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October 21–24, 2026 – Cluj-Napoca

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Assist. Prof. Dr. Adriana Cojocaru
President of RSCANP


NEUROLOGICAL ASPECTS IN A TEENAGER GIRL WITH TAKAYASU ARTERITIS

Off , Vol.: 2013,

Autor: Simona Roșu Bogdan Roșu
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The Takayasu arteritis (TA)is a rare inflammatory vasculitis of the large vessels, with a progressive course, frequently affecting young females. During its evolution, a wide range of complications may occur, including neurological/ cerebral issues. We present the case of a 13 years teenage girl, with a late recognition and diagnostication of  Takayasu arteritis, who eventually developed  an acute ischemic stroke with convulsive manifestations.

INTRODUCTION

The Takayasu arteritis is a rare inflammatory vasculitis, with a progressive course, frequently affecting young females, especially of asian origin. During its evolution, a wide range of complications can occur, including neurological/ cerebral issues.Takayasu arteritis is a systemic disorder that affects multiple organs. The diagnosis of TA can be a challenge, especially in its initial phases: there is no diagnostic serologic test and symptoms are generally constitutional, including malaise, fever, fatigue, headaches and arthralgia.

 

CASE REPORT

We present the case of a 13 years old teenage girl who was brought in the Emergency room of our hospital in the 19-th January 2013 with a postictal state, after repeated generalized tonic- clonic seizures, preceded by intense  headache and paresthesias of the right arm.

The girl had a normal development and her familial and personal antecedents were unremarkable until the last year, when she has had several episodes of dyspneea, coughing, mild fever interpreted as pneumonias and treated with antibiotics. She also had several complaints, such as headaches, fatigue, weight loss, sporadic vomiting, visual loss in standing position, claudicating of the lower limbs and a maculo-papular eruption on the abdominal skin. Although she was hospitalized  in Pediatrics, she was only diagnosed with Anemia. Gastro-duodenal reflux. Emotional disorders. Exematides. As the constitutional simptomatology did not improve, eventually she was referred to the Psychiatric Department, were she was treated as an out- patient for a depressive disorder, but she didn’t feel better, so she was admitted into the in-pacient unit. During her stay in the Psychiatry Dept. , she started to develop palpebral and maleolar edemas and, at some point, she presented a severe, acute dyspneic attack and vomiting. She was transferred to the ICU where she was diagnosed with congestive cardiac failure and high arterial blood pressure.. X-ray examination showed cardiomegaly and increased pulmonary vascularisation ; abdominal echography revealed hepatosplenomegaly and ascitis; on the echocardiographic exam the left cardiac cavities were dilated, and the ejection fraction was only 30%. After the intensive treatment with cardiotonics, diuretics, vasodilatators and AB, the evolution was good, except for the high values of the blood pressure. An abdominal CT scan was performed, with contrast, which showed the thickening of the abdominal aorta. The vascular Doppler could not visualize the abdominal aorta at all, so that the suspicion of aortic coarctation emerged at that point. Further investigations  were performed in Cluj and Bucharest (08.01.2012) and angio CT aspect, as well as the angio RMN of aortic and carotidian axis  suggested the Takayasu arteritis in a very advanced stage ( 80% stenosis of the thorachic descendent aorta, 75% stenosis of the left common carotidian artery, complete stenosis of the left subclavicular artery and significant stenosis of the left vertebral artery). Cardiac MRI revealed severe left ventricular insufficiency with EF VA=22-23%, but no signs of miocardic  infarction  or inflammation and thoracic aortic stenosis of more than 90% on a distance of 1-1,5 cm.

The corticotherapy in high doses (Prednisone  2 mg/kg/day) was initiated by the rheumatologist, followed by Solu Medrol (1,5 g) and Ciclophosphamida (400mg) infusions, in addition to the cardiologic treatment. The tolerance was good, so the girl was discharged from the Rheumatology Clinic in January 18, 2013.

On admission in our unit, the girl was afebrile,  confused, agitated, dyspneic, with a mild deficit on the left side. Her skin was pale, the facies was cushingoid , the abdomen was enlarged due to hepato-splenomegaly and ascitis. She had a significant muscular hypotrophy in the lower limbs and swelling of the left arm. The blood-pressure was 165/88 mmHg in the right arm and 103/60mmHg in the left arm, the pulse was 84/ min, feeling different in the arms (very feable in the left arm) and was absent in  the lower limbs.Abdominal and left carotidian bruises were audible. The laboratory investigations were: Na 116mmM/l,  Htc 28,4%, glicemia 195 mg/dl, seric amilazemia 753U/L, GOT 88UI, gamma GT 59 U/l, serum creatinine 1,56% mg/dl and all the rest within normal limits. The CT scan revealed a left parietal cortico- subcortical ischemic area of 20/24 cm.

After 2 weeks of therapy in the ICU, the patient was stabilized, with no neurological deficit, but with persistence of high blood pressure values  and was referred to a vascular surgery facility.

 

DISCUSSIONS

Takayasu’s arteritis, also known as pulseless disease, is a chronic inflammatory disease of unknown etiology that affects the aorta and its main branches. It was first described by Dr. Mikito Takayasu, a Japanese ophthalmologist, in 1905.  Takayasu’s arteritis is most commonly seen in asian females between the ages of 11 and 30.(1) The etiology of Takayasu’s arteritis is unknown, but evidence suggests an autoimmune process, given the association with certain human leukocyte antigen (HLA) alleles and other autoimmune processes such as sarcoidosis and inflammatory bowel disease. It is also suggested that tuberculosis may have an association, given a high prevalence of active and past infection in patients with Takayasu’s arteritis.

