Basilar artery stroke in Crohn’s disease treated with endovascular thromboembolectomy

Abstract

We describe a girl in middle childhood with newly diagnosed Crohn’s disease, who presented with seizures and altered mental status. MRI showed an abnormal vascular signal at the basilar artery, but no evidence of acute ischaemia. Her weakness worsened over the next 8 hours to dense quadriplegia. CT angiography of the brain, approximately 24 hours after the initial onset of symptoms, identified an acute basilar artery occlusion with infarction. She received endovascular thromboembolectomy emergently. She showed significant improvement over 8-month period from quadriplegia to walking unassisted. This case highlights the importance of recognising stroke in patients with inflammatory bowel disease and the need for emergent radiological assessment and potential intervention.

Background

Crohn’s disease is associated with an increased risk of thromboembolic events (TE), including acute stroke.¹ Stroke in patients with inflammatory bowel disease (IBD), especially involving the basilar artery, can be challenging to diagnose given the rarity and variable clinical presentation.

Best efforts should be made to recognise strokes in paediatric patients to maximise the odds of functional neurological recovery. Stroke presentations can create confusion due to rarity of stroke in children and numerous stroke mimics. Paediatric strokes are always difficult to diagnose and can be missed. Thromboembolic basilar artery occlusion can be successfully treated by endovascular thromboembolectomy even 24 hours after presentation with improved long-term neurological outcome.

Case presentation

A girl in middle childhood diagnosed with Crohn’s disease a month prior presented to an outside hospital for lower gastrointestinal bleeding. Social and family history were unremarkable. She was prescribed metronidazole, lansoprazole and increased dose of corticosteroids for management of an acute exacerbation of her Crohn’s symptoms. On her fifth day of hospitalisation, she developed seizure-like activity with up-rolling eyes, incoherent speech, moaning and crying. Right arm weakness and dilated, sluggish left pupil was also noted. There was no change in the clinical status after administration of mannitol and fosphenytoin. Non-contrast head CT showed no acute intracranial process. She was transferred to the Paediatric Critical Care Unit at a children’s hospital for further evaluation and management (figure 1).

Figure 1.

Timeline of events. OSH, outside hospital.

On admission she was afebrile with heart rate 103 beats per minute, respiratory rate 22 per minute, BP 106/60 mm Hg and SpO₂ 100% breathing room air. Cardiovascular and respiratory examinations were normal. She was moaning, crying and had right sided weakness. Left sided motor examination was normal. Pupils were circular and reactive to light with the left pupil slightly larger than the right. Emergent MRI, Magnetic Resonance Angiography (MRA) and Magnetic Resonance Venography (MRV) were performed to evaluate for stroke.

Due to the initial presentation of as focal seizures, Todd’s (postictal) paralysis was considered as an explanation of her weakness in absence of MRI findings of stroke. She was observed overnight. Her weakness progressed to quadriplegia by early morning (NIHSS 25).

Investigations

Emergent MRI, MRA and MRV showed no brain abnormality, specifically no acute ischaemia, mass or haemorrhage. There was a poor enhancement of the mid-basilar artery and distal basilar artery with differential diagnosis of sluggish flow, occlusion or congenital stenosis. Posterior cerebral artery flow was intact with supply from patent bilateral posterior communicating arteries. Cervical segments of the carotid and vertebral arteries were normal. Electroencephalogram (EEG) showed encephalopathy, but no seizures. Complete blood count (CBC) showed a high white cell count (26 900/UL) with neutrophilic predominance. The coagulation panel and cerebrospinal fluid (CSF) analysis were normal.

Due to the progression of weakness to quadriplegia, a stroke protocol CT (non-contrast head CT and CT angiography (CTA) of neck and brain) was ordered emergently approximately 20 hours after admission. Head CT showed ventral pontine hypo-attenuation consistent with ischaemia. Intracranial CTA demonstrated absent enhancement of the mid-basilar artery, consistent with severe stenosis or occlusion (figure 2).

Figure 2.

CT angiography (CTA) demonstrates absent enhancement of the mid-basilar artery and distal basilar artery.

Differential diagnosis

Postictal paralysis versus stroke were differential diagnoses entertained on admission.

Treatment

She was taken emergently to the neurointerventional suite where she was diagnosed with an embolic occlusion of the mid-basilar artery (TICI 0). Mechanical thromboembolectomy of the basilar artery embolic occlusion (figure 3A, B) was performed with one pass of the Penumbra 3Max reperfusion catheter and three Max Separator wire utilising a combination of clot maceration and aspiration per device protocol. Post-thromboembolectomy angiography demonstrated patency of the mid-basilar artery with restoration of antegrade filling. Minimal residual subocclusive thrombus was present at the origin of the left posterior cerebral artery. A residual distal branch occlusion was present in the right parieto-occipital artery (figure 4A, B) (TICI 2b).

