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193
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_62
6 2
Idiopathic Retroperitoneal Fibrosis
62.1 Definition
Retroperitoneal fi brosis is a pathological condition, in which fi brotic tissue is
formed in the retroperitoneal space leading to compression of various organs in that
space. In most cases there is no detectable cause and is therefore termed idiopathic
retroperitoneal fi brosis (IRPF).
62.2 Incidence
IRPF is a rare clinical entity. Spinal cord compression due to IRPF is still rarer, very
few cases have been reported (Schoen E et al. 1975 ; De Sá et al. 1990 ); and Dorado-
bouix et al. 2004 ., which is abstracted here.)
62.3 Etiology
In most cases the etiology is unknown; however, some causative factors are proposed
especially when the disease is multifocal and is called multifocal fi brosclerosis:
• Ingestion of certain drugs e.g. methysegride, hydralazine
– Vaso-active drugs: which act on the wall of the aorta and other arteries
• Collagen disease
• Chronic infl ammatory disease: tuberculosis, histoplasmosis
• Neoplastic: reticulum cell sarcoma, Hodgkin’s disease
• Lupus erythematosus, periarteritis nodosa, scleroderma, autoimmune disease
amongst the multiple theories of the disease.
Abstracted from Dorado-Bouix et al. ( 2004 )
194
62.4 Pathogy
The disease involves the retroperitoneal space, but it may be multifocal, involving
the pelvis, the mediastinum, and separate organs including the gall bladder, bile
ducts, liver, thyroid, testis, cranial artery, orbit etc. Pathologically, there are mutlifoci of fi brosis without anatomical continuity. Sometimes it forms masses of fi brosis. Histiologically, it consists of fi brous tissue, adipose tissue with lymphocytes and
plasma cell infi ltration. The retroperitoneal mass may spread to the spinal canal
through the intervertebral foramena or intervertebral space to the epidural area and
cause cord compression aggravated by increased vascular retention and venous congestion. The authors also call it mediastinitus fi bromatosa and periaortitis cronica.
62.5 Clinical Picture
IRPF may be symptomless, symptoms of retroperitoneal organ compression e.g. the
ureters, the aorta or its branches, the venous system may be manifested clinically.
Compression myelopathy may be manifested by pain in the back or in the lower
extremities, low grade fever, weakness of the limbs, urinary dysfunction, inability to
walk, paraparesis, sensory changes. There may be associated signs of hydronephrosis and vascular compromise.
62.6 Diagnosis
Diagnosis based on:
1. Clinical picture
2. CT Scan and myelography show epidural mass with extension into the interver-
tebral foramena.
3. MRI shows deformity or displacement of the spinal cord or even partial atrophy.
The mass is enhanced by gadolinium infusion.
4. Erythrocyte sedimentation rate (ESR) somewhat elevated
5. CSF increased protein level
62.7 Management
Surgical treatment according to De Sá et al. a laminectomy and removal of the mass
compressing the cord extramedullary.
Conservative treatment according to Dorado-Bouix et al:
• Use of corticosteroids
• Chemotherapy cyclophosphamide, azathioprine etc. in cases resistant to steroids.
• Hormonal therapy by tamoxifen
They claim good response to medical therapy.
62 Idiopathic Retroperitoneal Fibrosis
195
Updates
Abstracted by from Bell and Thomson (2012)
The authors report a case of retroperitoneal fi brosis causing spinal claudication.
The case is a 47-year-old man complaining of claudication pains in both lower
limbs after walking about 200 yards. The pain was aching in nature and more present in his left leg. The pain was worse when walking up an incline and was continued when walking was recommenced. The claudication distance was 50 yards.
The patient’s pain ceased when walking was stopped and when he bends forward
for a couple of minutes. The pain starts in the thighs, radiates down to the ankles and
up to the buttocks and to the groins. There was no rest pain, no sphincter trouble and
no sensory disturbance.
Medicine routinely taken were:
Ramipril 10 mg P.O. qd (for hypertension)
Sodium Valporoate: 800 mg bid (for epilepsy)
Co-Codamol: 30/500 mg up to eight times a day (analgesic). He also had a probable
history of Perthe’s disease in the left hip.
Physical exam showed marked varicose veins of both legs extending to the anterior abdominal wall. A left sided varicocele was found. The peripheral arterial systems were normal. There were no neurological defi cits.