TA is rarely reported in children, but there are data suggesting that it can be identified as soon as age 18 months. (2)

The most common debut presentation is with secondary hypertension, frequently associated with general, unspecific symptoms (headache, fatigue, muscular pain, fever, weight loss). The study of Safia et.al.(3) indicates 2/4 cases with clinical signs of  hypertension at the first presentation, but with long preexisting general unspecific symptoms. An other study, mentioned by the same author, conducted in Turkey, reports 86% of the subjects with arterial  hypertension and 71,4% with constitutional signs. In a cohort of patients with TA and stroke, 52,3 % first presented with high arterial blood pressure. (4) Absence of pulse and claudicating occur in more advanced stages of the disease. Despite the fact that our patient has showed  already signs of intermittent claudicating, as well as an impressive aspect of muscular hypotrophy in the legs, her complaints remained unnoticed. The neurological complications (stroke, convulsions, aneurisms) are  mentioned in the literature  with a prevalence of 10-20% (2, 4) , while the inaugural stroke in TA is exceptional. (there is one case mentioned by Sikarood et.al (4.). The mean period between the diagnostication of TA and the neurological complications is, according to the current literature, 1,6 years (4). In our case, due to the important delay in the recognition of TA,  the neurological involvement  occurred as soon as 11 days after the diagnostic of TA was confirmed.

The mechanisms of the stroke in TA are intricated (ischemia and mostly, thrombembolia). MRI analysis revealed that most of the ischemic lesions were located at MCA branches or in the internal/cortical border-zone area. Large lobar type and cortical border-zone infarctions were documented in 33.3% and 28.6% of cases, respectively. Wedge-shaped large lobar and cortical border-zone infarctions are related to embolic mechanisms. In comparison, internal border-zone infarctions – which are known to occur via a hemodynamic compromise mechanism – accounted for only 4.8% of all the stroke events. These results highlight not only the hemodynamic compromise in large-artery stenosis but also the thromboembolic mechanism in ischemic stroke of TA patients.(3). Outcome and prognostic of TA are poor. The disease has a progressive course, with activation  and remission periods of the inflammatory process. There is no specific serologic marker for predicting the evolution of the disease. No known serologic marker exists to diagnose or track progression of disease. ESR, the most commonly used marker to identify systemic inflammatory conditions, is not a reliable indicator and has been found to underestimate disease activity when compared to arterial histopathology or angiography. CT, Doppler angiography and MRI/magnetic resonance angiography can aid in diagnosis and can be used to track disease progression and response to therapy (4). The cardiac, renal and neurological complications are responsible for the morbidity and the mortality in TA. In several follow-up studies, 5-year survival rates of 80% to 90% have been reported.(5). The best predictors for ischemic stroke are the visual symptoms, especially unilateral amaurosis fugax and transient ischemic attacks. (4, 6) In our patient, the alarm symptoms were present long before the occurrence of stroke, but they were considered unremarkable and so they could not prevent the neurological complication. Fortunately, the early therapeutic intervention could prevent further cerebral damage and rapid and ad integrum neurological restitution was achieved. Nevertheless, due to the late recognition of the vasculitis, the general prognosis of the case is quite poor.

The presentation of this case, besides its importance as a rare pathology with neurological involvement, reminds us that the clinical examination of our patients is still the “golden standard” of the medical practice, even in the era of modern, sophisticated technology.

 

BIBLIOGRAPHY

  1. Matthew Silver (2012 December) – Takayasu’s Arteritis – An Unusual Cause of Stroke in a Young Patient. West J Emerg Med; 13(6): 484–487.
  2. Pamela F Weiss,1 Diana A Corao,2 Avrum N Pollock,3 Terri H Finkel,1Sabrina E Smith 4 (2008) Takayasu arteritis presenting as cerebral aneurysms in an 18 month old: A case report . Pediatr Rheumatol Online J. ; 6: 4.
  3. Safia Al abrawi,1 Marine Fouillet-Desjonqueres,1 Louis David,1 Xavier Barral,2 Pierre Cochat,1 and Rolando Cimaz (2008) Takayasu arteritis in children. Pediatr Rheumatol Online J.  6: 17.
  4. Jaechun Hwang,a Suk Jae Kim,a Oh Young Bang,a Chin-Sang Chung,a Kwang Ho Lee,a Duk Kyung Kim, Gyeong-Moon Kim. (2012 June) Ischemic Stroke in Takayasu’s Arteritis: Lesion Patterns and Possible Mechanisms J Clin Neurol. 8(2): 109–115.
  5. Fadi Saab, Robert P. Giugliano, Gregory R. Giugliano,Tex Heart Inst J. 2009; 36(5): 470–474. Takayasu Arteritis in a Young Woman A 4-Year Case History Pediatr Rheumatol
  6. Bindu Menon, A Himabindu(2010)- Takayasu’s disease presenting as convulsive syncope which had been misinterpreted as epilepsy: a case report J Med Case Reports; 4: 352.
  7. Elisa F. Ciceri, Valeria Cuccarini, Luisa Chiapparini, Veronica Saletti, Luca Valvassori (2011) -Paediatric Stroke: Review of the Literature and Possible Treatment Options, including Endovascular Approach  Stroke Res Treat ; 2011: 781612.
  8. Mukund Vidhate, Ravindra Kumar Garg, Rajesh Yadav, Neera Kohli, Praveen Naphade, H. K. (2011) Anuradha-An unusual case of Takayasu’s arteritis: Evaluation by CT angiography- Ann Indian Acad Neurol. Oct-Dec; 14(4): 304–306.
  9. Edworthy SM, Fauci AS, Fries JF, Leavitt RY, et al. (1990) The American College of Rheumatology 1990 criteria for the classification of vasculitis. Patients and methods. Arthritis Rheum. 33:1068–1073

Correspondence to:
Rosu Simona, Oradea, str. J.Pannonius 4

 

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