Figure 3.

(A) Pretreatment: AP angiogram of the left vertebral artery demonstrates an embolic obstruction of the basilar artery just distal to the vertebrobasilar junction and (B) pretreatment: lateral angiogram of the left vertebral artery demonstrates an embolic obstruction of the basilar artery just distal to the vertebrobasilar junction.

Figure 4.

(A) Post-treatment: AP angiogram of the left vertebral artery demonstrates the removal of the embolus from the basilar artery and the restoration of antegrade flow in the posterior circulation and (B) post-treatment: lateral angiogram of the left vertebral artery demonstrates removal of the embolus from the basilar artery and the restoration of antegrade flow in the posterior circulation.

Outcome and follow-up

Following thromboembolectomy, the patient continued to have flaccid paralysis of her extremities and aphasia with NIHSS 25. Subsequent hypercoagulable workup was negative. She was started on aspirin 81 mg once a day for 3 years. Five days after thromboembolectomy, brain MRI revealed multiple foci of acute/subacute infarct in the cerebellar hemispheres bilaterally, the pons, the midbrain and the right thalamus. Magnetic resonance angiographic examination demonstrated improved flow-related enhancement of the basilar artery.

She was discharged to a rehabilitation institute. Four months after discharge she could nod her head and say a few words but needed assistance with daily living activities. She was assigned a Modified Rankin Score of 4.

Fifteen months after her stroke her neurological assessment showed significant improvement with the remaining symptoms being difficulty walking and speaking. She was assigned a Modified Rankin score of 3. She ultimately returned to the public school in life skills classes (provided for children with special needs) (figure 1).

Discussion

Crohn’s disease is an IBD which presents with gastrointestinal and extraintestinal manifestations which may include thromboembolism.¹ The activation of coagulation factors and hypofibrinolysis during active Crohn’s disease is thought to contribute to the risk of TE.¹ Patients with IBD have a 10%–25% increased risk of stroke and myocardial infarction compared with the general population.² It is estimated that up to 3.3% of children with IBD develop cerebrovascular complications sometime during their disease.³ Ischaemic strokes account for up to 10% of the hypercoagulable manifestations in patients with IBD.⁴ Lazzerini et al. identified only two patients out of reviewed 92 published cases of TE in children with IBD, in which basilar artery was involved with variable clinical presentation ranging from headache to coma, but none described quadriplegia and seizure as an initial neurological manifestation of the patient.⁵

Our case highlights the importance of rapid stroke recognition in hospitalised patients, particularly for patients with comorbidities and confounding factors such as young age. A recent analysis of stroke delivery systems identified differences in the care of community-onset versus hospitalised patients. This prospective cohort study showed that hospitalised patients had delays in diagnosis and treatment. In-hospital stroke patients were also found to have longer hospital stays in comparison to community-onset stroke patients. This study further supports the need for timely diagnosis of stroke in hospitalised patients of all ages.⁶

In our patient initial MRA interpretation was inconclusive. Recent stroke related research proves that neurovascular imaging is an important tool in the triage of patients with acute stroke to determine the best therapy and prognosis.⁷ The Multicentre Randomised Clinical Trial of Endovascular Treatment for Acute Ischaemic Stroke in the Netherlands (MR CLEAN study) showed that emergent cerebrovascular intervention within 6 hours of stroke is safe and effective.⁷ Three recent studies (SWIFT PRIME, ESCAPE and EXTEND-IA) have confirmed the benefit of intervention compared with medical therapy alone.^8–10 Soutome et al. performed mechanical thrombectomy using ADAPT with a Penumbra device for PFO-related childhood paradoxical cerebral embolism of the basilar artery about 21 hours after the symptom onset with favourable neurological outcome.¹¹

Patient’s perspective.

While our daughter was in the hospital due to Crohn’s disease, she suffered from what we thought was seizure activity. After several tests trying to figure out what was going on with her, we were told she is suffering from a stroke due to a blood clot in her brain stem. When the doctors came to our room to tell us emergency surgery needed to be done, one of the risks mentioned was that they were going to use the adult tools since paediatric tools were not available. And what seemed like forever we were informed that the surgery was successful. She still has some residual deficits from the stroke; however she is able to do activities of daily living.

Learning points.

• A high index of suspicion for stroke should be maintained in the presence of altered mental status and seizures in the setting of inflammatory bowel disease.

• Due to the rarity of stroke in the paediatric population, there are no evidence-based guidelines reported in the literature for diagnosis and treatment.

• Modern endovascular interventional techniques have been proven to improve outcomes in stroke patients if delivered in a timely manner.

Ethics statements

Patient consent for publication

Consent obtained from parent(s)/guardian(s).