Acute-Disseminated-Encephalomyelitis-ADEM-A-Case-Report.pptx

ADEMS

1. Acute Disseminated Encephalomyelitis (ADEM): A Case Report Acute disseminated encephalomyelitis (ADEM) is a rare, autoimmune inflammatory disorder of the central nervous system (CNS) that affects the brain and spinal cord. It typically presents as a rapidly progressing neurological illness that can cause a wide range of symptoms, including altered mental status, seizures, paralysis, and coma. This case report presents a comprehensive review of ADEM in a 15-year-old female patient, Alpana Harijan, who presented to the emergency room with altered sensorium, fever, slurred speech, and difficulty swallowing. This case report aims to shed light on the clinical presentation, diagnostic evaluation, treatment, and prognosis of ADEM. BY DR. SUNITA ROY

2. Patient History: Alpana Harijan, 15-year-old Female presented in casulty with 1 Altered Sensorium Alpana Harijan presented with an altered sensorium, indicating a disturbance in her level of consciousness and awareness. She exhibited disorientation, confusion, and impaired responsiveness. The onset of her altered sensorium was ten days prior to presentation. 2 Fever Alpana also experienced fever for ten days leading up to her admission. The fever was likely a sign of inflammation in the body, from 10 days 3 Slurred Speech & Difficulty Swallowing Alpana presented with slurred speech and difficulty swallowing, symptoms often associated with neurological dysfunction involving the brain stem or cranial nerves. These issues were attributed to inflammation and edema in the brain, potentially impacting motor control and coordination of the muscles responsible for speech and swallowing. 4 5

3. Clinical Presentation and Evaluation Initial Assessment Upon arrival at the emergency room, Alpana's Glasgow Coma Scale (GCS) score was 10/15 (E4V2M4), indicating a mild to moderate impairment in consciousness. This score is broken down into three categories: eye opening (E), verbal response (V), and motor response (M). The E4 indicates that Alpana could open her eyes to painful stimuli, the V2 indicates that she could only utter incomprehensible sounds, and the M4 indicates that she could withdraw from pain in all limbs. These findings highlight the severity of her altered sensorium. Neurological Findings Neurological examination revealed increased muscle tone in both her upper and lower limbs, indicating rigidity and resistance to passive movements. This finding further supported the presence of neurological dysfunction. Her deep tendon reflexes were brisk, suggesting exaggerated and hyperactive responses to stimuli. The upgoing planters, also known as Babinski's sign, is a neurological sign that indicates damage to the corticospinal tract in the brain. This finding is often associated with central nervous system dysfunction. Cranial Nerves Alpana's cranial nerves were assessed and found to be normal. This suggests that the inflammatory process, while impacting other areas of the brain, did not significantly affect the cranial nerves. This information is important for understanding the extent of the neurological involvement in her case.

4. Initial Treatment and Management Medication Dosage Route of Administration Frequency Xone (Methylprednisolone) 2 grams Intravenous (IV) Twice daily (BD) Acyclovir 500 mg IV in 100 mL Normal Saline (NS) BD Vancomycin 1 gram IV BD Doxycycline 100 mg IV BD Dexamethasone 8 mg IV Three times daily (TDS) Pantoprazole 40 mg IV Once daily (OD)

5. MRI Findings and Diagnostic Considerations Extensive T2 & FLAIR Hyperintensities The MRI revealed extensive hyperintensities on T2-weighted and Fluid-Attenuated Inversion Recovery (FLAIR) images, indicating areas of inflammation and edema in the white matter of the brain. These hyperintensities were predominantly observed along the cortical lining and subcortical white matter of the right cerebral hemisphere. Edema & Mass Effect The right cerebral hemisphere displayed edematous thickening of the cortex, indicating swelling and fluid accumulation. This edema resulted in mass effect, compressing the right lateral ventricle. The diffuse white matter edema further contributed to the mass effect, leading to subtle narrowing of the left foramen of Monro and mild dilation of the left lateral ventricle. Additional Findings The MRI also revealed hyperintensities in the left basal ganglia, thalamic nuclei, and right cerebral peduncle. The absence of any blooming signal on Susceptibility Weighted Imaging (SWI) ruled out the presence of focal or diffuse hemorrhage.

6. Investigations and Laboratory Tests Complete Blood Count (CBC) The CBC provides information on the number and types of blood cells, including red blood cells, white blood cells, and platelets. In ADEM, the CBC may reveal an elevated white blood cell count, indicating inflammation. C-Reactive Protein (CRP) CRP is a protein that is produced in the liver in response to inflammation. An elevated CRP level can indicate inflammation throughout the body, potentially suggesting the presence of ADEM. Liver Function Tests (LFTs) LFTs measure the levels of various enzymes and proteins in the blood that are produced by the liver. Abnormal LFTs may indicate liver dysfunction, which can sometimes occur as a complication of ADEM. Renal Function Tests (RFTs) RFTs assess the function of the kidneys by measuring levels of creatinine and urea in the blood. Kidney dysfunction can occur as a complication of ADEM, potentially due to inflammation affecting the kidneys or due to medication side effects.

7. Treatment Approach: Management Strategies for ADEM 1 Immunomodulatory Therapy Immunomodulatory therapy is a crucial component of ADEM management. It aims to suppress the immune system's attack on the CNS. Corticosteroids, such as methylprednisolone (Xone), are often the initial treatment, followed by a tapering dose of oral prednisone. Other immunosuppressants, like intravenous immunoglobulin (IVIG) or plasma exchange, may be considered for severe cases. 2 Supportive Care Supportive care is essential to manage the symptoms and potential complications of ADEM. This includes monitoring vital signs, maintaining hydration, and managing fever. Anticonvulsants may be used to prevent or control seizures. Nutritional support may be necessary if the patient is unable to eat or drink due to swallowing difficulties. 3 Rehabilitation Rehabilitation plays a vital role in the recovery process. Physical, occupational, and speech therapy can help patients regain lost function and independence. Rehabilitation interventions may focus on improving mobility, strength, coordination, and speech. Regular therapy sessions are essential for optimal recovery.

8. Prognosis and Outcomes: Expected Course and Potential Complications Recovery Most patients with ADEM experience significant improvement with treatment. However, recovery can be slow and may take months or even years. The long-term prognosis depends on the severity of the initial presentation and the extent of neurological involvement. Potential Complications Possible complications of ADEM include residual neurological deficits, such as weakness, paralysis, or cognitive impairment. In some cases, ADEM can be fatal, particularly in individuals with severe or rapidly progressing disease. Monitoring and Follow-Up Close monitoring of the patient's neurological status is crucial during and after treatment. Regular follow-up appointments with a neurologist are essential to assess recovery, manage potential complications, and provide ongoing support.

9. Conclusion: Importance of Early Recognition and Treatment of ADEM This case report highlights the importance of early recognition and prompt treatment of ADEM. Early diagnosis and intervention are crucial for improving outcomes and minimizing long-term neurological deficits. ADEM is a complex and potentially serious condition, but with appropriate medical care, many patients experience significant recovery. Further research is needed to better understand the causes and pathogenesis of ADEM, as well as to develop more effective therapies for this rare and challenging disorder.

10. References National Institute of Neurological Disorders and Stroke. (2022). Acute disseminated encephalomyelitis (ADEM). Retrieved from https://www.ninds.nih.gov/Disorders/All-Disorders/Acute-Disseminated-Encephalomyelitis-ADEM