follow-up, both the clinical features of parkinsonism and the changes on magnetic resonance imaging resolved. Parkinsonism due to EPM should be recognized as it has a good prognosi

Post, B.

van Gool, W.A.

Tijssen, M.A.J.

International Migration, Integration and Social Cohesion online publications

UvA-DARE is a service provided by the library of the University of Amsterdam (https://dare.uva.nl)UvA-DARE (Digital Academic Repository)Transient parkinsonism in isolated extrapontine myelinolysisPost, B.; van Gool, W.A.; Tijssen, M.A.J.DOI10.1007/s10072-009-0088-9Publication date2009Document VersionFinal published versionPublished inNeurological SciencesLink to publicationCitation for published version (APA):Post, B., van Gool, W. A., & Tijssen, M. A. J. (2009). Transient parkinsonism in isolatedextrapontine myelinolysis. Neurological Sciences, 30(4), 325-328.https://doi.org/10.1007/s10072-009-0088-9General rightsIt is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s)and/or copyright holder(s), other than for strictly personal, individual use, unless the work is under an opencontent license (like Creative Commons).Disclaimer/Complaints regulationsIf you believe that digital publication of certain material infringes any of your rights or (privacy) interests, pleaselet the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the materialinaccessible and/or remove it from the website. Please Ask the Library: https://uba.uva.nl/en/contact, or a letterto: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. Youwill be contacted as soon as possible.Download date:09 Mar 2023CASE REPORTTransient parkinsonism in isolated extrapontine myelinolysisBart Post Æ W. A. van Gool Æ M. A. J. TijssenReceived: 24 April 2008 / Accepted: 15 April 2009 / Published online: 15 May 2009 The Author(s) 2009. This article is published with open access at Springerlink.comAbstract Extrapontine myelinolysis (EPM) is a rarecause of parkinsonism. In this case report, we describe a63-year-old woman with parkinsonism due to EPM aftercorrection of hyponatremia. During a 4-year follow-up,both the clinical features of parkinsonism and the changeson magnetic resonance imaging resolved. Parkinsonismdue to EPM should be recognized as it has a goodprognosis.Keywords Parkinsonism  Extrapontine myelinolysis HyponatremiaIntroductionParkinsonism is mostly due to Parkinson’s disease, vas-cular parkinsonism, dementia-parkinsonism or drug-induced parkinsonism. Other than drug-induced causes ofsecondary parkinsonism account for a small percentage ofcases of parkinsonism and can be due to several causes.One of the possibilities is extrapontine myelinolysis(EPM). Myelinolysis results from rapid correction ofhyponatremia and is generally located central in the pons,so-called central pontine myelinolysis (CPM) but can alsobe located outside the pons (EPM). Here, we describe acase of parkinsonism due to EPM without CPM with 4-yearclinical and radiological [magnetic resonance imaging(MRI)] follow-up and we review the recent literature onthis subject.Case reportA 63-year-old woman was admitted to the hospital becauseof nausea, vomiting, difficulty speaking and impairment ofcoordination of her arms and legs. Laboratory studiesshowed a severe hyponatremia of 104 mmol/l whichresulted in an acute metabolic encephalopathy. This wascorrected initially using hypertonic NaCl 1.5% and laterusing isotonic NaCl 0.9% infusions. After 18 h the sodiumconcentration raised to 121 mmol/l and 36 h later it was130 mmol/l. During 1 week of hospitalisation, she expe-rienced an improvement and was discharged from the wardwith a slight dysartria. In the past she had been diagnosedwith breast carcinoma, diverticulosis and had undergone acholecystectomy. Furthermore, she suffered from hyper-tension and was on drugs for this: B-blocking agent anddiuretics (triamtereen-epitizide). The use of diuretics incombination with the vomiting was thought to have causedthe severe hyponatremia and the drugs were discontinued.Eight days later she was readmitted to hospital withprogressive dysartria, bradykinesia and loss of ‘‘strength’’of her arms and legs. Within several days she deterioratedto a bed-ridden stage. Two weeks after this deterioration,without a clear