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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_6042_Библиотеки_им_академика_М_И_Перельмана.pdf
135
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_44
4 4
Acute Transverse Myelitis in an Infant After Dtap Vaccination
44.1 Definition
This is a condition of edema of the spinal cord developing after vaccination with
diphtheria-tetanus-acellular pertussis (DTaP) vaccination as evidenced by neurological exam and MRI imaging.
44.2 Incidence
The case report by Riel-Romero seems to be the only one developing acute transverse myelitis after DTap 17 days earlier, although the child had two previous vaccinations (DTaP) without reaction. Kulenkampff et al. described a case of an infant,
6-month-old, who developed fl accid quadraparesis with respiratory compromise 17
days after DTaP vaccination (1974). Other cases of acute transverse myelitis (ATM)
in childhood were reported due to hepatitis B vaccination (Fonseca et al. 2003 ;
Iniguez et al. 2000 ). A case of Japanese B encephalitis vaccine was followed by
transverse myelitis (Matsui et al. 2002 ). Whittle and Robertson reported a case of a
7-month-old infant who developed fl accid paraplegia after receiving DT inoculation
and oral polio vaccine ( 1977 ). Zanoni et al. in 2002 , reported a 15-month-old girl
who developed ATM 21 days after receiving measles-mumps-rubella vaccination;
similarly, Lim et al. 2004 with a 9-year-old girl who developed ATM 16 days post
measles and rubella vaccination.
Abstracted from Riel-Romero ( 2006 )
136
44.3 Etiology
The cause of developing ATM after DTaP vaccination is not quite clear:
• Riel-Romero's hypothesis is that the infant had acute respiratory infection 2
weeks before the onset of the myelitis, suggesting a viral agent. In an analysis of
33 patients of ATM post vaccination the author found, including adults, that 45 %
had a preceding infection, most of them were respiratory with 73 %, gastrointes-
tinal infection was 13 and 13 % had fl u-like symptoms (Jeffery et al. 1993 ).
• The offending agent triggers an autoimmune reaction to the myelin sheath of the
central nervous system (CNS), mainly to the spinal cord (Kerr and Ayetey 2002 ).
The risk of ATM as an autoimmune response may depend on genetically predis-
posed child.
• Myelitis may have developed as an incidental condition.
44.4 Pathology
There is no pathological data in these cases; however, in hepatitis B vaccination it
shows the infl ammatory reaction, edema, and demyelination (Sindern et al. 2001 ).
The disease seems to be self and time limited illness and is generally monophasic.
Improvement occurs in 2–12 weeks after maximal development, but the faster the
development the worse the prognosis is. Resolution occurs especially after steroid
therapy; however, a scar is formed and motor recovery is minimal.
44.5 Clinical Picture
(From Riel-Romero 2006 ) An infant 7-months-old was admitted for leg weakness
17 days after DTaP vaccination. He received two injections without problems. He
developed priapism and dribbling of urine. He had constipation previously, which
was resolved with fl eet enema. His past history was negative except for acute
upper respiratory infection before admission. Examination of his systems was
unremarkable. The neurological exam showed fl accid paraplegia. Noxious stimuli
of the legs gave triple fl exion response (no anesthesia). The lower limbs tendon
refl exes were +1.
44.6 Diagnosis
Diagnosis is based on:
1. Clinical picture
2. Laboratory tests
44 Acute Transverse Myelitis in an Infant After Dtap Vaccination
137
(a) Blood count shows leucocytosis 16,000 and poly 67 %
(b) CSF showed 2 RBC/uL and 7 WBC/uL, 4 lyphocytes, and 3 monocytes
(c) Myelin basic protein was increased at 4.9 mg/dL (normal: 0–2.3)
(d) Other tests were within normal including IgG, and tests for viruses and bac-
teria were negative.
3. MRI: brain was negative. Spine showed diffused edema of the spinal cord with
increased T2 signal within the spinal cord from C3 to T6, dorsal and ventral
veins were normal and no evidence of AVM. After gadolinium injection there
was a faint and patchy encroachment of the spinal cord.
The diagnosis was, from the clinical and imaging pictures, ATM 17 days after
DTaP vaccination.
44.7 Management
Riel-Romero gave the following treatment:
• IV methylpredinsolone: 1.5 mgm/kg/day for 7 days followed by
• Oral predinsolone tapering at 1 mg/kg/day for 2 weeks
• Acyclovir pending on the result of CSF, HSV-DNA PCR.
• Bowel and bladder care
• Rehabilitation program
Within a week priapism resolved and the lower extremities gained tone and there
was a clonus. At three weeks the constipation resolved and urine stream was established but signs of pyramidal involvement persisted. Three months later MRI
showed resolution of the cord edema and its signals but showed reduction of the
cord caliber. Ten months after the onset spastic paraplegia persisted.