Ultrasonic exam showed marked refl uxes in the peripheral veins bilaterally. The
arterial tree was not sclerotic. A left varicocele was detected.
MRI of the lumbar spine showed minimal disc bulges at L3-L4 and L4-L5 without
nerve compression. There was epidural spinal varicosity, and dilatation of subcutaneous veins in the lumbar area, mild hydronephrosis, and possible retroperitoneal mass.
CT scan of the abdomen showed retroperitoneal fi brosis involving both ureters
causing right hydronephrosis. The left kidney was atrophic. The inferior vena cava
(IVC) was involved in the fi brosis. The patient was referred to vascular surgery for
decompression of the IVC and to urology for ureteric stent for hydronephrosis.
Reference
Bell S, Thomson S. Retroperitoneal fi brosis causing spinal claudication. Br J
Neurosurg. 2012;26(4):564–5.
References
De sá J, Pimentel J, Carvalho M, Evangelista P, Martins P. Spinal cord compression secondary to
idiopathic retroperitoneal fi brosis. Neurosurgery. 1990;26(4):678–81.
Dorado-bouix L, Millán-torné M, Capellades-font J, Gomà-gállego M, Olivé A. Spinal cord com-
pression: an unusual clinical manifestation of retroperitoneal fi brosis. Rev Neurol. 2004;
39(12):1143–5.
Schoen E, Creissard P, Auquier L. [Cauda equina syndrome during retroperitoneal fi brosis: discov-
ery of another coincidental etiology]. Ann Med Interne (Paris). 1975;126(4):297–302.
References
197
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_63
6 3
Isolated Spinal Neuro-Behçet Disease
63.1 Definition
Behçet disease (BD) was fi rst described by a Turkish dermatologist Hulnsi Behçet
in 1937. BD is a chronic relapsing multisystem disease. It commonly affects young
adults an can invade the skin, mucous membranes, eyes, joints, vascular system,
lungs, gastrointestinal tract, and the central nervous system (CNS). The focus of this
paper will be on neuro-Behçet disease (NBD) with special description on involvement of the spinal cord.
63.2 Incidence
Prevalence, the disease is most common in the countries around the Mediterranean
Sea, the Middle East, and the Middle Easter Asia, particularly in Japan. In Turkey
the prevalence is higher in rural areas 37/1,000,000 in the United Kingdom
4/100,000. In hospital population, in Istanbul the prevalence is 5.3 %, in Casa Blanca
it is 16 %, Alexandria 25 %, Iraq 3.3 %; in the United States according to Calamia
there is a prevalence rate of 6.6 per 100,000. According to Abda BD affects the CNS
in about 23 % of the United States patients. The age range is 16–61 (Al-Araji et al.);
males are three times more like to get BD than females. NBD has a very variable
prevalence in different countries from 2.2–50 % depending on the interest in the
disease, the study group, the region, and the good of research. In Iraq’s study, it was
14.3 %. The duration of the disease varies from 0.4 to 26 years. Family history was
positive in 10 % of Iraq study.
Abstracted from S Cakirer ( 2003 ), Green and Mitchell ( 2000 )
198
63.3 Etiology
The etiopathogenesis of the disease is still unknown however there are some
theories:
• Viral: suggested when the disease was discovered in 1937, by Behçet. The
viruses suggested were and reviewed with great interest
• Bacterial: streptococci were suggested, the infl ammatory viral and bacterial the-
ories waned
• Antigenic cross-reactivity and auto-immunity: Kurhan-Yauwz et al. looked at
T-cell responses to synthetic peptide B27PD and the retinal S antigen patients
with and without eye disease. The synthetic peptide shares common antigenic
sequence with HLA-B molecules of the retinal S antigen. Patients with BD and
retinitis had signifi cant proliferative and cytokine response, suggesting that the
immune response self antigen may be pathogenic in BD.
• T-cells: T-cells were found in peripheral blood of patients with BD increased in
number and producing interferon-Y and tumor necrotizing factor alpha (TNF-α),
suggesting that TNF-α, interferon-Y, and Th1 cytokines are important in the
pathogenesis of BD.
• Cytokine Th 1: peripheral blood lymphocytes are considerably higher in periph-
eral blood of patients with BD. Conjoined with higher blood interleukin 12,
which is a positive correlation with the progress of BD.
• Vasculitis: especially phlebitis, not excluding larger vessels, cerebral venous
thrombosis is evidence.
