|Year : 2023 | Volume
| Issue : 1 | Page : 132-134
A case of acute confusional state in a patient of chronic hemolytic anemia with secondary Moya–Moya disease
Kavya Koneru, Pradnya Diggikar, Varsha Bhatt, Prashant Gopal
Department of General Medicine, D.Y. Patil Medical College and Hospital, Pune, Maharashtra, India
|Date of Submission||26-Oct-2021|
|Date of Decision||21-Nov-2021|
|Date of Acceptance||24-Mar-2022|
|Date of Web Publication||11-Jul-2022|
101, B. G. Vastu, Pradhikaran, Nigdi, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
Acute confusional state is characterized by disturbed consciousness, cognitive function, or perception. It can develop over a period of hours to days. It can be because of intracranial hemorrhage, central venous sinus thrombosis, stroke, meningoencephalitis, metabolic abnormalities, adverse drug reactions, etc. Moya–moya disease is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in the basal ganglia and means “puff of smoke” appearance which describes the look of the tangled vessels. It can present as stroke or recurrent transient ischaemic attacks. Secondary moya–moya disease can be caused by infections, connective tissue disorders, vasculitis, autoimmune disorders, trauma, hematological conditions, atherosclerosis, etc. Here, we present a case of 14-year-old female, known case of Beta Thalassemia who presented with acute confusional state, incidentally diagnosed with secondary moya–moya and tuberculous meningitis. Acute confusional state here was attributed to tuberculous meningitis.
Keywords: Acute confusional state, beta thalassemia major, cerebral digital subtraction angiography, magnetic resonance imaging, moya–moya disease, tuberculous meningitis
|How to cite this article:|
Koneru K, Diggikar P, Bhatt V, Gopal P. A case of acute confusional state in a patient of chronic hemolytic anemia with secondary Moya–Moya disease. Med J DY Patil Vidyapeeth 2023;16:132-4
|How to cite this URL:|
Koneru K, Diggikar P, Bhatt V, Gopal P. A case of acute confusional state in a patient of chronic hemolytic anemia with secondary Moya–Moya disease. Med J DY Patil Vidyapeeth [serial online] 2023 [cited 2023 Mar 24];16:132-4. Available from: https://www.mjdrdypv.org/text.asp?2023/16/1/132/350685
| Introduction|| |
Acute confusional state is disturbed consciousness, cognitive function, or perception. Moya–moya, is a rare, progressive cerebrovascular disorder caused by blocked arteries at the base of the brain in the basal ganglia and the tangled vessels formed to compensate for the blockage. Primary is associated with gene mutations in RNF213 gene. Secondary can be caused by any underlying disease.
We present a case of 14-year-old female, known case of Beta-thalassemia, with acute confusional state who was incidentally diagnosed with secondary moya–moya and tuberculous meningitis.
In literature, thalassemia and tuberculous meningitis are rare aetiologies for secondary moya–moya.
| Case History|| |
A 14-year-old female, a known case of beta-thalassemia major (IVS 1-5 homozygous), receiving recurrent blood transfusions, presented with altered sensorium, headache, and two to three episodes of vomiting for 1 day. During admission the patient had developed irrelevant talk and irritable behaviour. Patient was conscious, confused, disoriented, and irritable. On examination patient was febrile with a temperature of 100.8°F, pulse of 110 beats/min, blood pressure of 110/70 mm Hg, and SPO2 of 98% on room air. On physical examination she had pallor, mild icterus, and signs of meningeal irritation. On systemic examination splenomegaly was present and other systems were normal. Routine laboratory investigations showed hemoglobin of 6.6 g/dL, total leucocyte count of 2,900/μL, and platelet count of 90,000/μL. Liver function tests had total bilirubin of 4.87 mg/dL, direct bilirubin of 0.48 mg/dL, indirect bilirubin of 4.39 mg/dL, and normal liver enzymes. Renal function tests, serum electrolytes, blood ammonia, and random blood sugar were normal. Dengue serology, rapid malaria test, and Widal test were negative. Blood and urine cultures were sent to rule out sepsis which later had no growth. Procalcitonin was negative which ruled out sepsis. Magnetic Resonance Imaging (MRI) brain (plain + contrast) showed no neuro parenchymal abnormality, gross-calvarial thickening, and widening of diploic space of skull vault due to extra medullary hematopoiesis [Figure 1]. Magnetic resonance angiography (MRA) showed severe narrowing in the clinoid and supraclinoid portions of bilateral (B/L) internal carotid arteries, Right (R) > Left (L) M1 segments of B/L middle cerebral arteries showed severe narrowing with paucity of flow and multiple collateral vessels, narrowing of A1 segment of B/L anterior cerebral arteries and P1 of B/L posterior cerebral arteries suggestive of secondary moya–moya disease [Figure 2] and [Figure 3]. Cerebral digital subtraction angiography (DSA) was done to confirm moya–moya disease [Figure 4] and [Figure 5]. Cerebrospinal fluid analysis was suggestive of tubercular meningitis. Adenosine deaminase (ADA) and cartridge-based nucleic acid amplification test (TB-PCR) were positive. Chest x-ray was normal. Patient was started on weight-based anti- tubercular drugs, anti-platelet agent aspirin, statins, and anti-inflammatory steroids. Patient was continued with tab. hydroxyurea 500 mg and tab. thalidomide 100 mg for beta-thalassemia. Patient improved gradually in 2–3 days and was discharged with regular follow-up.
