Case Report Sarcoidosis: A Cause For Bilateral Facial Palsy

Journal of Neurological Sciences
2005;22(3)
Journal of Neurological Sciences [Turkish] 22:(3)# 43; 304-308, 2005
http://www.jns.dergisi.org/text.php3?id=43
Case Report
Sarcoidosis: A Cause For Bilateral Facial Palsy
Kadir KOTIL, Bekir KILINÇ
Haseki Educational and Research Hospital, Neurosurgery, İSTANBUL, Türkiye
Abstract
Sarcoidosis is a systemic, chronic, granulomatous disease and it mainly affects lung. Bilateral
facial nerve involvoment due to occult sarcoidosis is an extremely rare phenomenon that can
be the presenting complaint in a wide spectrum of the disease.Case report. A 38 year-old man
presented to our clinic with facial diplegia. His general, neurologic (except facial diplegia),
ocular examination and laboratory tests were normal. There was no respiratory system
symptom. Chest X-ray revealed bilateral hilar and paratracheal lymphadenopathy without
atelectesia. In sarcoidosis, definitive diagnosis was made by bronco-alveoalar leavage.
Corticosteroid therapy was not applied, because, he had have stage I sarcoidosis. First left
sided and later right sided facial palsy detoriated and almost recovered in 7 weeks from
admission. The abnormalities seen on chest x-ray resolved slowly over this period. He has
been followed up in our out-patient clinic without any medication after discharge and no
recurrent sign or symptoms have appeared so far.
This paper describes a spesific unique case of occult facial diplegia occuring in a patient
having stage I occult sarcoidosis. We believe, this phenomenon proved that facial diplegia can
be created by the infiltration just only the seventh cranial nerves and the most important point
to be stressed in this phenomenon should be the investigation of sarcoidosis among the facial
diplegic cases
Keywords: Sarcoidosis, facial diplegia
Sarkoidoz: İki Taraflı Yüz Felcinin Bir Nedeni
Özet
Sarkoidoz sistemik, kronik, granülomatöz bir hastalıktır ve başlıca etkilediği organ
akciğerlerdir. Sarkoidozdan dolayı iki taraflı yüz siniri etkilenimi bu hastalığın çok geniş
spektrumunda, ilk sunu yakınması olarak ortaya çıkması oldukca nadirdir.Olgu:38 yaşında
erkek olgu iki taraflı yüz felçi ile başvurdu. Genel sistemik, nörolojik ve göz muayenesi
bilateral yüz felci hariç normal bulundu. Solunum sistemine ait semptomu yoktu. Göğüs
röntgeninde, atelektazi olmadan bilateral hilar ve paratrakeal lenfadenopati tespit edildi. Kesin
tanısı, bronkoalveolar lavajla kondu. Kortikosteroid tedavisi uygulamadan önce sol taraf
sonrada sağ taraf olmak üzere hastanın tanısından 7 hafta sonra fasiyal diplejisi düzelmiştir.
Bu süre içinde de göğüs filmindeki görüntüler kayboldu. Hastaya evre I sarkoidoz olduğundan
kortikosteriod tedavisi uygulanmamıştır ve takibi süresi boyunca rekürens oluşmamıştır.
Bu makale, evre I sarkoidozda yanı daha sarkoidoz belirtileri ortaya çıkmadan fasiyal dipleji
ile ortaya çıkabileceğini gösteren ilk olgudur. Bu nedenle fasiyal diplejili olgularda bu
fenomeni hatırlamak ve araştırmayı bunun üzerine kurmak gerekir kanısındayız.
Anahtar Kelimeler: Sarkoidozis, bilateral yüz felci
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J.Neurol.Sci.[Turk]
Introduction
Facial nerve paralysis ( prosopoplgia ) is a
facial neuropathy commonly seen with
Bell’s palsy having an annual incidence
rate of 23 to 25 patients per 100,000
population 5,6,8,14-19. Bilateral form occurs
in only 0.3 – 2.0 % of patients with
idiopathic facial paralysis, and it differs
from the more common recurrent or
alternating palsy 4,14-20. Facial diplegia
might rarely be the sole presenting sign of
sarcoidosis – a multisystemic chronic
granulomatous disease of unknown
etiology – frequently affecting individuals
20 - 40 years of age 1,3,5,7,9,10,11-22. Although
sarcoidosis emerges primarily in the lungs
causing infiltration as a response to
immune system disorder , it can affect any
organ other than the lungs. Neurological
manifestations occur in approximately 5 %
of patients with sarcoidosis 2,16 and 50 %
of these patients suffer from peripheral
facial palsy 14. Another clinical study
records that 4 % of sarcoidosis patients
have neuro logical involvements, half of
these have facial nerve involvement and
one-sixth have facial diplegia and this
contributes approximately 7 cases per
1,000 patients 2.Although cranial nerve
palsy is the most common complication, its
mechanism
remains
unclear
.