diagnoses, she was referred to the neurol-ogy department in our hospital.During neurological examination the patient was alertand well-oriented. MMSE was 28/30, where she lost twopoints on memory and visuo-construction. She had amasked face and hypo-phonic speech. Examination of theB. Post  W. A. van Gool  M. A. J. TijssenDepartment of Neurology and Clinical Neurophysiology,Academic Medical Center, Amsterdam, The NetherlandsB. Poste-mail: b.post@amc.uva.nlM. A. J. Tijssen (&)Department of Neurology H2-222, Academic Medical Center,P.O. Box 22660, 1100 DD Amsterdam, The Netherlandse-mail: m.a.tijssen@amc.uva.nl123Neurol Sci (2009) 30:325–328DOI 10.1007/s10072-009-0088-9cranial nerves showed no abnormalities, with normal eyemovements. Her upper and lower limbs showed, left morethan right-sided, rigidity with cogwheeling and bradyki-nesia. She had difficulty raising from a chair, and whenwalking her armswing was decreased. The postural reflexeswere impaired. No tremor, dystonia or dyskinesias werenoted. The remaining neurological examination was with-out abnormalities.Blood tests showed no abnormalities. Sodium was142 mmol/l, potassium 3.5 mmol/l, ceruloplasmin andcopper were normal, thyroid function and lactate in bloodwere normal, as was the chest X-ray. The T2-weightedimages of the MRI of the brain showed bilateral andsymmetric increased intensities in the nucleus caudatus andthe putamen with sparing of the globus pallidus (Fig. 1,top). There was no abnormal signal in the pons (Fig. 1,top). T1-weighted images of both regions were normal.Based on the clinical course, history of hyponatremiawith correction and the abnormalities on the MRI-scan thediagnosis EPM without CPM was made. The patientrecovered slowly and was discharged home with parkin-sonism; dysartria, bradykinesia and rigidity. During follow-up at the outpatient clinic the patient gradually recoveredfrom her parkinsonism. Three years and 9 months afterdischarge neurological examination showed only a mildmasked face, but further neurological examination wasFig. 1 Top T2-weightedmagnetic resonance scan imageshowing bilateral symmetricalhyperintensities in caudatenucleus and putamen withsparing of the pons; there is alsotypical sparing of the pallidum.Bottom T2-weighted magneticresonance scan after 4 years offollow-up showing a normalintensities of caudate nucleus,putamen and pons326 Neurol Sci (2009) 30:325–328123unremarkable. MRI at this stage showed no abnormalitieson T2-weighted and FLAIR images in the nucleus caudatusand the putamen (Fig. 1, bottom).DiscussionWe describe a case of parkinsonism after correction ofhyponatremia with radiological evidence of EPM withoutCPM with the longest follow-up reported in the literature.EPM without CPM causing parkinsonism has beenreported in Pubmed seven times since 1990 (Table 1)[1–7]. In all reported cases, including our own case, asevere hyponatremia with fast correction was described,similar to our case. In seven of these cases an initialimprovement of general symptoms due to the hyponatremia(e.g. confusion, epilepsy) was described after correction ofthe hyponatremia, with a secondary deterioration afterwhich symptoms of parkinsonism developed. Spontaneousrecovery of this second episode occurred in days to weeks.Six out of eight showed good recovery, which does notmean complete recovery, after a variable follow-up time.EPM without CPM is described in 22% of cases withmyelinolysis [8]. These EPM lesions were most frequentlyseen in the cerebellum (55%), lateral geniculate body(41%), putamen (34%), thalamus (34%) and cerebral cor-tex/subcortex (34%) [8]. CPM was first described in 1959by Adams. [9] In a series of 58 cases of myelinolysis withbrain autopsy in 47% of patients myelinolysis was confinedto the pons, while in 53% of patients lesions were alsolocated outside of the pons; EPM. In 1976 Tomlinsonsuggested rapid correction of sodium as an etiologicalfactor for myelinolysis [9]. Myelinolysis, however, notonly appears in adult or alcoholic patients with hypona-tremia but can also occur with normal or elevated serumsodium levels [10]. Nowadays it is believed that myeli-nolysis is caused by a dysbalance between brain electro-lytes, especially sodium and chloride, and organic