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Spinal Cord Compression Due to Intradural Extramedullary Hematopoiesis in a Young Adult with Cooley’s Anemia Mesiano, Taufik; Rasyid, Al; Kurniawan, Mohammad; Hidayat, Rakhmad; Pangeran, David; Lubis, Anna Mira; Saekhu, Mohamad; Gunawan, Kevin; Putri, Stefanie Karina; Rahmana, Arizari Haj; Harris, Salim
Acta Neurologica Indonesia Vol. 1 No. 01 (2023): Acta Neurologica Indonesia
Publisher : Departemen Neurologi Fakultas Kedokteran Universitas Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.69868/ani.v1i01.4

Cooley's anemia, also known as beta thalassemia major, is an inherited multisystemic disorder characterized by skeletal and non-skeletal complications resulting from hemoglobinopathies. Extramedullary Hematopoiesis (EMH) is a complication of thalassemia major due to insufficient erythropoiesis expansion. The incidence rate of paraspinal EMH in beta-thalassemia is rare but tends to be on the rise. We present a case of spinal cord compression due to intradural EMH in a 21-year-old man with Beta Thalassemia major, who exhibited acute lower motor, sensory, and autonomic disorder, along with severe anemia, and electrolyte imbalance. Patients were treated with corticosteroids, blood transfusions, electrolyte correction and pain medications. Several days later, the patient experienced clinical improvement in reduced pain and motor improvement. The patient was planned to undergo elective surgery and radiotherapy after reaching the stabilized condition. Management options of spinal cord compression due to EMH include corticosteroids, adequate blood transfusion, hydroxyurea, radiotherapy, surgical decompression, exchange transfusion, or a combination of these approaches. The choice of therapy should be based on the patient's clinical condition, diagnostic evaluations, and the size of the mass exerting pressure on the spinal cord. The optimal management of EMH remains uncertain. We need further research to establish effective treatment strategies of spinal cord compression due to EMH in Cooley’s Anemia.

Optimizing Stroke Care in Indonesia: A Policy Brief on Expanding Access to Thrombolysis for Improved Outcomes Hidayat, Rakhmad; Rima, Siti Pujiwati Permata; Pangeran, David; Mesiano, Taufik; Kurniawan, Mohammad; Rasyid, Al; Harris, Salim
Acta Neurologica Indonesia Vol. 1 No. 01 (2023): Acta Neurologica Indonesia
Publisher : Departemen Neurologi Fakultas Kedokteran Universitas Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.69868/ani.v1i01.12

Balancing Act: Exploring the Complexities of Drug Restriction Policies Ulwanda, Ramadhanti Salma; Nagpal, Chand Dhiraj; Delarosa, Dinda Olinda; Pradiptaloka, Ediva; Ririhena, Fiorentina Cemerlang; Siniwali, Ida Mas Ayu; Rahmadi, Ikhlas; Jeremia, Ivan; Saputri, Liana Alviah; Agustina, Lisa; Alsakina, Nashiha; Marwadhani, Sarah Shafa; Ni'am, Syauqi Faidhun; Pangeran, David
Acta Neurologica Indonesia Vol. 2 No. 02 (2024): Acta Neurologica Indonesia
Publisher : Departemen Neurologi Fakultas Kedokteran Universitas Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.69868/ani.v2i02.28

Challenges in Managing Oral Manifestations of Anticonvulsant-Induced Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome Fajrin, Fildzah Nurul; Wardhany, Indriasti Indah; Rahmayanti, Febrina; Ikhsan, Muhammad; Pangeran, David
Odonto : Dental Journal Vol 12, No 3 (2025): December 2025
Publisher : Faculty of Dentistry, Universitas Islam Sultan Agung

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.30659/odj.12.3.332-345

Background : Antiepileptic drugs were common causative agents for DRESS syndrome, which was a severe, idiosyncratic drug hypersensitivity reaction characterized by a prolonged latency period, fever, rash, and marked eosinophilia. The oral manifestations present a diagnostic challenge due its similarity to other hypersensitivity reactions as well as its comprehensive management. Objective: This case report aims to describe the challenges in comprehensive management of DRESS syndrome severe oral manifestations, highlighting the challenges from diagnosis to management for patient with complex comorbidities. Case: A 19-year-old female inpatient was referred to Oral Medicine division, Dental and Oro-maxillofacial unit of Universitas Indonesia Hospital (RSUI) for severe oral sores after treated with phenytoin and lamotrigine for her temporal lobe epilepsy and bipolar affective disorder. Patient shows general weakness and clinical examination revealed multiple intra oral erosions, lips erosions with exfoliation and haemorrhagic crust, generalized maculopapular rash, facial oedema, tender lymphadenopathy, and. Laboratory findings confirmed a severe hyper-eosinophilia (32%) and lymphopenia (13.5%), while liver function was normal. A diagnosis of DRESS syndrome was finally established. Case management were immediate withdrawal of phenytoin and lamotrigine, followed by multidisciplinary care including intra vena and topical corticosteroids. The patient's condition significantly improved after collaborative comprehensive treatment. Conclusion: Definitive diagnosis decisions requires careful consideration on the correlation of clinical findings, medication history, and hallmark laboratory results, especially in patients with complex comorbidities and intraoral clinical features with characteristics similar to other hypersensitivity reactions. Comprehensive collaborative management, including patient’s and other healthcare professionals collaboration, is crucial for successful treatment.