However, it is thought to be infiltration of
nerve by sarcoid granuloma 15.
This paper describes a spesific case of
facial diplegia occuring in a patient having
stage 1 occult sarcoidosis.
Another dazzling point in this case is that
facial diplegia was created by the
infiltration just only in the seventh cranial
nerve.
Figure1 Direct postero-anterior chest x-ray
performed at presentation revealing hilar,
mediastinal and paratracheal lymphadenopathy
(arrows).
Since he had dry eyes bilaterally so that
eyes closed therapy with artificial tears and
vitamin replacement were commenced.
Corticosteroid therapy wasn’t applied. First
2,14
Case Presentation
A 38 year-old man presented to our clinic
with bilateral facial diplegia. Developing
on the left side one week before, and on
the right three days prior to admission. He
was able to close both of his eyes almost
completely and demonstrated bilateral
Bell’s phenomenon. There was no both
nasal reflux of fluids on swallowing and no
palatal paresis. His general examination
and laboratory tests were normal. Serum
angiotensin converting enzyme (ACE)
level was normal. There is no other neural
involvement. There was no respiratory
symptom. Magnetic resonace imaging
(MRI) of the brain was normal.
Cerebrospinal fluid examination showed
no abnnormalitiy.
Chest X-ray revealed bilateral hilar and
paratracheal lymphadenopathy without
atelectesia (Fig. 1), proving typical
characteristics for the presentation of stage
I sarcoidosis. The tracheal aspiration was
performed, and it was diagnosed as
sarcoidosis
by
histopathological
examination. Histopathologic examination
of mediastinoscopic biopsy samples
disclosed
noncaseating
granulomas
composed of epithelioid cells with
scattered lympohcytes and Langhans' type
giant cells. No Schaumann bodies, asteroid
bodies or calcium oxalate crystals were
found (Fig 2 A&B)
305
J.Neurol.Sci.[Turk]
left sided and later right sided palsy further
detoriated and almost recovered in 7 weeks
from admission. The abnormalities seen on
chest X-ray resolved slowly over this
period. He has been followed up in our
out-patient clinic without medication after
discharge and no recurrent sign or
symptoms have appeared so far.
associated with bilateral facial paralysis 5.
Over all, the cranial nerves are involved in
5 % of cases of sarcoid 3,10. Multiple
cranial nerve palsies are often present 3
with facial nerve, being the most
frequently involved 2,3,10,16-21. Unilateral
facial nerve involvement occurs 22-66 %
of cases and bilateral involvement occurs
in 11 % of cases 3,10,17. In most cases, the
paralysis is only temporarily affected from
the lower motor neuron origin 11,15.
The pathogenesis is thought to be
infiltration of nerve by sarcoid granuloma
11
. The onset of the palsy is often sudden
and the palsy may be incomplete 10. The
response to steroids is thought to be
favorable 3 and facial nerve decompression
is indispensable only if there is no response
to steroids 3,9-11,15-17. Although the course is
variable, the palsy usullay resolves
spontenously 1,5,7,10,16-22, as in our case
report.
Figure2: A Many small, well circumscribed
granulomas (arrow) (H&E x100). B. Typical
noncaseating hard granuloma with Langhans' type
giant cell (H&E x400)
In a case of facial diplegia, the unique most
important diagnostic tool is a history and a
careful full examination, as this may
suggest to definitive diagnosis. Elevated
serum ACE level may not elevated all
patients, for thus it is not diagnostic tool
1,5,7,15-18
. In conclusion, this report obtains
evidence that facial diplegia in occult
sarcoidosis: a) starts in suddenly, b) may
fully reversible without steroid therapy, c)
this phenomenon proved that facial
diplegia can be created by the infiltration
just only the seventh cranial nerves and the
most important point to be stressed in this
phenomenon should be the investigation of
sarcoidosis among the facial diplegic
cases.