brainosmolites, e.g. creatine, taurine, glutamate. With the cor-rection of serum hyponatremia a net shrinkage of the brainTable 1 Cases described in literature from 1990 until now with parkinsonism due to EPM without CPMArticle Cause of hyponatremia Hyponatremia Lucent intervalbetween correctionhyponatremia andstart myelinolysisFollow-up PrognosisPost (2009)(currentcase)Diuretics and vomiting Initial: 104 mmol/l; correction to121 mmol/l in 18 h and to130 mmol/l in 30 h? 4 years Good recoverySajith (2006) Addison’s disease; exacerbatedby dehydration and vomitingInitial: \100 mmol/l; correctionto 130 mmol/l over 5 days? 2 months Good recoveryOkada (2005) History transphenoidal resectionand gamma knife treatment ofprolactinoma; now episode ofpneumonia and general sicknessInitial: 109 mmol/l; correction to120 mmol/l in 28 h? 3 years Good recoveryKoussa (2003) Diuretics Initial: 99 meq/l; correction to127 meq/l \ 24 h? 4 months Good recoveryKim (2003) Cis-platinum and vomiting Initial: 98 meq/l; correction to128 meq/l in 20 h? 4 months Good recoverySalvesen [5] Transphenoidal resection ofpituarity adenoma. On hormonalsubstitution therapy; episode ofwater poisoningInitial: 110 mmol/l; correction to123 mmol/l within the first12 h and to 139 mmol/l after28 h? 6 months Good recoveryWaragi (1998) Germinoma of the pineal glandand radiation withpanhypopituitarism.On hormonal substitutiontherapy; adrenergic crisisfollowing coldInitial: 105 meq/l andere EH?;correction to 148 meq/l in7 days? Not stated Not statedTomita (1993) History of hypophysectomy afterapoplexy with concurrentdiabetes insipidusInitial: 103 meq/l andere EH?;correction 126 meq/l in 3 days– 10 months Progressiveparkinsonismwithout reaction todopaminergictherapyNeurol Sci (2009) 30:325–328 327123will occur. Oligodendroglial cells form a network aroundlarge neurons and appear to be vulnerable to physical stressrelated to shrinkage [10] leading to myelinolysis in thetypical predeliction sites for CPM and EPM. This dysbal-ance hypothesis has led to the recommendation that cor-rection of hyponatremia should not excess 8 mmol/l perday [9].We conclude that EPM is a rare cause of parkinsonismthat occurs after an initial period of recovery after rapidcorrection of hyponatremia. The prognosis based, also onthe long term seems favourable, although the possibility ofpublication bias can lead to overestimation of this goodprognosis.Open Access This article is distributed under the terms of theCreative Commons Attribution Noncommercial License which per-mits any noncommercial use, distribution, and reproduction in anymedium, provided the original author(s) and source are credited.References1. Sajith J, Ditchfield A, Katifi HA (2006) Extrapontine myelinol-ysis presenting as acute parkinsonism. BMC Neurol 6:332. Okada K, Nomura M, Furusyo N, Otaguro S, Nabeshima S,Hayashi J (2005) Amelioration of extrapontine myelinolysis andreversible parkinsonism in a patient with asymptomatic hypopi-tuitarism. Intern Med 44(7):739–7423. Koussa S, Nasnas R (2003) Catatonia and Parkinsonism due toextrapontine myelinolysis following rapid correction of hypona-tremia: a case report. J Neurol 250(1):103–1054. Kim JS, Lee KS, Han SR, Chung YA (2003) Decreased striataldopamine transporter binding in a patient with extrapontinemyelinolysis. Mov Disord 18(3):342–3455. Salvesen R (1998) Extrapontine myelinolysis after surgicalremoval of a pituitary tumour. Acta Neurol Scand 98(3):213–2156. Waragai M, Satoh T (1998) Serial MRI of extrapontine myeli-nolysis of the basal ganglia: a case report. J Neurol Sci161(2):173–1757. Tomita I, Satoh H, Satoh A, Seto M, Tsujihata M, Yoshimura T(1997) Extrapontine myelinolysis presenting with parkinsonismas a sequel of rapid correction of hyponatraemia. J Neurol Neu-rosurg Psychiatry 62(4):422–4238. Gocht A, Colmant HJ (1987) Central pontine and extrapontinemyelinolysis: a report of 58 cases. Clin Neuropathol 6(6):262–2709. Martin RJ (2004) Central pontine and extrapontine myelinolysis:the osmotic demyelination syndromes. J Neurol Neurosurg Psy-chiatry 75(Suppl 3):iii22–iii2810. Brown WD (2000) Osmotic demyelination disorders: centralpontine and extrapontine myelinolysis. Curr Opin Neurol13(6):691–697328 Neurol Sci (2009) 30:325–328123

