Clinical rheumatology, 1994, 13, N ° 2 309-311

Case Report
Ossification of the Posterior Longitudinal Ligament
in One of a Pair of Identical Twins Concordant for
Ankylosing Spondylitis
I. O L I V I E R I , N. P A P P O N E * , A. P A D U L A * * , C. R E N G O * , G . P . R U J U * * ,
A. P U C I N O * * * , D. T R I P P I * * , S. F E R R I , G. P A S E R O * *

Summary A pair of identical twins suffering from ankylosing spondylitis is

reported. One brother developed an earlier-onset disease and showed ossification
of the posterior longitudinal ligament and the flavum ligament in his cervical
spine.

Key words Posterior Longitudinal Ligament Ossification, Ankylosing

Spondylitis, Monozygotic Twins

INTRODUCTION Physical examination disclosed forward craning of the

Ossification of the posterior longitudinal ligament
(PLL) is a condition with an obscure etiopathogenesis
mainly reported in Japanese patients (1,2) and in sub-
jects with diffuse idiopathic skeletal hyperostosis (DISH)
(3). Only a few cases of patients with coexisting anky-
losing spondylitis (AS) and PLL ossification have been
reported (4-6). We have recently seen one more pa-
tient with AS showing PLL ossification without any signs
of DISH. He also showed ossification of the flavum lig-
ament, which has rarely been reported in AS (7). In-
terestingly, he is one of a pair of identical twins con-
cordant for AS.
CASE R E P O R T
The twins, born on 29 August 1963, were admitted
to the "Clinica del Lavoro" Foundation in April 1991.
Twin 1 related a history of inflammatory spinal pain
and a decreasing range of movement of the cervical and
lumbar spine, which began at the age of 16.
From the Rheumatic Disease Unit, S. Orsola-Malpighi Hospital, Bo-
logna *the "Clinica del Lavoro" Foundation, Institute of Care and
Scientific Research, Campolidel Monte Taburno (BN); ***the Rheu-
matologyUnit of the 2nd Medical School,Universityof Napoli, Napo-
li; **the Rheumatic Disease Unit of the Institute of Medical Pathol-
ogy 1 and the Institute of Radiology,Universityof Pisa, Pisa, Italy.
cervical spine, thoracic kyphosis, loss of lumbar lordo-
sis and severe restriction of chest expansion and both
cervical and lumbar spine in all directions. There was
tenderness over the right greater trocliaiater and mod-
erate limitation of movement of the right hip.
Laboratory evaluation showed erythrocyte sedimen-
tation rate (ESR) (Westergren) 36 mm/h., C-reactive
protein 4.8 mg/dL (normal <0.5), fibrinogen 4.8 mg/dL
(normal 2-4.5), alpha 2-globulin 11% (85 g/L total pro-
tein).
An anteroposterior view of the pelvis revealed fusion
of both sacroiliac joints, together with narrowing of the
right hip joint. Radiographs of the cervical, dorsal and
lumbar spine showed squaring of the vertebral bodies,
apophyseal joint sclerosis and ankylosis, marginal syn-
desmophytes and osteoporosis. In the cervical spine (Fig-
ure la), ossification of the PLL and the flavum liga-
ment was visible extending from C1 to C6.
Twin 2 showed no symptoms of seronegative spondy-
loarthropathy till the age of 18, when he complained of
inflammatory low back pain and stiffness. Over the fol-
lowing years, the s)~zaptoms extended to the dorsal and
cervical spine, and a gradual limitation of spine move-
ment developed.
Laboratory evaluation revealed ESR 48 mm/h., CRP
24 mg/dL, fibrinogen 4.5 mg/dL, alpha 2-globulin 12.5%
(6.6 g/L total protein).
An anteroposterior view of the pelvis showed grade
3 sacroiliitis on the right sacroiliac joint and grade 4 on
310 I. Olivieri, N. Pappone, A. Padula, et al.

Figs. la and lb: Lateral radiographic view of twins' cervical spines. (a) Twin 1 shows marginal syndesmophytes,vertebral body squaring,
apophysealjoint sclerosis and fusion, and posterior ligament ossification(white open arrows) separated from the posterior aspect of the ver-
tebral bodies by a radiotucent line (black arrowheads), and flavumligamentossification(white solid arrow), (b) Twin 2 shows only vertebral
body squaring, sclerosis of the apophysealjoint and one syndesmophyte.

the left one. Dorsal and lumbar spine radiographs re-
vealed apophyseal joint sclerosis and ankylosis, margin-
al syndesmophytes and vertebral body squaring. Cervi-
cal spine radiographs (Figure lb) showed only verte-
bral body squaring, apophyseal joint sclerosis and one
syndesmophyte.
The twins denied any history of dysuria, diarrhoea,
uveitis, conjunctivitis, psoriasis or cardiac symptoms.
There was no family history of seronegative spondy-
loarthropathy or other B27-associated syndromes. Their
H L A typing showed A1, A l l , B8, B27, Cw2, Cw7,
D R w l l , DRwl3, and DQw7(w3). They were identical
also for ABO and Rhesus red blood cell grouping. They
were given naproxen and sulphasalazine and were taught
the exercise regimen for AS which they have since been
following at home.
DISCUSSION
Nineteen isolated monozygotic twin pairs with AS have
been reported, 13 of which were concordant and 6 dis-
cordant (8-10). Our twin pair is concordant, but twin 1
developed an earlier onset and more severe disease. He
developed ossification of the PLL and the flavum lig-
ament. We intend to follow up these twins to see if
also twin 2 will develop a spine involvement similar to
that of twin 1, including PLL and flavum ligament ossi-
fication.

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Received: 8 June 1993.
Accepted: 31 August 1993
Correspondence to: Dr. I. OLIVIERI,
Servizio di Reumatologia, Ospedale S. Orsola-Malpighi, Via Mas-
sarenti 9, 40138 Bologna, Italy.