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OJHAS Vol. 7, Issue 1: (2008
Jan-Mar) |
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An interesting
case of acute disseminated encephalomyelitis |
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Satish Kumar,
Senior Resident,
Mohit Sharma
Senior Resident,
Ashok Sharma
Professor,
Rajiv Raina
Associate Professor,
Department of Medicine, Indira Gandhi
Medical College, Shimla, Himachal Pradesh, India - 171001 |
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Address For Correspondence |
Registrar Gastroenterology,
Department of Medicine,
I. G. Medical College,
Shimla, Himachal Pradesh, India - 171001.
E-mail:
docsatishkumar@gmail.com |
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Kumar S, Sharma M, Sharma A, Raina R. An interesting
case of acute disseminated encephalomyelitis. Online J Health Allied Scs. 2008;7(1):11 |
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Submitted Feb 17, 2008; Accepted Mar
20, 2008; Published: Apr 10, 2008 |
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| Abstract: |
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Acute disseminated encephalomyelitis (ADEM) is an uncommon inflammatory
demyelinating disease of the central nervous system. The disease typically
occurs after infections or vaccinations. However, in many patients with
ADEM, no evidence of prior infection or vaccination can be found. We
are reporting a patient who developed clinical and radiological features
of acute disseminated encephalomyelitis after trauma of repeated attempts
at lumber puncture for spinal anesthesia.
Key Words:
Acute disseminated encephalomyelitis
(ADEM), Monophasic, Exanthematous fevers |
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Acute disseminated encephalomyelitis (ADEM) is an unusual monophasic
or multiphasic demyelinating disorder of the CNS. Although the pathophysiology
of ADEM is largely unknown, an autoimmune response to myelin basic protein
triggered by infection or immunization is strongly suspected to be the
main etiological factor.1-2 The disease typically occurs
after infections or vaccinations.2-3 However, in many patients
with ADEM, no evidence of prior infection or vaccination can be found. Some
investigators have reported that ADEM can follow treatment with certain
medications, can occur on the heels of trauma, and can develop with
absolutely no precipitating cause (idiopathic ADEM)4. We
report a case of acute disseminated encephalomyelitis presenting after
trauma of repeated attempts at lumbar puncture.
A 56 year old male government employee was referred to our institution
with complaint of inability to move both lower limbs for last five days.
History revealed that patient had been posted for surgery for direct
inguinal hernia of the right side under spinal anesthesia. Patient was
taken to operation theater but surgery had to be deferred because repeated
attempts at lumbar puncture failed and spinal anesthesia could not be
administered. The CSF was not aspirated during any of the attempts and
the anesthetist did not inject any anesthesia. Patient reported loss
of sensations in both lower limbs and inability to move both lower limbs
from the time he was shifted back from operation theater. There was
also history of bowel and bladder incontinence from that day. Patient
underwent surgery next day under local anesthesia and was subsequently
referred to our institution. There was no history of recent febrile
illness, symptoms of upper respiratory tract illness or any immunization.
On examination patient was conscious and his general physical examination
was within normal limits. Nervous system examination showed that higher
mental functions, speech and cranial nerves were within normal limits.
Sensory system examination showed symmetrical pan-sensory loss below
nipples. Motor examination showed power was reduced in both lower limbs
and power of various groups of muscles ranged from grade 1 to grade
4. Power in upper limbs was normal. Knee jerks were exaggerated bilaterally,
rest of the deep tendon reflexes were normal. Superficial reflexes were
bilaterally absent and plantars were bilaterally mute. Abdominal examination
showed a healthy scar of right inguinal hernia surgery. Rest of the
systemic examination was within normal limits. Investigations including
blood urea, serum creatinine, serum bilirubin, AST, ALT, Alkaline phosphatase,
serum electrolytes, complete blood count, chest x ray were normal. MRI
brain and spinal cord showed focal hyperintensities in upper cervical
spinal cord opposite 3rd to 7th cervical vertebra (Fig.1) and white
matter hyperintensities in Rt. Cerebellar hemisphere, medulla, Pons,
B/L Basal Ganglia and B/L Temporal lobes (Fig.2). Radiological diagnosis
of acute disseminated encephalomyelitis was kept. Patient was treated
with intravenous methylpredenisolone 1 gram o.d for 3 days followed
by an oral taper. Patient showed steady improvement during his stay
in the hospital and was able to walk and perform his daily chores with
minimal support and had regained complete bowel and bladder control
within one week of admission.
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Fig 1 |
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Fig 2 |
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Acute disseminated
encephalomyelitis (ADEM) is an uncommon inflammatory demyelinating disease
of the central nervous system. The true incidence of the disease in
India is undetermined and is likely to be more frequent than reported,
as the common antecedent events eg. exanthematous fevers which predispose
to ADEM, are still prevalent. Differentiation of ADEM from the first
attack of multiple sclerosis (MS) is important from prognostic as well
as therapeutic point of view. However, in the absence of biological
markers, at times differentiation of ADEM from the initial presentation
of MS may not be possible even by combination of clinical, CSF analysis,
and MRI. This differentiation is more relevant to India where the incidence
of MS is low.5
Trauma rarely has been reported as a preceding event in pathogenesis
of acute disseminated encephalomyelitis.4 However in the
patient discussed here causal relation ship with the trauma of preceding
repeated lumbar puncture is probable as there are no other apparent
probable precipitating events. The MRI findings and the fact that the
dural space was not reached during attempts of lumbar puncture indicate
that the deficit was not due to the complications of lumbar puncture
itself or of any anesthetic agent. It is still difficult to differentiate
between acute disseminated encephalomyelitis and the first manifestation
of multiple sclerosis. The criteria for the diagnosis of multiple sclerosis
are not met because of the monophasic, acute course of the disease.
In addition, the age of onset (56 years) is uncommon for a first presentation
of multiple sclerosis.2 Though there was definite temporal
relation ship of trauma and ensuing neurological deficit in our patient,
it is difficult to say that the trauma of attempted lumber puncture
had led to the development of acute disseminated encephalomyelitis because ADEM usually occurs several days after trauma or vaccination. It may
just be a coincidence of idiopathic ADEM and trauma of attempted lumbar
puncture.
- Pohl-Koppe
A, Burchett SK, Thiele EA, et al. Myelin basic protein reactive
Th2 T cells are found
in acute disseminated encephalomyelitis. J
Neuroimmunol 1998;91:19–27.
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Storch-Hagenlocher
B, Griffin D. Acute disseminated encephalomyelitis
(parainfectious and postvaccinal encephalitis). In:HackeW, Hanley D,
Bleck T et al.
eds. Neurocritical care. Berlin: Springer, 1994:493–9.
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Murthy J, Yangala R, Meena
A et al. Acute disseminated encephalomyelitis:
clinical and MRI study from south India. J Neurol Sci
1999; 165:133–8.
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David Irani. Neuroimmunological
Lessons Learned from Acute Disseminated
Encephalomyelitis. Transverse Myelitis Association Journal Volume 1 -
January 2006
Article 11.
-
Murthy JM.
Acute disseminated encephalomyelitis. Neurol India 2002; 50:238.
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