UvA-DARE is a service provided by the library of the University of Amsterdam (https://dare.uva.nl)UvA-DARE (Digital Academic Repository)Transient parkinsonism in isolated extrapontine myelinolysisPost, B.; van Gool, W.A.; Tijssen, M.A.J.DOI10.1007/s10072-009-0088-9Publication date2009Document VersionFinal published versionPublished inNeurological SciencesLink to publicationCitation for published version (APA):Post, B., van Gool, W. A., & Tijssen, M. A. J. (2009). Transient parkinsonism in isolatedextrapontine myelinolysis. Neurological Sciences, 30(4), 325-328.https://doi.org/10.1007/s10072-009-0088-9General rightsIt is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s)and/or copyright holder(s), other than for strictly personal, individual use, unless the work is under an opencontent license (like Creative Commons).Disclaimer/Complaints regulationsIf you believe that digital publication of certain material infringes any of your rights or (privacy) interests, pleaselet the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the materialinaccessible and/or remove it from the website. Please Ask the Library: https://uba.uva.nl/en/contact, or a letterto: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. Youwill be contacted as soon as possible.Download date:10 Nov 2022CASE REPORTTransient parkinsonism in isolated extrapontine myelinolysisBart Post Æ W. A. van Gool Æ M. A. J. TijssenReceived: 24 April 2008 / Accepted: 15 April 2009 / Published online: 15 May 2009 The Author(s) 2009. This article is published with open access at Springerlink.comAbstract Extrapontine myelinolysis (EPM) is a rarecause of parkinsonism. In this case report, we describe a63-year-old woman with parkinsonism due to EPM aftercorrection of hyponatremia. During a 4-year follow-up,both the clinical features of parkinsonism and the changeson magnetic resonance imaging resolved. Parkinsonismdue to EPM should be recognized as it has a goodprognosis.Keywords Parkinsonism  Extrapontine myelinolysis HyponatremiaIntroductionParkinsonism is mostly due to Parkinson’s disease, vas-cular parkinsonism, dementia-parkinsonism or drug-induced parkinsonism. Other than drug-induced causes ofsecondary parkinsonism account for a small percentage ofcases of parkinsonism and can be due to several causes.One of the possibilities is extrapontine myelinolysis(EPM). Myelinolysis results from rapid correction ofhyponatremia and is generally located central in the pons,so-called central pontine myelinolysis (CPM) but can alsobe located outside the pons (EPM). Here, we describe acase of parkinsonism due to EPM without CPM with 4-yearclinical and radiological [magnetic resonance imaging(MRI)] follow-up and we review the recent literature onthis subject.Case reportA 63-year-old woman was admitted to the hospital becauseof nausea, vomiting, difficulty speaking and impairment ofcoordination of her arms and legs. Laboratory studiesshowed a severe hyponatremia of 104 mmol/l whichresulted in an acute metabolic encephalopathy. This wascorrected initially using hypertonic NaCl 1.5% and laterusing isotonic NaCl 0.9% infusions. After 18 h the sodiumconcentration raised to 121 mmol/l and 36 h later it was130 mmol/l. During 1 week of hospitalisation, she expe-rienced an improvement and was discharged from the wardwith a slight dysartria. In the past she had been diagnosedwith breast carcinoma, diverticulosis and had undergone acholecystectomy. Furthermore, she suffered from hyper-tension and was on drugs for this: B-blocking agent anddiuretics (triamtereen-epitizide). The use of diuretics incombination with the vomiting was thought to have causedthe severe hyponatremia and the drugs were discontinued.Eight days later she was readmitted to hospital withprogressive dysartria, bradykinesia and loss of ‘‘strength’’of her arms and legs. Within several days she deterioratedto a bed-ridden stage. Two weeks after this deterioration,without a clear diagnoses, she was referred to the neurol-ogy department in our hospital.During neurological examination the patient was alertand well-oriented. MMSE was 28/30, where she lost twopoints on memory and