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241
In immunocompetent patients there is reversal of the neurological defi cits totally
or subtotally.
In immunocompromised patients, chances are small with these two measures
added to them in a few cases was plasma exchange.
Updates
Abstracted from Karunarathne et al. (2012)
Case Report
The authors only found only ten published cases of CMV associated transverse
myelitis have been reported. They report a patient with clinical myelitis followed by
previously unreported fi nding of cytomegalovirus deoxyribonucleic acid in CSF. A
40-year-old immunocompetent male presented with acute onset progressive bilateral lower limb weakness. His spinal MRI, CSF analysis, and clinical picture were
compatible with transverse myelitis. Polymerase chain reaction of the CSF for cytomegalovirus was positive. He was treated with IV ganciclovir with partial clinical
response.
Reference
Karunarathne S, Govindapala D, Udayakumara Y, Fernando H. Cytomegalovirus
associated transverse myelitis in an immunocompetent host with DNA detection in
cerebrospinal fl uid; case report. BMC Res Notes. 2012;5:364–8.
References
Fux CA, Pfi ster S, Nohl F, Zimmerli S. Cytomegalovirus-associated acute transverse myelitis in
immunocompetent adults. Clin Microbiol Infect. 2003;9(12):1187–90.
Kerr DA, Ayetey H. Immunopathogenesis of acute transverse myelitis. Curr Opin Neurol.
2002;15(3):339–47.
References
243
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_74
7 4
Degos Disease (DD)
74.1 Definition
Degos disease (DD) is a condition of angiitis of the small vessels leading to occlusive vasculitis initially involving the skin. It may, in its simple form, be limited to
the skin or may be a lethal multi-organ systemic variant. The disease has multiple
nomenclatures of these a few are mentioned: Kiohliner-Degos disease, DegosDelert- Tricot syndrome, malignant atrophic papulosis, arteriolar cutaneogastrointestinal thrombosis, etc. Systemic DD is usually fatal in 1–2 years
(Scheinfeld et al. 2005 )
74.2 Incidence
In the United States DD is rare; about 150 cases have been reported worldwide
(Scheinfeld et al. 2005 ). Matsura et al., have recently reported a case of spinal cord
and optic nerve involvement by DD.
74.3 Etiology
According to Schienfi eld et al. ( 2005 ),
In 2003, Ball et al. 2003 , thought that DD might be a variant of lupus erythemato-
sus. In 2005, Scheinfeld et al. ( 2005 ), adduced strong evidence that DD is a dis-
tinct condition, since it starts with photosensitivity, does not respond to steroids,
and its systemic form is fatal in 1–2 years. Some authors think that DD is due to
a defect in the vascular endothelium, resulting in thrombosis, but it has not been
Abstracted from Matsuura et al. ( 2006 ).
244
proven yet. Vasculitis is also remote since there are no pathological infl amma-
tory signs in the vessel wall. Recent suggested theories:
• immunity disturbance
• viral infection
• abnormality in blood clotting
Familial autosomal dominance has not been proven.
74.4 Pathology
• Cutaneous lesions: These are raised papules with umbilicated porcelain centers
and a surrounding erythematous rim. Microscopically, there are degenerated
wedge-shaped collagens. There is interface dermatitis and there is squamatiza-
tion of the dermoepidermal, function, melanin incontinence and epidermal atro-
phy. There are areas of dermal papillary sclerosis. See more details in Scheinfeld
et al. ( 2005 ).
• Mesenteric vessels: Thrombosis leads to infarction and perforation microaneu-
rysm have been encountered, gastrointestinal ulcerations and infarcted areas
have been detected.
• Kidney: There is thickening in the glomerular arterioles and capillary basement
membrane. Other organs may be affected as well
• Spinal cord: The lesions are mostly in the lateral and posterior columns showing
demyelination and axonal loss, the same in the nerve roots. Vascular thrombosis
and endothelial proliferation are noted. Similar changes are noticed in the brain
and the optic nerve was degenerated. Vascular thrombosis is a basic phenomenon.
In the case described involvement of the optic nerve preceded the spinal cord
changes. There was also central retinal artery thrombosis.
74.5 Clinical Picture
Initially, the disease starts by characteristic skin lesions: erythematous or red skin
papules, which heal leaving scars which are pathognomic with porcelain white atrophic center. The papules have a peripheral telangiectatic rim.
