Pseudotumor cerebri following traumatic brain injury in a 29-year-old man

Abstract

We describe a case of pseudotumor cerebri in a young man developing 4 years post-traumatic brain injury (TBI). A 29-year-old man was admitted after sustaining a fall with headache, and no clinical deficits were noted on examination. CT brain demonstrated an extradural hematoma. This was successfully evacuated after his symptomatic worsening. Following this, he developed bone flap infection and had the infected bone flap removed. He developed chronic mild-to-moderate headache following these procedures, which failed to respond to medical treatment. Pseudotumor cerebri was diagnosed. A lumboperitoneal (LP) shunt, ventriculo-peritoneal (VP) shunt, and bitemporal craniectomy were performed as a part of management at different stages. Post-TBI patients may present with chronic headache and in such circumstances, a possibility of pseudotumor cerebri must be considered. Investigations should include neuroimaging in the form of MRI/MRV and fundoscopy to look for papilledema. Management in the form of CSF flow diversion techniques (VP and LP shunt) with medical management results in good clinical outcomes

INTRODUCTION

We describe a case of pseudotumor cerebri in a young man developing 4 years post-traumatic brain injury (TBI). This is a rare clinical scenario and delayed presentation following TBI with chronic headaches should raise this possibility. Detailed clinical history and examination with appropriate investigations must be performed to diagnose this condition. The aetiopathogenesis of this occurrence is discussed with the review of literature.

CASE REPORT

A 29-year-old male was admitted to our unit with head injury following a road traffic accident. His Glasgow Coma Score was 15/15 at admission and had no neurological deficits on examination. CT brain showed small right frontal extradural hematoma with associated pneumocranium, right orbital roof, and cribriform plate fracture. His symptoms worsened over the next 48 h associated with nausea and vomiting. A repeat CT brain scan revealed an increase in the size of the extradural hematoma following which he underwent a frontoparietal craniotomy and evacuation of the extradural hematoma. He recovered well postoperatively and was discharged home. Since discharge, he experienced intermittent frontotemporal headache and worsening of short-term memory. Five months later, he developed a right-sided facial swelling and erythema, with frontal headache, fever, and episodes of vomiting. His blood inflammatory markers were found to be elevated. A post-contrast CT brain revealed bone flap infection. Removal of infected bone flap was performed, and Staphylococcus aureus was isolated from the bone flap. He was treated with intravenous antibiotics for 6 weeks. His headache persisted following treatment, and he had no signs suggestive of raised intracranial pressure.

Six months postoperatively, he was found collapsed following a possible seizure activity. EEG was negative, and he was commenced on sodium valproate for seizure control. He remained well for the next 12 months apart from episodes of headaches on and off. Two years later, a right frontal cranioplasty was performed. MRI brain on follow-up showed no radiological abnormality. He re-presented with worsening headache the following month associated with bilateral papilledema. A lumbar puncture was performed after imaging and was noted to have an elevated CSF pressure of 20 mmHg and a diagnosis of pseudotumor cerebri was made. He underwent a lumbar-peritoneal (LP) shunt and postoperatively his symptoms improved transiently over the next few weeks. He had gained 9 kg of weight over the course of 4 years .Two months subsequent to LP shunt insertion, he presented with worsening headaches and a new lumbar puncture showed an opening pressure of 25 mmHg, and closing pressure of 8 mmHg with no evidence of CSF infection. CT brain revealed no evidence of increase intracranial pressure, and a right ventriculo peritoneal (VP) shunt was inserted. His headache persisted following VP shunt insertion and subsequent to this he underwent bitemporal decompression (craniectomy) in 3 weeks time with the removal of LP shunt [Figure 1]. He had two further VP shunt blockages and he developed a slit ventricle syndrome despite the shunt malfunction [Figure 2]. A CT venogram was performed 1 year later, which demonstrated a stenosis of the left transverse sinus and sigmoid sinus with hypoplastic right transverse and sigmoid sinuses. The cerebral venous pressure was measured with significant left transverse sinus stenosis, and pressure gradient of 26–12 mmHg, >50% gradient. This was managed nonoperatively, and it was found not to contribute to his on-going symptoms. He was also commenced on acetazolamide for the management of Pseudotumor cerebri. He currently remains clinically well and being followed up regularly.

Figure 1.

CT brain demonstrating bitemporal decompressive craniectomy

Figure 2.

CT brain with VP shunt in situ and slit-ventricle with an underlying shunt malfunction

DISCUSSION

Pseudotumor cerebri is more commonly seen in young obese women than men.[1,2] In a study by Durcan et al. determining the incidence of pesuedotumor cerebri in Iowa and Louisiana, it was noted that obesity increased the incidence in both males and females with a overall female-to-male ratio of 8:1.[1] The mean weight was 38% above ideal weight for height in the study.[1] Pseudotumor cerebri is a relatively common neurologic illness and may be an important preventable cause of blindness in obese young women.[1] Pseudotumor cerebri usually presents in adults with transient visual obscuration and blurred vision in contrast to development of diplopia in the pediatric age group.[3,4] Various clinical conditions and factors are associated with development of Pseudotumor Cerebri. In our case we focus on the possible etiology following TBI and have attempted to outline the possible causes for the development of pseudotumor cerebri.

The question then arises as to why all the patients with TBI do not develop pseudotumor cerebri. We believe the causative factors predisposing to pseudotumor cerebri may be multifactorial. Table 1 summarises the possible etiologies in our case. The mechanism of CSF flow dynamics alteration following central nervous system infection and cerebral blood flow alteration is usually seen during the period of insult to the brain and transient alteration in the physiology is a reversible phenomenon depending on the severity of head injury.[5] The pathological process reverts to normal and very rarely, as in this case, may lead to the development of pseudotumor cerebri.

Table 1.

Possible etiologies contributing to pseudotumor cerebri

Mastoiditis and subsequent venous thrombosis is a known to cause pseudotumor cerebri, and there was no evidence of sinus thrombosis on imaging in our case.[6,7] The bone flap infection may have altered the cerebral venous drainage and subsequently caused changes in the CSF slow dynamics. The patient had gained weight following the head injury, and weight gain and obesity are important etiological factors in the development of pseudotumor cerebri.[8,9]

The management was aimed at preventing the possibility of optic nerve damage and blindness in benign intracranial hypertension (BIH), VP shunt, and LP shunt are the surgical options available for pseudotumor cerebri and our patients visual symptoms got better following CSF diversion procedures. We experienced the difficulty in managing the patient in the later stage due to shunt dysfunctions with associated slit-ventricle syndrome.[10] Walter Dandy advocated bitemporal craniectomy for pseudotumor cerebri.[11] This procedure was resorted to, as a last option for controlling headaches and it is known to be effective in symptomatic control in the some group of patients described in the literature. The prognosis for vision in most patients with pseudotumor cerebri is excellent; however, visual loss, which is the only serious complication, may occur either early or late in the course of the disease.[12]

When a post-head injury patient presents with history of chronic headache with visual disturbance, pseudotumor cerebri should be considered a strong possibility. Neuroimaging in the form of brain CT, MRI, and MR venogram must be considered to rule out vascular pathology and a diagnostic lumbar puncture is recommended. CSF flow diversion techniques must be adopted to treat this condition, and these may require long-term follow-up.