visuo-construction. She had amasked face and hypo-phonic speech. Examination of theB. Post  W. A. van Gool  M. A. J. TijssenDepartment of Neurology and Clinical Neurophysiology,Academic Medical Center, Amsterdam, The NetherlandsB. Poste-mail: b.post@amc.uva.nlM. A. J. Tijssen (&)Department of Neurology H2-222, Academic Medical Center,P.O. Box 22660, 1100 DD Amsterdam, The Netherlandse-mail: m.a.tijssen@amc.uva.nl123Neurol Sci (2009) 30:325–328DOI 10.1007/s10072-009-0088-9cranial nerves showed no abnormalities, with normal eyemovements. Her upper and lower limbs showed, left morethan right-sided, rigidity with cogwheeling and bradyki-nesia. She had difficulty raising from a chair, and whenwalking her armswing was decreased. The postural reflexeswere impaired. No tremor, dystonia or dyskinesias werenoted. The remaining neurological examination was with-out abnormalities.Blood tests showed no abnormalities. Sodium was142 mmol/l, potassium 3.5 mmol/l, ceruloplasmin andcopper were normal, thyroid function and lactate in bloodwere normal, as was the chest X-ray. The T2-weightedimages of the MRI of the brain showed bilateral andsymmetric increased intensities in the nucleus caudatus andthe putamen with sparing of the globus pallidus (Fig. 1,top). There was no abnormal signal in the pons (Fig. 1,top). T1-weighted images of both regions were normal.Based on the clinical course, history of hyponatremiawith correction and the abnormalities on the MRI-scan thediagnosis EPM without CPM was made. The patientrecovered slowly and was discharged home with parkin-sonism; dysartria, bradykinesia and rigidity. During follow-up at the outpatient clinic the patient gradually recoveredfrom her parkinsonism. Three years and 9 months afterdischarge neurological examination showed only a mildmasked face, but further neurological examination wasFig. 1 Top T2-weightedmagnetic resonance scan imageshowing bilateral symmetricalhyperintensities in caudatenucleus and putamen withsparing of the pons; there is alsotypical sparing of the pallidum.Bottom T2-weighted magneticresonance scan after 4 years offollow-up showing a normalintensities of caudate nucleus,putamen and pons326 Neurol Sci (2009) 30:325–328123unremarkable. MRI at this stage showed no abnormalitieson T2-weighted and FLAIR images in the nucleus caudatusand the putamen (Fig. 1, bottom).DiscussionWe describe a case of parkinsonism after correction ofhyponatremia with radiological evidence of EPM withoutCPM with the longest follow-up reported in the literature.EPM without CPM causing parkinsonism has beenreported in Pubmed seven times since 1990 (Table 1)[1–7]. In all reported cases, including our own case, asevere hyponatremia with fast correction was described,similar to our case. In seven of these cases an initialimprovement of general symptoms due to the hyponatremia(e.g. confusion, epilepsy) was described after correction ofthe hyponatremia, with a secondary deterioration afterwhich symptoms of parkinsonism developed. Spontaneousrecovery of this second episode occurred in days to weeks.Six out of eight showed good recovery, which does notmean complete recovery, after a variable follow-up time.EPM without CPM is described in 22% of cases withmyelinolysis [8]. These EPM lesions were most frequentlyseen in the cerebellum (55%), lateral geniculate body(41%), putamen (34%), thalamus (34%) and cerebral cor-tex/subcortex (34%) [8]. CPM was first described in 1959by Adams. [9] In a series of 58 cases of myelinolysis withbrain autopsy in 47% of patients myelinolysis was confinedto the pons, while in 53% of patients lesions were alsolocated outside of the pons; EPM. In 1976 Tomlinsonsuggested rapid correction of sodium as an etiologicalfactor for myelinolysis [9]. Myelinolysis, however, notonly appears in adult or alcoholic patients with hypona-tremia but can also occur with normal or elevated serumsodium levels [10]. Nowadays it is believed that myeli-nolysis is caused by a dysbalance between brain electro-lytes, especially sodium and chloride, and organic brainosmolites, e.g. creatine, taurine, glutamate. With the cor-rection of serum hyponatremia a net shrinkage