case report

Wien Klin Wochenschr (2011) 123: 512514

DOI 10.1007/s00508-011-0010-3
Springer-Verlag 2011
Printed in Austria

Wiener klinische Wochenschrift

The Central European Journal of Medicine

Charcot shoulder caused by Chiari type I malformation

with syringomyelia with six-year follow-up
Gordan Grahovac1, Milorad Vilendecic1, Dubravka Srdoc2
1
2

Department of Neurosurgery, University Hospital Dubrava, Zagreb, Croatia

Department of Radiology, University Hospital Dubrava, Zagreb, Croatia

Received January 20, 2011, accepted after revision May, 13, 2011, published online July 12, 2011

Durch Chiari Typ 1 Malformation ausgelste

Charcot Schulter mit Syringomyelie: 6 Jahre
Follow-up.
Zusammenfassung. Wir berichten radiologische und
klinische Details einer Patientin mit einer durch Syringomyelie und Chiari Typ 1 Malformation ausgelsten
neuropathischen Gelenkserkrankung der Schulter. Neuropathische Gelenke sind 1868 erstmals von Charcot beschrieben worden. In der Folge wurden sie bei vielen Arten
von peripheren und zentralen Nervenerkrankungen beobachtet. Es gibt viele Grnde fr eine Syringomyelie einer
davon ist die Chiari Typ 1 Malformation. Schulter und Ellbogen sind am hufigsten von der Syringomyelie-induzierten Neuropathie befallen. Unsere Patientin wurde 6 Jahre
seit der Diagnosestellung einer neuropathischen Arthropathie verbunden mit einer durch Chiari Typ 1 Malformation
induzierten Syringomyelie kontrolliert. Nach der Operation zeigte sie keinerlei Progression der Erkrankung.
Summary. We are presenting a case report of neuropathic
arthropathy of the shoulder secondary to a syringomyelia
and Chiari type I malformation, with detailed clinical and
radiological findings. Neuropathic joints were first described in 1868 by Charcot, and subsequently were described in many types of peripheral diseases and central
neuron diseases. There are many causes of syringomyelia,
and one of the causes of this disease is a Chiari type I malformation. Subsequently, shoulders and elbows are most
commonly affected by syringomyelia-induced neuropathy. Our patient was observed for six years after being diagnosed with neuropathic arthropathy and syringomyelia
caused by Chiari and did not show any disease progression after their surgery.
Key words: Arnold-Chiari malformation, type 1, arthropathy, neurogenic, syringomyelia, shoulder.
Correspondence: Gordan Grahovac, MD, Department of
Neurosurgery, University Hospital Dubrava, 10000 Zagreb, Croatia,
E-mail: ggrahov@mef.hr

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Charcot shoulder caused by Chiari type I malformation

Introduction
Neuropathic joint disease, also called Charcot neuroarthropathy, is a progressive and chronic degenerative disease which causes loss of sensation and pain or destruction
of the affected joint. Patients with neuropathy are particularly prone to developing neuropathic arthropathy (NA).
The joints most frequently affected by syringomyelia are
shoulders and elbows, weight bearing joints such as knees
and hips in tabes dorsalis, and ankle and foot in diabetes
mellitus [1]. We will present a case of neuropathic arthropathy of the shoulder with secondary to syringomyelia and
Chiari type I malformation and a six-year follow-up with
the patient in question.

Case report
A 62-year-old woman was admitted to our department six
years ago because of a swelling of the left shoulder that had
started six months earlier. The patient stated that during
the last ten years she had experienced numbness and a
sensation of decreased temperature in her left arm, and
she was burnt on several occasions. Various specialists including neurologists and orthopedic surgeons had examined her. Six months before we admitted this patient, she
had noticed swelling and redness of the skin of the left
shoulder. During our neurological examination, there was
a loss of superficial feeling, pain and increased temperature in the C3-Th1 dermatomes of the left arm. Deep tendon reflexes were normal on the upper and lower
extremities. Other neurological examinations were within
normal ranges. During the examination, her left shoulder
was swollen with limited abduction, and with crepitation
in all directions of shoulder movement. Old burn scars
were evident on the left forearm.
An anteroposterior plain radiograph of the left shoulder
revealed the absence of the humeral head, bone fragmentation and subluxation of the articular surfaces (Fig. 1). A
magnetic resonance (MR) of the cervical and thoracic
spine revealed a Chiari type I malformation and syringomyelia from C1 to Th5 level (Fig. 2). An MR of the left shoul Springer-Verlag 1516/2011

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case report

Fig. 1. (A) Anterior-posterior radiograph of the left shoulder. Plain X-ray of the left shoulder is showing destruction of the left humeral head with
fragmentation. (B) A coronal T2 weighted MR image of the left shoulder showing erosion, destruction and dislocation of the humeral head

Fig. 2. Sagittal, T2-weighted images (A) of cervical and (B) of cervico-thoracic spine presenting syrinx from C1 to Th5 level with Arnold-Chiari
type I malformation. Ethics statement: The patient gave oral consent for publication of his data in medial literature. The patient was fully
informed that his medical data would be published only in scientific medical journal

der showed destruction and dislocation of the humeral

head and atrophy of the muscles in the left shoulder
(Fig. 1).
The patient underwent surgery; a sub-occipital craniotomy was performed with a laminectomy of the CI to CIII
vertebrae and reconstruction of dura mater with her pericranium graft. Our patient recovered successfully after
surgery, and we recommended arthrodesis for her left
shoulder. She did not show any progression of syringomyelia in follow-up MRs within the last five years, nor had
other joints of the upper extremities shown any sign of
neuropathic arthropathy.