In system DD, the gastrointestinal tract is affected in 50 % of the cases. Infarction
of the bowel with intestinal perforation and peritonitis are most common and are
fatal complication. Other systems especially the central nervous system may be
involved with lesions in the brain and the spinal cord. Systemic DD appears weeks
or months or years after the manifestation of the cutaneous disease. In rare cases it
precedes the skin pathology.
74 Degos Disease (DD)
245
74.6 Diagnosis
Diagnosis is based on:
1. Physical exam: cutaneous lesions, skin lesions, papules, erythremia around a
porcelain like center
2. Neurological exam: the brain and spinal cord
3. Laboratory tests: Hemogram for anemia with intestinal bleeding, elevation of the
thrombi-antithrombin III complex, plasmin-α2-plasmin inhibitor complex, cyto-
toxic T-cell subset (CD8, CD11) illustrating the coagulation, fi brinolytic and
immunologic implications of DD include proteins, CSF.
4. MRI of spinal cord: Shows high intensity lesions with saw-teeth appearance.
There is also a high intensity signal with a moth-eaten appearance in the periph-
ery of the cord. Patchy lesions from the periphery to the center are characteristic
of DD of the cord. The longitudinal nerve fi bers appear severed by the lesions.
5. MRI of the brain: Shows multiple infarcts and areas of hemorrhage.
6. MR angiography: shows stenotic or occluded vessels
7. EGG and electrophysiological studies
8. Laboratory tests: Hemogram for anemia with intestinal bleeding, elevation of the
thrombin-antithrombin III complex, plasmin-α2-plasmin inhibitor complex,
cytotoxic T-cell subset (CD8, CD11) illustrating the coagulation, fi brinolytic and
immunologic implications of DD include high proteins in the CSF.
74.7 Management
• Medical (medications non-surgical)
• Corticosteroids, heparin, warfarin, clopidogrel, aspirin, dipyradimale, pentaxo-
phybline, IV immunoglobulin, dextran, ACTH, etc.
• Topical Applications
• Surgery as indicated for intestinal infarcts, perforation, or GI bleeding.
References
Ball E, Newburger A, Ackerman AB. Degos’ disease: a distinctive pattern of disease, chiefl y of
lupus erythematosus, and not a specifi c disease per se. Am J Dermatopathol.
2003;25(4):308–20.
Matsuura F, et al. Optic nerve and spinal cord manifestations of malignant atrophic papulosis
(Degos disease). J Neurol Neurosurg Psychiatry. 2006;77:260–2.
Scheinfeld N. Degos’ disease is probably a distinct entity: a review of clinical and laboratory evi-
dence. J Am Acad Dermatol. 2005;52(2):375–6.
References
247
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_75
7 5
Dengue Myelitis
75.1 Definition
Dengue fever (DF) and Dengue hemorrhagic fever (DHF) are caused by Dengue
virus (DV) serotypes 1–4. It is transmitted by mosquito (Aedes Aegypti) bites.
75.2 Incidence
Dengue fever is an important virus infection in tropical and subtropical countries. In
the United States, the disease is not endemic since the vector is not present. From
1977 to 2004, 3806 suspected cases were reported. Epidemic cases six times in the
southern Texas, northern Mexico, and Hawaii were detected. There was no mention
of myelitis. In South America especially in Brazil where the disease is endemic, the
CNS complications are mostly encephalitis, myelitis is very rare. A case presented
by Leão et al. is presented. Chanthamat and Sathirapanya reported a case of acute
transverse myelitis associated with dengue fever. According to the authors there
have only been four previously reported cases of transverse myelitis associated with
dengue infection.
75.3 Etiology
The pathogenesis of the CNS disease is not clear and still little understood.
Frequently, dengue infection has been associated with encephalopathy followed by
shock and hypoxemia of the CNS (Leão et al.). Some authors including Chimelli
et al., Cordeiro, Cunha et al., and Miagostovich et al., and Patey et al. have shown
Abstracted from Leão et al. ( 2002 ) and Chanthamat and Sathirapanya ( 2010 ).
248
the criteria for breakdown of blood–brain barrier of the CNS. Lum et al., have supported the encephalopathy to be due to the dengue viruses. Ramos et al., have
detected the antigen by immunohistochemistry, and DEN-4 RNA in neurons, astrocytes, microglia, and endothelial cells. For more details see Seet et al.
75.4 Pathology
Myelitis is caused by dengue viruses 1–4. Severe gray matter myelitis may occur in
DV type 1. The myelitis may be exclusive as to be detected by MRI from the cervical region to the cauda. Sometimes myelitis appears after the infection subsides.
