Compared to other types of vasculitis, Takayasu arteritis, a rare disease, is more likely to cause neurological symptoms such as headaches, dizziness, stroke, and transient ischemic attacks [1]. The underlying pathophysiology involves multiple components, including thrombosis, embolism, stenosis, and vascular inflammation [1]. The best method for diagnosing Takayasu arteritis is computed tomography angiography (CTA) [2]. Treatment options include pharmacological therapy, endovascular procedures, and, if required, open bypass surgery [2]. This case report discusses a rare instance of Takayasu arteritis that could have caused acute cerebellar ischemic stroke and acute obstructive hydrocephalus.

The primary complaints of a 21-year-old male patient, who was referred from a primary care hospital with acute disorientation, vomiting, slurred speech, lethargy, and focal seizures affecting the upper limbs.
The patient appeared drowsy upon examination, and his blood pressure in the upper limbs measured 130/95 mm Hg in the right upper limb and non-measurable (unrecordable) in the left upper limb, while it was 155/110 mm Hg in the lower limbs. Pulse was 110 beats per minute in the right upper limb but non-palpable in the left upper limb. Oxygen saturation was 93% on room air. Cardiovascular examination revealed normal first and second heart sounds with the presence of a carotid bruit over the right carotid artery. Abdominal examination was unremarkable. Neurological examination revealed bilateral pinpoint pupils unresponsive to light, with a Glasgow Coma Scale (GCS) score of 10/15 (E3V3M4), right plantar withdrawal, and mute left plantar reflexes.

The routine blood tests revealed a normal coagulation profile, and liver and kidney functions; however, urine analysis showed 8-10 pus cells and urine culture demonstrated heavy growth of Acinetobacter species sensitive only to tigecycline. Cerebrospinal fluid (CSF) analysis was unremarkable, and the remaining investigations can be found in Table 1.

An echocardiogram revealed no abnormalities, while a magnetic resonance imaging (MRI) showed findings consistent with acute ischemic infarction in the right cerebellum, pons, middle cerebellar peduncle, and right posterior temporal region (Figures 1A and 1B) along with acute obstructive hydrocephalus (Figures 2C and 2D).

Following consultation with the Department of Neurosurgery, a ventriculoperitoneal shunting procedure was performed under general anesthesia. Upon emergence from general anesthesia, the patient experienced difficulty maintaining adequate oxygen saturation, necessitating transfer to the intensive care unit (ICU) for ventilatory support. Subsequently, a tracheostomy was performed to facilitate prolonged ventilatory assistance. He was extubated after three days. CTA revealed vasculitis of the arch of the aorta and its major branches suggestive of Takayasu arteritis type IIa (Figure 3).

Takayasu arteritis was diagnosed using the American College of Rheumatology (ACR) criteria in which 5 out 6 criteria were present in this patient (Table 2).

He was put on broad-spectrum intravenous (IV) antibiotics, initially meropenem, which was changed to tigecycline based on urine culture and sensitivity. IV linezolid, IV omeprazole, IV multivitamins, IV Dexamethasone, IV Valproic acid, IV levetiracetam, Beclomethasone, and ipratropium nebulization were further included in the treatment.

The patient recovered from hydrocephalus; however, he experienced persistent neurological deficits, such as motor weakness, coordination problems, and speech difficulties. He was discharged from the hospital and thereby referred to a Rehabilitation Centre for physical and speech therapy.

Takayasu arteritis is a rare type of vasculitis, that can result in stenosis, occlusion, and aneurysm of large arteries predominantly in females and is more common in Asians [1] but in our case, it affected the male patient. More than half of patients with Takayasu arteritis can show neurological manifestations like dizziness (78%), headaches (25%), transient ischemic attack (3-22%), and stroke (10-20%); however, our patient did not demonstrate dizziness or headache but presented with the rare manifestation of stroke.

Patho-physiologically, it is a condition characterized by inflammatory granulomatous vasculitis affecting medium and large arteries. This inflammation can result in the thickening, fibrosis, and ultimately occlusion of multiple blood vessels and consequent ischemic manifestations. Furthermore, the ischemic stroke may be caused by aneurysm and sinus venous thrombosis, meningeal and brain parenchymal involvement resulting from granulomatosis and perivasculitis, and encephalopathy due to cytokine damage [2].

 The diagnosis of Takayasu arteritis is frequently delayed due to its nonspecific clinical presentation. Takayasu arteritis is diagnosed based on Modified Ishikawa's criteria and the ACR criteria (Table 2) [3,4]. The treatment modalities for Takayasu arteritis include pharmacological therapy (the predominant modality used in our case), endovascular intervention, and open bypass surgery [4]. Commonly utilized agents encompass corticosteroids and traditional immunosuppressive medications, but for resistant or intolerant patients, biologic drugs such as tumor necrosis factor (TNF) inhibitors may be used. In cases of arterial stenosis, balloon angioplasty, stent graft replacement, or surgical bypass may offer benefits [5].

Strokes are less frequent in younger adults compared to older adults, with approximately 10-15% of all strokes occurring in individuals aged 18 to 50 years. Unexpectedly, this occurred in our study [6]. Vasculitis represents a rare etiology, accounting for 3-5% of strokes in individuals under the age of 50 [7], again highlighting the rarity of our study. Among individuals with TA, 59% experienced neurological impairment, 35% reported recurrent strokes, and 24% developed epilepsy [8].

Hydrocephalus in this case could be secondary to posterior fossa oedema caused by hypertensive posterior reversible encephalopathy syndrome [9,10]. It can emerge as a rare yet life-threatening complication of systemic autoinflammatory diseases, including primary vasculitis. To date, hydrocephalus has been reported only in a single case of TA, making our study a significant contribution to the medical literature. It is managed through anti-inflammatory or anti-infectious treatments, with surgical interventions such as ventriculoperitoneal shunts proving effective in the majority of cases [9,10].

In our study, we highlight a unique case of Takayasu arteritis in a young male patient who developed an acute cerebellar ischemic stroke and hydrocephalus as complications. Early diagnosis and management of Takayasu arteritis in young patients can prevent such complications from occurring.

In conclusion, this case report highlights the rare but significant complications of Takayasu arteritis in a young male patient, who presented with acute obstructive hydrocephalus and acute cerebellar ischemic stroke. The simultaneous occurrence of these complications underscores the complexity and diverse manifestations of this vascular disorder. This case serves as a reminder for healthcare providers to consider Takayasu arteritis as a differential diagnosis in young patients presenting with neurological symptoms. Prompt recognition and management are crucial to prevent further neurological deterioration and improve patient outcomes. Moreover, this case emphasizes the necessity of multidisciplinary collaboration involving rheumatologists, neurologists, and neurosurgeons in the comprehensive care of patients with Takayasu arteritis, particularly when encountering rare complications such as acute obstructive hydrocephalus and cerebellar ischemic stroke.

No conflict of interests.

Written informed consent was obtained from the patient for publication of this case report and accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this Journal on request.

The study was approved by the Institutional Research and Ethical Review Board of Khyber Medical College Peshawar in accordance with the declaration of Helsinki (2013), IREB No: 367, Dated: 9^th May 2024.

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

1. Writing the manuscript, Conceptualization: Muhammad Hashim (Main Author)

2. Review and editing: Asif Khan (Co-Author)

3. Writing the paper: Muhammad Khadim (Co-Author)

4. Data Curation: Sabir Shah (Co-Author)

5. Data Curation: Awais Ahmad (Co-Author)

6^.  Supervision, Proof Reading: Farhan Shahzad (Co-Author)