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| Journal of the New Zealand Medical Association,
19-February-2010, Vol 123 No 1309
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Annemarei Ranta, Rohitha Mokanahalli
Cerebral venous thrombosis is a potentially fatal
neurological emergency. Both systemic and cerebral thromboses have been reported
in common types of inflammatory bowel disease
(IBD).1–3 Here we present a male patient
with a diagnosis of autoimmune enteropathy, a rare autoimmune inflammatory bowel
disease,4 complicated by cerebral venous
thrombosis, whose thrombophilia screening revealed transient anti-thrombin III
deficiency (ATIII), which resolved during remission of his bowel ailment.
Case reportA 37-year-old right-handed man with autoimmune enteropathy,
based on small intestine biopsy showing absence of goblet cells and serum
positivity for enterocyte antibodies, presented to an emergency room with left
upper extremity weakness, left hemibody numbness, a severe headache and nausea.
A non-contrasted head CT was normal and he was discharged home with a diagnosis
of migraine.
Outpatient neurological evaluation 2 days later revealed a
normotensive, afebrile, thin male with cushingoid facial features due to chronic
steroid therapy. He had a mild left hemiparesis, left hemi-body numbness,
left-sided hyper-reflexia, and positive left Babinski sign. Emergent MRI
revealed increased T2 signal in a bifrontal, parasagittal, predominantly gyral
distribution and subsequent MRV confirmed superior sagittal sinus thrombosis.
The patient was started on IV heparin and showed marked
improvement within 24 hours. Total parenteral nutrition (TPN) was continued pre-
and post-hospitalisation as part of the management of his autoimmune
enteropathy. The autoimmune enteropathy itself was managed with a 1 gm IV bolus
of methylprednisolone followed by 30 mg daily thereafter.
Laboratory work-up included a complete blood count,
metabolic profile and complete thrombophilia screen. The only abnormality was
low ATIII activity at 71% (normal 80-120%).
At 3-month follow-up his neurological exam was normal, but
his autoimmune enteropathy remained very active. A repeat ATIII activity was low
at 74%. Warfarin was continued. Six months after discharge he was started on
infliximab and experienced a marked remission of his gastrointestinal (GI)
symptoms with return to a regular diet, discontinuation of TPN, and resolution
of diarrhoea.
At 9-month follow-up he had attained normal weight with
essentially complete resolution of GI symptoms. His neurological exam remained
normal and a repeat ATIII was normal at 89%. Warfarin was stopped. At 14-month
follow-up he remained stable with normal neurological and GI function and a
normal ATIII level. No further thrombotic events have occurred to date.
DiscussionAutoimmune enteropathy is a very rare IBD with onset
typically in childhood and only recently a handful of adult onset cases were
reported.5 Prior reports of an adult with any
type of associated thrombosis or a child with associated cerebral thrombosis do
not exist to the best of our knowledge. One single paediatric case of autoimmune
enteropathy complicated by systemic thrombosis was found in the
literature.4 Interestingly, no similar
thrombotic complications are reported in non-IBD autoimmune condition affecting
the bowels such as coeliac disease.6
Thrombosis is, however, a well established complication of
the commoner forms of IBD. Yet, the pathophysiology behind the association
between IBD and thomboembolic complications is poorly understood. IBD, in
general, does appear to be an independent risk
factor2 for thrombosis and degree of disease
activity appears to play an important role as risk of blood clot formation
appears limited to the active disease phase.7,8
Intestinal losses disturbing maintenance of important
factors in the clotting cascade during active disease has been
postulated.7 Various coagulation abnormalities
are reported in IBD including increase in pro-coagulants and decrease in natural
anticoagulant factors such as anti-thrombin III, protein S, protein C and
TFP1.3,6,7
This patient had transient acquired anti-thrombin III
deficiency that resolved with the remission of the underlying intestinal
disease. Some of the previously reported cases of thrombotic complications in
IBD have also been noted to be associated with ATIII
deficiency.1,7
Primary ATIII deficiency is a rare inherited thrombophilic
disorder. Acquired ATIII deficiency is described in certain clinical situations
like consumptive coagulopathy, sepsis and nephrotic syndrome and its clinical
significance in the setting of an inflammatory disease remains
unclear.6,9 Attempts to replace purified human
ATIII in patients with acquired ATIII deficiency have not lead to reduction of
thrombosis risk.9 Therefore, transient ATIII
deficiency is probably more of an association rather than a causative factor in
IBD related thrombosis leaving the actual cause yet to be determined.
The transient nature of the AT III deficiency noted here
stresses the importance of repeating the levels after a few months, especially
once inflammatory bowel symptoms go into remission, in order to avoid
unnecessary long-term anti-coagulation. In general, the duration of the
anti-coagulation is probably most appropriately gauged according to IBD disease
activity as manifested by clinical symptomatology. This case also emphasizes the
importance of suspecting the possibility of cerebral sinus thrombosis in
patients presenting with headache with or without focal neurology in the setting
of autoimmune enteropathy and other IBDs.
Author information: Annemarei Ranta,
Consultant Neurologist, Department of Neurology, Palmerston North Hospital,
Palmerston North, New Zealand, Rohitha Makonahalli, Neurology Registrar,
Department of Neurology, Monash Medical Centre, Melbourne, Australia
Correspondence: Dr Annemarei Ranta,
Consultant Neurologist, Department of Neurology, Palmerston North Hospital, PO
Box 11036, Palmerston North, New Zealand. Fax: +64 (0)6 3508391; email: anna.ranta@midcentraldhb.govt.nz
References:
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