References
Fonseca LF, Noce TR, Teixeira ML, Teixeira AL, Lana-peixoto MA. Early-onset acute transverse
myelitis following hepatitis B vaccination and respiratory infection: case report. Arq
Neuropsiquiatr. 2003;61(2A):265–8.
Iñiguez C, Mauri JA, Larrodé P, López del val J, Jericó I, Morales F. [Acute transverse myelitis
secondary to hepatitis B vaccination]. Rev Neurol. 2000;31(5):430–2.
Jeffery DR, Mandler RN, Davis LE. Transverse myelitis. Retrospective analysis of 33 cases, with
differentiation of cases associated with multiple sclerosis and parainfectious events. Arch
Neurol. 1993;50(5):532–5.
Kerr DA, Ayetey H. Immunopathogenesis of acute transverse myelitis. Curr Opin Neurol.
2002a;15(3):339–47.
Lim S, Park SM, Choi HS, et al. Transverse myelitis after measles and rubella vaccination. J
Paediatr Child Health. 2004;40(9–10):583–4.
Matsui M, Kawano H, Matsukura M, Otani Y, Miike T. Acute transverse myelitis after Japanese B
encephalitis vaccination in a 4-year-old girl. Brain Dev. 2002;24(3):187–9.
References
138
Riel-Romero RM. Acute transverse myelitis in a 7-month-old boy after diphtheria-tetanus-
pertussis immunization. Spinal Cord. 2006;44(11):688–91. Epub 29 Nov 2005.
Sindern E, Schröder JM, Krismann M, Malin JP. Infl ammatory polyradiculoneuropathy with
spinal cord involvement and lethal [correction of letal] outcome after hepatitis B vaccination.
J Neurol Sci. 2001;186(1–2):81–5.
Whittle E, Robertson NR. Transverse myelitis after diphtheria, tetanus, and polio immunisation.
Br Med J. 1977;1(6074):1450.
Zanoni G, Nguyen TM, Destefani E, Masala L, Nardelli E, Tridente G. Transverse myelitis after
vaccination. Eur J Neurol. 2002;9(6):696–7.
44 Acute Transverse Myelitis in an Infant After Dtap Vaccination
139
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_45
4 5
Paraplegia After Endovascular Repair of Abdominal Aortic Aneurysm
45.1 Definition
This is an ischemic condition of the spinal cord resulting in paraplegia 3 weeks after
endovascular repair of an abdominal aortic aneurysm (AAA)
45.2 Incidence
This is the fi rst case after endovascular repair of AAA. After open surgical technique the incidence of spinal cord ischemia is 0.25 %. According to the Eurostar
database, the incidence of cord ischemia is 0.21 %. Smaller arterial endovascular
procedures do not lead to spinal cord ischemia.
45.3 Etiology
Spinal cord ischemia after AAA repair is due to the following:
• interruption of the cord circulation due to clamping
• atheromatous embolization
• hypoperfusion due to hypotension
• interruption of the artery of Adamkiewicz
• reperfusion edema leading to cord hypoperfusion and ischemia
CSF drainage can increase spinal cord perfusion, acting against the upper
mechanisms.
Abstracted from Godstein et al. ( 2010 )
140
45.4 Pathology
There is ischemia, edema of the cord, leading to demyelination and apoptosis,
resulting in permanent myelopathy.
45.5 Clinical Picture
The case presented is a 75-year-old man: diabetic, chronic renal failure, coronary
heart disease, aortic valve replacement, and an asymptomatic 5.5 cm infrarenal
AAA, and concomitant 3.5 cm right common iliac artery aneurysm
45.6 Diagnosis
CT scan showed a fusiform aneurysm 3 cm below the right renal artery.
45.7 Management
Endovascular repair by aorticuniiliac (AUI) graft and femoral-femoral bypass. The
patient developed several pressure ulcers postoperatively, which got septic. Twentyone days post-op he was found paraplegic. MRI showed diffuse edema of the cord,
due to ischemia. He needed feeding gastrotomy tube and left iliac colostomy and
tracheostomy. There was no further report.
Reference
Goldstein LJ, Rezayat C, Shrikhande GV, Bush HL. Delayed permanent paraplegia after endovas-
cular repair of abdominal aortic aneurysm. J Vasc Surg. 2010;51(3):725–8.
45 Paraplegia After Endovascular Repair of Abdominal Aortic Aneurysm
141
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_46
4 6
Spinal Cord Infarction After Endoscopic Esophageal Varix Ligation
46.1 Definition
According to Stedman’s medical dictionary “an infarct is an onset of necrosis,
resulting from a sudden insuffi ciency of arterial or venous blood supply.”
46.2 Incidence
These cases are rare. Tofuku et al. reported a case in 2007. Debette et al. reported a
case in 2003. In 1996, Heller et al. reported on case. The number of cases is very
small.