Disscussion
Leprosy, syphilis, bacterial meningitis,
leukemia, sarcoidosis and Guillan-Barre
syndrome are the diseases, most commonly
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J.Neurol.Sci.[Turk]
References
1) Chapelon C, Ziza JM, Piette JC.
Neurosarcoidosis: signs, course and
treatment in 35 confirmed cases. Medicine
1990;69:261-276.
2) Colover J. Sarcoidosis with involvement
of the nervous system. Brain 1948;71:451454
3) Delany P: Neurologic manifestations of
sarcoid : review of the literature with a
review of 23 cases. Ann Intern Med
1977;87(3):336-45
4) Friedman JH, Challenor Y, Brust JCM .
Bilateral facial paralysis associated with
Stevens-Jhonson
syndrome.
Neurol
1979;29 :1304-1306.
5) George MK, Pahor AL. Sarcoidosis: a
cause for bilateral facial palsy. Ear Nose
Throat J. 1991:70;492-3.
6) Hauser WA, Karnes WE, Annis J.
Incidence and prognosis of Bell’s Palsy in
the population of Rochester, Minnesota.
Mayo Clin Proc 1971;46:258-264.
7) Haydar AA, Hujairi NM, Tawil A,
Savaya R. Bilateral facial paralysis: what's
the cause? MJA 2003; 179 (10):553.
8) Hoitsma E, Faber CG, Drent M, Sharma
OP. Neurosarcoidosis: a clinical dilemma.
Lancet Neurol. 2004;3(7):397-407
9) James DG, Sharma OP. Neurological
complications of sarcoidosis. J R Soc Med
1967;62:1169-1170
10) Katusic SK, Beard CM, Wiederholt
WC. Incidence clinical features, and
prognosis in Bell’s palsy, Rochester,
Minnesota,1968-1982.
Ann
Neurol
1986;20:622- 627.
11) Lambert V, Richards SH. Facial palsy
in Heerfordt’s syndrome. J Laryng Otol
1964;78:684-693.
Correspondence
Kadir KOTIL
Haseki Educational and Research Hospital,
Neurosurgery, İSTANBUL, Türkiye
e-mail: [email protected]
12) Lower EE, Broderick JP, Brott TG.
Baughman RP. Diagnosis and management
of neurological sarcoidosis.Arch Intern
Med. 1997;157(16):1864-8.
13) Matthews WB. Sarcoidosis of the
nervous system. J Neurol Neurosurg
Psychiatry 1965;28 :23-9.
14)
McIntosh
WE,
Brenner
JF,
Aschenbrenner JE. Bilateral facial
paralysis as the sole presenting feature of
sarcoidosis: report of a case. J Am
Osteopath Assoc. 1987 Mar; 87(3) 245-7
15) Oki M, Takizawa S, Ohnuki Y. MRI
findings
of
VIIth
cranial
nerve
involvement in sarcoidosis. Br J Radiol
1997;70:859-861
16) Rontal E, Sigel ME. Bilateral facial
pralysis. Laryngoscope 1971; 82: 607-616.
17) Scott TF. Neurosarcoidosis: progress
and clinical aspects. Neurology 1993;43
(1):8-12
18) Sharma OP, Sharma AM. Sarcoidosis
of the nervous system. A clinical
Approach.
Arch
Intern
Med
1991;151(7):1317-21
19) Sharma SK, Mohan A. Uncommon
manifestations of sarcoidosis. J Assoc
Physicians India. 2004 Mar;52:210-4.
20) Sharma OP. Neurosarcoidosis: a
personal perspective based on the study of
37 patients. Chest 1997;112(1):220-8.
21) Silverstein A, Feuer MM, Siltzbach
LE. Neurologic sarcoidosis. Arch Neurol
1965;12:1-11.
22) Stern BJ, Krumholz A, Johns C.
Sarcoidosis
and
its
neurological
manifestations.
Arch
Neurol.
1985;42(9):909-17.
Recived by: 29.08.2005
Revised by: 02.09.2005
Accepted: 06.09.2005
307
J.Neurol.Sci.[Turk]
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