• Cytokine activation: IL-1, IL-6, IL-12, SIL-2R, TNF, INF are all higher in BD
and are involved in immune system activation.
• Genetic: In the Mediterranean HLAB51 is signifi cantly higher in patients with
BD, in Turkey it was found in 84 % of patients.
63.4 Pathology
BD is a multisystem disease with will be briefl y presented, focusing more on the
spinal cord.
System review
General Description
The disease is episodic, characterized by recurrent oral and genital ulcerations,
with constitutional symptoms of malaise, fatigue, and loss of weight. The onset may
be acute, subacute, or chronic with relapses. It may be missed.
• Cutaneous lesions: erythema no dosum, pustular eruptions, pseudofolliculitis,
skin ulcer which resemble pus fi lled bumps, erythema no desum seen on the legs,
ankles or toes. Skin involvement from Turkey study occurs in 84 % (following
are from the same study).
• Arthritis or arthalgia: 56 %
63 Isolated Spinal Neuro-Behçet Disease
199
• Thromophlebitis: 33 %
• Arterial lesions: 3.5 %
• Pulmonary involvement: 7 %
• Gastrointestinal involvement: 3 %
• CNS involvement:
– Parachymal lesions at the mesencephalic function with the brain stem and
diencphelon: 46 %
– Pontobulbar lesions: 46 %
– Hypothalamis thalamic lesions: 23 %
– Basal ganglia: 12.8 %
– Telencphelon: 5 %
– Cerebellum: 3 %
– Cervical spinal cord: 3 %
– Non-parenchymal: increased intercranial tension due to venous thrombosis
(aseptic meningitis is another manifestation)
– Peripheral nerve involvement: optic nerve, 8th nerve
• Spinal cord BD: 5 cervical cases have been reported (Green et al.), a sixth case
added by Kakirer. In autopsies up to 28 %. The thoracic cord is the most involved.
Microscopically ther are multifocal necrotizing areas marked infl ammatory cell
reaction. Necrosis may affect both gray and white matter probably due to
vasculitis.
63.5 Clinical Picture
• The association of cutaneous, oral, genital, and ocular manifestations of BD is
characteristic
• Other system involvement
• Spinal cord pathology manifested by neurological defi cits, both motor and sensory. This may be masked by brain stem involvement. There may be bladder
dysfunction due to direct involvement and other CNS involvement, either cerebellar and/or cerebral.
63.6 Diagnosis
Diagnosis is based on:
1. History: suspicion in endemic areas
2. Physical exam: associated lesions in different systems, including EENT exam,
genitalia, etc.
3. Laboratory tests: cerebral spinal fl uid (CSF) will show elevated proteins and
pleocytosis, lymphocytes especially in the acute phase. Pressure may be
increased. CBC increased lymphocytes, ESR elevated, evoked potential
studies
63.6 Diagnosis
200
4. Electro-diagnostic tests: there is no diffuse mater axonal loss (for more details
see Marciniak and Rosenfeld)
5. MRI of spinal cord and CT scan: usually negative. MRI should include the
brain stem, as it may show some bulbar signs
6. Criteria of the international study group 2 of the following
• Recurrent oral ulcerations
• Recurrent genital ulcerations
• Eye lesions
• Skin lesions
• Positive pathergy test
7. GI endoscopy for colon ulcerations
8. Pathological test: skin needle punctures result in a bump in a couple of days, but
not always.
9. Immunological studies: Leukocyte antigen B51
10. A number of cytokines and makers of vascular disease are under investigation.
Neuro-Behçet disease needs to be differentiated from Ruter’s disease, Crohn’s dis-
ease, vasculitis, multiple sclerosis (MS), systemic lupus erythematosus (SLE), human
immunodefi ciency virus (HIV), syphilis, and other neurological diseases, especially
of the brain.
63.7 Management
• Ophthalmous lesions treated with topical or intralesional cortisone, dapsone is
another choice
• Thalidomide in male patients suppresses mucocutanieous lesions
• Calcium in more serious cases
• Calcium plus penicillin of use in mucocutaneous lesions and arthritis
• Azothroppin for young male patients with eye disease
• Interferon is useful in eye lesions
• Corticosteroids for acute cases, but it does not suppress CNS lesions and does
not prevent blindness from posterior ureitits or retinal disease.