46.3 Etiology
The causes of spinal cord venous infarction are:
• congenital arteriovenous malformation (AVM)
• epidural hematoma
• tumors
• iatrogenic: ligation of esophageal varices; sclerotherapy of esophageal varices
• decompression sickness
Because of the rarity of cases the etiology is not quite clear.
Abstracted from Tofuku et al. ( 2008 )
142
46.4 Pathology
The ligation or injection of the esophageal varices leads to hemodynamic changes
in venous circulation of the cord resulting in congestion and thrombosis, which
ultimately leads to tissue ischemia and necrosis.
46.5 Clinical Picture
Following varices interruption by 1–3 days, a sense of numbness of the extremities,
followed by weakness and paralysis with sphincter dysfunction.
46.6 Diagnosis
Neurological exam will detect motor and sensory loss and usually hyporefl exia.
MRI with and without gadolinium. Lab tests include INR, platelet count, liver
function tests.
46.7 Management
Conservative treatment of paraplegia. Tofuku et al. used hyperbaric oxygen therapy
(HBO2) at 2 ATA wit 15 min descent and 15 min ascent for 1 h daily for 20 days,
with good outcome. Experience with HBO2, treatment in decompression sickness
DCS justifi ed its use in the particular case.
Reference
Tofuku K, Koga H, Yamamoto T, Yone K, Komiya S. Spinal cord infarction following endoscopic
variceal ligation. Spinal Cord. 2008;46(3):241–2.
46 Spinal Cord Infarction After Endoscopic Esophageal Varix Ligation
143
© Springer International Publishing Switzerland 2016
I.M. Eltorai, Rare Diseases and Syndromes of the Spinal Cord,
DOI 10.1007/978-3-319-45147-3_47
4 7
Subacute Combined Degeneration Provoked by Nitrous Oxide Anethesia Patients with Cobalamin Deficiency
47.1 Definition
Vitamin B 12 defi ciency leads to neuropathological changes in humans, principally
subacute combined degeneration (SCD) of the spinal cord and polyneuropathy. Less
commonly it causes encephalopathy leading to degeneration and optic neuropathy.
Myelopathy may develop after nitrous oxide anesthesia in asymptomatic patients or
those with borderline vitamin B
12
defi ciency or overlooked cases. It can result from
chronic recreational use of N 2 O or industrial exposure. This presentation will focus
on myelopathy and SCD.
47.2 Incidence
SCD is a manifestation of vitamin B 12 defi ciency in one-third of the patients.
Following nitrous oxide anesthesia cases of SCD are rare. In our English literature
search we came across 11 reported cases, which means extreme rarity.
47.3 Etiology and Pathology
Kando et al. showed that nitrous oxide causes multiple defects in Cb1 metabolism.
N 2 O is a patent oxidant that disrupts the methonine synthetase reaction by causing
oxidation of Co ++ to Co
+++
, which blocks regeneration of the cobalamine coenzyme
methylcobalamin producing a condition simulating Cbl defi ciency, inhibits methylation of myelin phospholipids, and alters incorporation of fatty acids into myelin
sheaths. In patients with normal Cbl stores, a suffi cient quantity of unoxidized Cbl
Abstracted from Ahn and Brown 2005
144
may be available to maintain enzyme function; in patiens with borderline Cbl stores,
even short exsposures to N 2 O may be suffi enct to precipitate Cbl defi ciency syndrome. The mature cobalamine is excreted so that exposure to N 2 O will deplete the
Cbl store.
47.4 Clinical Picture
The duration of anesthesia varies from 2 to 8 h; the onset is usually after 2–6 weeks.
SCD is manifested by:
• Parathesia, numbness, weakness of the extremities, optic changes, loss of muscle
tone, loss of position sense, gait instability, ataxia, and eventual spastic paraplegia.
The picture is modifi ed in the presence of peripheral neuropathy. Bladder and
bowel dysfunctions may be observed.
47.5 Diagnosis
Clinical picture confi rmed by neurological exam, electromyography EMG, nerve
conduction studies, Cbl serum level, Schilling test, evoked potential.
MRI will show High intensity signals in the post-lateral column.
47.6 Management
A short course of IV hydroxoocabalamin 5000 μg/day followd by IM cyanocobalamin 1000 μg/day × 5 days followed by 5000 μg QOD and then weekly and then
monthy until recovery. This may take months since N
2
O irreversibly oxidizes the
cobalt ion of cobalamin.
Updates
2009
Chaugny et al.
Nitrous oxide is widely used in anesthesia. It is usually safe but may be associated with severe side effects when it is used repeatedly or on a prolonged time. Here,
we report a case of drug-induced subacute combined spinal cord degeneration
related to cobalamin defi ciency.
47 Subacute Combined Degeneration Provoked by Nitrous Oxide