|Figure 1: MRI brain flair image showing no neuro parenchymal abnormality|
Click here to view
|Figure 2: MRA showing severe narrowing in the clinoid and supraclinoid portions of bilateral (B/L) internal carotid arteries with paucity of flow and multiple collateral vessels as “puff of smoke” appearance suggestive of moya–moya disease|
Click here to view
|Figure 3: MRA showing severe narrowing in the clinoid and supraclinoid portions of bilateral (B/L) internal carotid arteries with paucity of flow and multiple collateral vessels as “puff of smoke” appearance suggestive of moya–moya disease|
Click here to view
|Figure 4: Cerebral DSA showing high-grade stenosis at bilateral supraclinoid internal carotid artery|
Click here to view
|Figure 5: Cerebral DSA showing high-grade stenosis at bilateral supraclinoid internal carotid artery|
Click here to view
| Discussion|| |
Moya–moya syndrome (MMS) is a rare cerebrovascular disease with progressive bilateral narrowing of intracranial parts of the internal carotid artery and proximal parts of the anterior and middle cerebral artery resulting in recurrent hemodynamic ischemic attacks, strokes, and hemorrhages and has rarely been described with thalassemia., In thalassemia, anaemia and low hemoglobin levels may cause tissue hypoxia and hypertrophic vascular endothelium, leading to microvascular stenosis. Multiple lacunar infarcts and arterial occlusions can be due to chronic hypercoagulable state.,,
Tuberculous meningoencephalitis, caused by Mycobacterium Tuberculosis, is characterised by inflammation of meninges and brain causing altered sensorium, irritable behavior, seizures, and hydrocephalus in severe cases. Its incidence is higher in developing counties and in immune-compromised patients. Our patient is a known case of beta-thalassemia major on recurrent blood transfusions having ferritin overload could have contributed to meningoencephalitis. Early diagnosis of tubercular meningitis and immediate treatment with weight-based anti-tubercular drugs and anti-inflammatory steroids is necessary as patients may progress to obstructive hydrocephalus, develop stroke, seizures, etc. India being a tropical country and incidence of tuberculosis being high, patients presenting with altered sensorium, fever, and meningeal signs, tubercular meningitis should also be considered.
Chronic hemolytic anemia patients with recurrent blood transfusions, hypercoagulable states develop stroke, seizures, etc., Our patient has developed secondary moya–moya attributable to hypercoagulable state of beta-thalassemia. Moya–moya disease is initially managed conservatively with anti-platelets, statins, and anti-inflammatory drugs. At later stages if disease progresses direct revascularisation of superficial temporal artery-middle cerebral artery (STA-MCA) bypass can be done to improve blood supply. Our patient was managed conservatively with antiplatelets, statins and is on regular follow-up.
Our patient who was a known case of beta-thalassemia major IV 1-5 homozygous had developed secondary moya–moya and acute confusional state due to tubercular meningitis and was managed conservatively and is on regular follow-up.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
Beta-thalassemia major and tuberculous meningitis are rare aetiologies of secondary moya–moya disease.
| References|| |
Rai D, Garg RK, Malhotra HS, Verma R, Jain A, Tiwari SC, et al
. Acute confusional state/delirium: An etiological and prognostic evaluation. Ann Indian Acad Neurol 2014;17:30-4.
] [Full text]
Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease. Disease showing abnormal net-like vessels in base of brain. Arch Neurol 1969;20:288-99.
Wang Y, Zhang Z, Wei L, Zhang Q, Zou Z, Yang L, et al
. Predictive role of heterozygous p.R4810K of RNF213
in the phenotype of Chinese moyamoya disease. Neurology 2020;94:e678-86.
Das S, Dubey S, Acharya M, Chatterjee S, Lahiri D, Das G, et al
. Thalassemia and Moyamoya syndrome: Unfurling an intriguing association. J Neurol 2019;266:2838-47.
Doctor PN, Choudhary A, Verma M, Merchant RH. Moyamoya syndrome in hemoglobin E-beta thalassemia: A rare presentation and association. J Postgrad Med 2018;64:240-2.
] [Full text]
Sarkar KN, Deoghuria D, Sarkar M, Sarkar S. Moya Moya syndrome in a HbE beta thalassaemic child presenting with hemiplegia in rural West Bengal. IOSR J Med Dent Sci 2016;15:113-8.
Roy S. Moyamoya syndrome in a child with β-Thalassemia major. Pediatr Neurol 2020;18:214-6.
Chin JH. Tuberculous meningitis: Diagnostic and therapeutic challenges. Neurol Clin Pract 2014;4:199-205.
Mahadevan A. The enigma of tuberculous vasculopathy-Is it time to review our dogmas? Neurol India 2016;64:864-7.
] [Full text]
Li Y, Cikla U, Baggott C, Yilmaz T, Chao C, Baskaya MK. Surgical treatment of adult moyamoya disease with combined STA-MCA bypass and EDAS: Demonstration of technique in video presentation. Turk Neurosurg 2015;25:126-31.
Jameson JL. Harrison's Principles of Internal Medicine. McGraw-Hill Education; 2018.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]