A Case of Deep Cerebral Venous Thrombosis

Presenting Like Acute Necrotizing Encephalopathy

Acute necrotizing encephalopathy is a rare, severe encephalopathy

seen more commonly in Asian countries. It is thought to be
triggered by a viral infection (influenza, HHV-6) in a genetically
susceptible host. MRI finding are characterized by symmetrical brain
lesions preferentially affecting the thalamus bilaterally, which help to
make a prompt diagnosis of acute necrotizing encephalopathy. However,
neuroimaging findings in such cases should be interpreted very
cautiously because venous sinus thrombosis of deep cerebral veins can
produce similar neuroimaging findings characterized by infarction and
vasogenic edema of bilateral thalami.
We report a 2 and half year old girl who presented with complaints of
irritability which lasted for 2 days followed by altered sensorium for 1
day. 2 weeks before this presentation patient was admitted for
bronchopneumonia and was discharged after 7 days of i.v antibiotics. 7
days after being discharged child developed excessive irritability with
decrease intake of oral feeds that lasted for 2 days followed by altered
sensorium. Patient was developmentally normal with no history of any
other major illness in past. On admission patient GCS was E2V2M5
with blood pressure 116/68 mmHg, HR 120/min, RR 26/min. Her
cranial nerves were intact. She had no weakness, ataxia, sensory
disturbance, or meningeal signs. Motor examination revealed increased
tone of both upper limb and lower limb, power <3/5 with brisk deep
tendon reflexes and extensor plantar response. Patient developed
seizures on admission and she was started on i.v. phenytion, valproate
and levetiracetam.
Magnetic resonance (MR) imaging brain revealed large areas of
restricted diffusion appearing T2W/FLAIR hyperintense and T1W
hypointense involving bilateral thalami suggestive of acute necrotizing
encephalitis.
 A Case of Deep Cerebral Venous Thrombosis
Presenting Like Acute Necrotizing Encephalopathy

Magnetic resonance imaging of brain showing large areas restricted diffusion(g)

appearing T2W hyperintense(b) and T1W hypointense(a) involving bilateral
thalami, neuroimaging findings consistent with acute necrotizing encephalitis.
Patchy areas of blooming(c) on SWI are seen in region of bilateral thalami.
 A Case of Deep Cerebral Venous Thrombosis
Presenting Like Acute Necrotizing Encephalopathy

Based on clinical presentation and neuroimaging findings a tentative

diagnosis of acute necrotizing encephalitis was made. Child was started
on I.V methylprednisolone pulse therapy (30mg/kg OD). However child
did not respond to treatment, magnetic resonance venography was
planned which revealed deep cerebral venous thrombosis involving right
transverse and sigmoid sinus, straight sinus, bilateral internal cerebral
veins and vein of galen.

MR venography of brain showing absent flow related enhancement in right tranverse

and sigmoid sinuses, straight sinus, bilateral cerebral veins.(a,b,c,d)

As the diagnosis of deep cerebral venous sinus thrombosis was made, I.v
methylprednisolone was stopped and low molecular weight heparin
started. Patient prothrombotic workup was sent.
 A Case of Deep Cerebral Venous Thrombosis
Presenting Like Acute Necrotizing Encephalopathy

The etiology and the pathogenesis of ANEC remain partially

clear. Usually, it develops secondary to viral infections, including
influenza A and influenza B, parainfluenza, varicella and enterovirus.
Pathologically, the lesions show edema, hemorrhage, and necrosis.

In most cases of ANEC, there is bilateral symmetrical thalamic

involvement. Abnormal signals on MRI are hypointense on T1 and
hyperintense on T2. These findings can be quite extensive. Hemorrhage,
cavitation, and post contrast enhancement are also seen which are
associated with a worse prognosis. Deep cerebral venous thrombosis also
result in vasogenic edema, hemorrhage, and necrosis in the bilateral
thalami which result in similar neuroimaging findings.

The present case reports highlight that in the presence of bilateral

thalami lesions deep cerebral venous thrombosis must be considered in
addition to acute necrotizing encephalopathy. Delays in diagnosis of
DCVT and commencement of anticoagulant therapy can lead to
unfavorable outcomes.