Challenges in Managing Oral Manifestations of Anticonvulsant-Induced Drug Reaction with Eosinophilia and Systemic Symptoms Syndrome Fajrin, Fildzah Nurul; Wardhany, Indriasti Indah; Rahmayanti, Febrina; Ikhsan, Muhammad; Pangeran, David
Odonto : Dental Journal Vol 12, No 3 (2025): December 2025
Publisher : Faculty of Dentistry, Universitas Islam Sultan Agung

Show Abstract | Download Original | Original Source | Check in Google Scholar | DOI: 10.30659/odj.12.3.332-345

Background : Antiepileptic drugs were common causative agents for DRESS syndrome, which was a severe, idiosyncratic drug hypersensitivity reaction characterized by a prolonged latency period, fever, rash, and marked eosinophilia. The oral manifestations present a diagnostic challenge due its similarity to other hypersensitivity reactions as well as its comprehensive management. Objective: This case report aims to describe the challenges in comprehensive management of DRESS syndrome severe oral manifestations, highlighting the challenges from diagnosis to management for patient with complex comorbidities. Case: A 19-year-old female inpatient was referred to Oral Medicine division, Dental and Oro-maxillofacial unit of Universitas Indonesia Hospital (RSUI) for severe oral sores after treated with phenytoin and lamotrigine for her temporal lobe epilepsy and bipolar affective disorder. Patient shows general weakness and clinical examination revealed multiple intra oral erosions, lips erosions with exfoliation and haemorrhagic crust, generalized maculopapular rash, facial oedema, tender lymphadenopathy, and. Laboratory findings confirmed a severe hyper-eosinophilia (32%) and lymphopenia (13.5%), while liver function was normal. A diagnosis of DRESS syndrome was finally established. Case management were immediate withdrawal of phenytoin and lamotrigine, followed by multidisciplinary care including intra vena and topical corticosteroids. The patient's condition significantly improved after collaborative comprehensive treatment. Conclusion: Definitive diagnosis decisions requires careful consideration on the correlation of clinical findings, medication history, and hallmark laboratory results, especially in patients with complex comorbidities and intraoral clinical features with characteristics similar to other hypersensitivity reactions. Comprehensive collaborative management, including patient’s and other healthcare professionals collaboration, is crucial for successful treatment.

Posterior Reversible Encephalopathy Syndrome (PRES) with Hypertensive Retinopathy and Hyperaldosteronism Nagpal, Chand; Mesiano, Taufik; Rasyid, Al; Hidayat, Rakhmad; Pangeran, David; Kurniawan, Mohammad; Harris, Salim
Acta Neurologica Indonesia Vol. 4 No. 01 (2026): Acta Neurologica Indonesia
Publisher : Departemen Neurologi Fakultas Kedokteran Universitas Indonesia

Show Abstract | Download Original | Original Source | Check in Google Scholar

Introduction: Posterior Reversible Encephalopathy Syndrome (PRES) is a neurological syndrome with an acute or subacute onset characterized by reversible cerebrovascular dysregulation. Hypertension is the primary risk factor for PRES, however other conditions such as hyperaldosteronism accompany and precipitate PRES. Case Report: This case illustrates a 32-year-old man without prior medical history with loss of consciousness and seizures 1 day prior to admission with blurry vision and vomiting 2 weeks prior that progressed gradually. Brain MRI revealed vasogenic edema of bilateral occipital lobes. The patient was assessed for hypertensive retinopathy and suspected primary hyperaldosteronism. Management with anti-hypertensives, anti-epileptic medications, electrolyte correction and supportive therapy was given, and the patient improved gradually and was discharged 10 days later without any complications. Discussion: No diagnostic criteria is available for PRES, however brain MRI is the crucial to establish a diagnosis of PRES. The resolution of symptoms with blood pressure regulation is another validation of the diagnosis of PRES. Ruling out differentials is prudent to ensure appropriate therapy. Management of PRES encompasses blood pressure regulation, anti-epileptic medications, treating other causes such as drug exposure and metabolic disorders. Previous reports PRES due to secondary hypertension attributed to primary hyperaldosteronism have been reported, although evidence about this causality is limited, nonetheless must be explored and treated as a cause of the hypertension triggering PRES. Conclusion : PRES is a neurological syndrome that is reversible, with hypertension as the primary risk factor that could be secondary due to disorders like primary hyperaldosteronism, that could ultimately trigger PRES.

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