• Immunosupression using cyclophosphomide or chlorambucil for uncontrolled
eye disease, CNS disease, large vessel vasulitis, and recurrent deep vein thrombosis (DVT). Chlorambucil leads to male infertility and possibly malignancy, but
more useful to CNS lesions
• Heparin and cortisone are used for cerebral venous thrombosis
Treatment of the spinal cord
Moskau S et al. ( 2003 ) treated their patient with IV methylprednisolone for
6 weeks followed by oral steroid, which gave improvement to SCI lesion. In the case
of Cakirer, paque lesions on MRI disappeared within 8 weeks with IV steroids, exact
dosage and treatment not detailed. Green et al. treated a similar case with IV methylpredinsolone 1 gm/day × 3 days followed by prednisolone p.o. in decreasing doses.
Patient’s clinical condition resolved and complete resolution was achieved.
63 Isolated Spinal Neuro-Behçet Disease
201
References
Cakirer S. Isolated spinal neurobehçet disease. MR imaging fi ndings. Acta Radiol.
2003;44(5):558–60.
Green AL, Mitchell PJ. Spinal cord Neurobehçet’s disease detected on magnetic resonance imag-
ing. Australas Radiol. 2000;44(2):201–3.
Moskau S, Urbach H, Hartmann A, Schmidt S. Multifocal myelitis in Behçet’s disease. Neurology.
2003;60(3):517.
References
203
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_64
6 4
Subacute Progressive Ascending Myelopathy (SPAM)
64.1 Definition
This is a condition of degeneration of the neurological defi cit after spinal cord injury
due to extension of the cord damage cephalad through four or more segments developing within 4–86 days, with median of 13 days.
64.2 Incidence
According to Hans Frankel this occurs after SCI in 1.5 % of all cases. Planner et al.
reported 11 cases over 15 years from Stoke Mandeville Spinal Center in the UK, which
admits 175 patients per year. 80 % are traumatic cord injury; age 18–50 (mean 35);
males 9:11; cervical injuries 9:11. The injuries mostly due to motor vehicle accidents.
64.3 Etiology
The exact cause is not clear. Frankel hypothesized that subacute progressive ascending myelopathy (SPAM) is due to thrombosis or infl ammatory changes at the injury
site. Planner also exposed the possible etiological factors:
• In the cases stabilized by surgery:
• Arterial occlusion including fi brocartilaginous embolism
• Prolonged hypotension
• Infection
• Altered apoptotic cascade
Abstracted from Planner et al. ( 2008 )
204
• Unregulated immune response
• Venous thrombosis
• Disturbance of the CSF producing a mass effect
• Possible intraspinal hematoma
• Possibly viral infection since the picture is similar to myelopathy following herpes simplex, Epstein Barr, or Coxsackie’s viral infections. In SPAM some
patients had fever and some lymphocytic rise in the CSF
64.4 Pathology
There is myelopathy cephalad to the cord injury invading from 4 to 11 segments
with a mean of 6.2. There is expansion of the cord and central cord involvement.
Planner et al. stated that pathologically no vascular occlusion is detected since ischemia is irreversible, while these lesions mostly improve within months. There is
some degree of myelomalacia and there is no evidence of syrinx. In cervical cases
the ascent may involve the brain stem, leading to death.
64.5 Clinical Picture
Some cases are subclinical and are diagnosed only by MRI. However, clinical neurological degeneration is usually evident. A rise of neurological defi cit at least two
segments cephalad is detected. There may be a complaint of shoulder pains and
sensory changes and the patient of the 11 cases recorded had dysphagia and another
patient had respiratory diffi culties due to brain stem involvement, which ended
fatally (6 %). The symptoms may remain subclinical but with MRI evidence. In few
cases a history of fever may be obtained.
64.6 Diagnosis
Diagnosis is based on MRI: Abnormal signals within the cord were demonstrated in at
least fi ve segmental levels cephalad to the original injury. The maximal ascension
found was 11 segments. There are high signals on T2-weighted images and minimal
changes on T1-weighted images. There is cord expansion with minimal changes in the
cord rim. In one case there was an extension into the medulla oblongata. Recovery to
the original status was incomplete. Recovery was limited to a level cephalad to but not
to the original level. There was no evidence of etiological factors so far (see etiology).
64.7 Management
No specifi c therapy. In cervical regions watch for respiratory diffi culty and possible
ventilator need. According to Planner et al. all cases recovered except the one death
due to respiratory failure.
64 Subacute Progressive Ascending Myelopathy (SPAM)