Discussion
Lesions of the upper and lower motor neurons can lead to
arthropathy. Diseases like multiple sclerosis, syringomywkw 1516/2011 Springer-Verlag

elia, tabes dorsalis, and leprosy can cause central sensory

lesions, and diseases such as diabetes mellitus, alcoholism, infections, pernicious anemia, and amyloidosis can
cause peripheral sensory lesions. All these diseases can
cause neuropathic arthropathy by causing sensory impairment [1, 2]. Neuropathic arthropathy commonly affects
weight-bearing joints such as the ankle, knee, hip, and in
patients with Chiari type 1 and syringomyelia, the shoulder and elbow are the most commonly affected joints
[13].
Syringomyelia is a chronic and progressive disease of
the spinal cord, characterized by longitudinal cavitations
of the spinal cord containing cerebrospinal fluid which involves the cervical and cervicothoracic regions [3]. It develops in approximately 7585% of patients with a Chiari
type I malformation [4]. The clinical presentation of syringomyelia depends on the extent and localization of the
Charcot shoulder caused by Chiari type I malformation

513

case report

syrinx. The syrinx cavity disrupts the adjacent gray and

white matter, which consequently causes destruction of
pain and temperature sensory fibers because they cross
the midline. This is the reason that loss of pain and temperature sensation in upper extremities is the first sign of
disease in such patients [5]. The loss of pain and temperature sensation in her left arm was also the first sign of disease in our patient.
There are two theories that try to explain the pathogenesis of neuropathic arthropathy in extremities. According
to the French theory, the destruction of joints is due to
damage of the central nervous system that controls joint
and bone nutrition, and according to the German theory,
the destruction of joints is due to the accumulation of subclinical joint trauma that is unnoticed because of a lack of
sensation in such joints [6]. Bower and Allmand showed
that even a bed-ridden patient can develop neuropathic
arthropathy which excludes a mechanical basis of this disease alone. Now it is believed that both theories play a role
in the pathophysiology of this rare disease. It is presumed
that a neurovascular mechanism has an initial role in the
pathogenesis of neuropathic arthropathy, and the neurotraumatic theory has a compounding effect on the diseases progression [6].
The radiological appearance of a diseased joint in the
advanced stages of disease is characterized by debris, destruction, dislocation, sclerosis, and disorganization of the
joint [7]. Radiographic findings of hypertrophy or atrophy
of the joint are believed to be two natural stages of this disease [4, 8]. Our patient experienced destruction and dislocation of the shoulder, but other joints in the left upper
extremities were unaffected.
In most cases, the treatment is conservative. It is necessary to treat the underlying disease and decrease the
progression of joint deformity [9]. Immobilizing the joint is
indispensable in the treatment of NA, combined with
splinting and joint aspiration for reducing the joints
ligamentous laxity [9]. Nonsteroidal anti-inflammatory
(NSAID) drugs may control the inflammation of the swollen synovium [1, 10]. Our patient also was treated with
NSAID drugs, but refused immobilization or splinting of
the shoulder.
More aggressive modalities of treatment such as arthrodesis have been tried, but the results were confounding.
Arthroplasty is strictly contraindicated in such patients because of bone weakness and because the disease has a potential to be progressive [2, 7].
Neuropathic arthropathy due to syringomyelia caused
by Chiari type I has complex findings, and slow disease
progression usually is diagnosed in late stages such as in
our case.
Our case is interesting because patient did not show
disease progression six years after surgery in other joints of
the left upper extremities and right upper extremities. In a
case of NA, it is necessary to make image examinations of
the spinal cord due to lesions of cervical spinal cord which

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Charcot shoulder caused by Chiari type I malformation

can cause NA. There are several cases in current literature

which describe NA of the shoulder and other joints of the
upper extremities combined with Chiari type I and syringomyelia, but this remains a rare disease which in many
cases is still diagnosed late, but properly treated [2, 5,
7, 10].

Conclusion
Nevertheless, neuropathic arthropathy is a slow progressing disease, and it still remains undetected until it reaches
late phases of progression. It is necessary to keep in mind
that many diseases can cause neuropathic arthropathy,
and that prompt diagnosis and treatment of the primary
disease can prevent neuropathic arthropathy. Neuropathic
arthropathy should be considered in cases where extreme
destruction of the affected joint is detected without complaints of pain from the patient, and neurological underlying problem should be investigated to avoid disease
progression.

Disclaimer
None.
Conflict of interest
None of authors, their immediate family, and any research
foundation with which they are affiliated did not receive
any financial payments or other benefits from any commercial entity related to the subject of this article.

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