of the brainTable 1 Cases described in literature from 1990 until now with parkinsonism due to EPM without CPMArticle Cause of hyponatremia Hyponatremia Lucent intervalbetween correctionhyponatremia andstart myelinolysisFollow-up PrognosisPost (2009)(currentcase)Diuretics and vomiting Initial: 104 mmol/l; correction to121 mmol/l in 18 h and to130 mmol/l in 30 h? 4 years Good recoverySajith (2006) Addison’s disease; exacerbatedby dehydration and vomitingInitial: \100 mmol/l; correctionto 130 mmol/l over 5 days? 2 months Good recoveryOkada (2005) History transphenoidal resectionand gamma knife treatment ofprolactinoma; now episode ofpneumonia and general sicknessInitial: 109 mmol/l; correction to120 mmol/l in 28 h? 3 years Good recoveryKoussa (2003) Diuretics Initial: 99 meq/l; correction to127 meq/l \ 24 h? 4 months Good recoveryKim (2003) Cis-platinum and vomiting Initial: 98 meq/l; correction to128 meq/l in 20 h? 4 months Good recoverySalvesen [5] Transphenoidal resection ofpituarity adenoma. On hormonalsubstitution therapy; episode ofwater poisoningInitial: 110 mmol/l; correction to123 mmol/l within the first12 h and to 139 mmol/l after28 h? 6 months Good recoveryWaragi (1998) Germinoma of the pineal glandand radiation withpanhypopituitarism.On hormonal substitutiontherapy; adrenergic crisisfollowing coldInitial: 105 meq/l andere EH?;correction to 148 meq/l in7 days? Not stated Not statedTomita (1993) History of hypophysectomy afterapoplexy with concurrentdiabetes insipidusInitial: 103 meq/l andere EH?;correction 126 meq/l in 3 days– 10 months Progressiveparkinsonismwithout reaction todopaminergictherapyNeurol Sci (2009) 30:325–328 327123will occur. Oligodendroglial cells form a network aroundlarge neurons and appear to be vulnerable to physical stressrelated to shrinkage [10] leading to myelinolysis in thetypical predeliction sites for CPM and EPM. This dysbal-ance hypothesis has led to the recommendation that cor-rection of hyponatremia should not excess 8 mmol/l perday [9].We conclude that EPM is a rare cause of parkinsonismthat occurs after an initial period of recovery after rapidcorrection of hyponatremia. The prognosis based, also onthe long term seems favourable, although the possibility ofpublication bias can lead to overestimation of this goodprognosis.Open Access This article is distributed under the terms of theCreative Commons Attribution Noncommercial License which per-mits any noncommercial use, distribution, and reproduction in anymedium, provided the original author(s) and source are credited.References1. Sajith J, Ditchfield A, Katifi HA (2006) Extrapontine myelinol-ysis presenting as acute parkinsonism. BMC Neurol 6:332. Okada K, Nomura M, Furusyo N, Otaguro S, Nabeshima S,Hayashi J (2005) Amelioration of extrapontine myelinolysis andreversible parkinsonism in a patient with asymptomatic hypopi-tuitarism. Intern Med 44(7):739–7423. Koussa S, Nasnas R (2003) Catatonia and Parkinsonism due toextrapontine myelinolysis following rapid correction of hypona-tremia: a case report. J Neurol 250(1):103–1054. Kim JS, Lee KS, Han SR, Chung YA (2003) Decreased striataldopamine transporter binding in a patient with extrapontinemyelinolysis. Mov Disord 18(3):342–3455. Salvesen R (1998) Extrapontine myelinolysis after surgicalremoval of a pituitary tumour. Acta Neurol Scand 98(3):213–2156. Waragai M, Satoh T (1998) Serial MRI of extrapontine myeli-nolysis of the basal ganglia: a case report. J Neurol Sci161(2):173–1757. Tomita I, Satoh H, Satoh A, Seto M, Tsujihata M, Yoshimura T(1997) Extrapontine myelinolysis presenting with parkinsonismas a sequel of rapid correction of hyponatraemia. J Neurol Neu-rosurg Psychiatry 62(4):422–4238. Gocht A, Colmant HJ (1987) Central pontine and extrapontinemyelinolysis: a report of 58 cases. Clin Neuropathol 6(6):262–2709. Martin RJ (2004) Central pontine and extrapontine myelinolysis:the osmotic demyelination syndromes. J Neurol Neurosurg Psy-chiatry 75(Suppl 3):iii22–iii2810. Brown WD (2000) Osmotic demyelination disorders: centralpontine and extrapontine myelinolysis. Curr Opin Neurol13(6):691–697328 Neurol Sci (2009) 30:325–328123