The picture is involvement of the gray matter like poliomyelitis, but there is sensory
involvement as well. Partial resolution may occur in DV types 2 or 3.
75.5 Clinical Picture
The condition starts by fever, muscular rash, or capillary hemorrhages. There is fl accid paralysis, paraparesis, or tetraparesis, pin-prick sensations is decreased.
Sphincter dysfunction usually retention of urine; in some cases there is encephalopathy as well. High spinal involvement results in respiratory distress requiring
ventilator assistance.
75.6 Diagnosis
Diagnosis is based on the following criteria:
1. History of patient in endemic area
2. Physical exam shows rash, fever or dengue hemorrhagic fever
3. Neurological exam: sensory changes, onset of fl accid paralysis
4. Laboratory tests
(a) blood count shows thrombocytopenia,
(b) Anti-DV IgM to IgG in the serum and CSF
(c) Plaque neutralization assays for antibodies for DV types
(d) Laboratory tests for other viruses
(e) Cultures for bacteria, mycobacteria, and fungi
5. MRI: high intensity signals limited to the gray matter region in any region from
the cervical to the lumbar on T2 weighted images
6. Electrophysiology: peroneal compound muscle action potentials (CMAP) were
absent in the case reported.
75 Dengue Myelitis
249
75.7 Management
Patient in Kunishige et al., case report was treated with:
• IV methylpredinsolone
• IV immunoglobulin
• Non-specifi c antibiotics
• Plasmapheresis
In the authors’ case a tetraplegic, the upper limbs improved but remained paraplegic. After treatment the laboratory tests changed, the elevated DV antibody index
came down IgM/IgG in both serum and the CSF.
References
Chanthamat N, Sathirapanya P. Acute transverse myelitis associated with dengue viral infection.
J Spinal Cord Med. 2010;33(4):425–7.
Leão RN, Oikawa T, Rosa ES, et al. Isolation of dengue 2 virus from a patient with central nervous
system involvement (transverse myelitis). Rev Soc Bras Med Trop. 2002;35(4):401–4.
References
251
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_76
7 6
Dental Extraction
76.1 Definition
This is a paraplegia developing as a result of metastatic epidural abscess after dental
extraction and is due to oral bacteria from poorly hygienic teeth.
76.2 Incidence
One case reported by Larkin and Scott.
76.3 Etiology
The bacteria found in the epidural abscess are:
Streptococcus salivarius
Mutans
Mitis
No prophylactic or post extraction antibiotics were given.
76.4 Pathology
The most common source of pyogenic infection of the spine is metastatic bacteriemia source oropharyngeal infections are recognized as primary sources for metastatic infection. The infection settles in the vertebral cancellous bone and if
Abstracted from Larkin and Scott ( 1994 )
252
uncontrolled it spreads outwards and destroys the intervertebral disc. Epidural
abscess forms and may compress the spinal cord.
76.5 Clinical Picture
The recorded case was 65 year old male who had dental extraction, developed back
pain, which was treated by analgesic and bed rest. Five weeks post-extraction he
developed paraplegia, spastic type, and with sensory level at T8.
76.6 Diagnosis
Diagnosis is based on the following criteria:
1. Neurological exam
2. Radiography of the spine showing degenerative changes at T8-T9
3. Myelogram showed complete block at T8-T9
76.7 Management
• IV antibiotics
• Surgery I: exploratory through a left costo-transversectomy, degenerated disc
material was evacuated as well as the questionable bone.
• Surgery II: 4 days later through a laminectomy decompression and removal of
septic granuloma.
There was no neurological improvement perhaps due to delay.
Reference
Larkin EB, Scott SD. Metastatic paraspinal abscess and paraplegia secondary to dental extraction.
Br Dent J. 1994;177(9):340–2.
76 Dental Extraction
253
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_77
7 7
Holospinal Epidural Abscess
77.1 Definition
This is a relatively rare condition in which epidural abscess extend form the cervical
to the lumbar region.
77.2 Incidence
The authors reviewed the literature and found only 13 cases reported and added two
of their own.
77.3 Etiology
Predisposing factors:
• Diabetes mellitus
• Low immunity, e.g. receiving chemotherapy
Predisposing causes:
• Psoas abscess
• Crohn’s disease
• Congenital sacral teratoma
• Osteomyelitis of the spine
• Idiopathic
Abstracted and reported by Lau et al. ( 2014 )