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Extrapontine myelinolysis (EPM) is a rare cause of Parkinsonism. In this case report, we describe a 63-year-old woman with Parkinsonism due to EPM after correction of hyponatremia. During a 4-year follow-up, both the clinical features of Parkinsonism and the changes on magnetic resonance imaging resolved. Parkinsonism due to EPM should be recognized as it has a good prognosi

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Transient Parkinsonism in isolated extrapontine myelinolysis

Amelioration of extrapontine myelinolysis and reversible parkinsonism in a patient with asymptomatic hypopituitarism.

Catatonia and Parkinsonism due to extrapontine myelinolysis following rapid correction of hyponatremia: a case report.

Central pontine and extrapontine myelinolysis: a report of 58 cases.

Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes.

Decreased striatal dopamine transporter binding in a patient with extrapontine myelinolysis.

Extrapontine myelinolysis after surgical removal of a pituitary tumour.

Extrapontine myelinolysis presenting as acute parkinsonism.

Extrapontine myelinolysis presenting with parkinsonism as a sequel of rapid correction of hyponatraemia.

Osmotic demyelination disorders: central pontine and extrapontine myelinolysis.

Serial MRI of extrapontine myelinolysis of the basal ganglia: a case report.

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Transient parkinsonism in isolated extrapontine myelinolysis

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