Acute Disseminated Encephalomyelitis
Acute disseminated encephalomyelitis (ADEM) is a monophasic, demyelinating disease of the central nervous system that predominately affects prepubertal children. It is typically characterized by an abrupt onset of neurologic symptoms preceded by an infection or recent immunization. ADEM is a diagnosis of exclusion in many cases, though the differential diagnosis is extensive. An adult female diagnosed with ADEM represents an atypical presentation; she was subsequently treated successfully with high-dose steroids. Nursing considerations for patients with ADEM include teaching patients and families about the disease, its clinical course, treatment, and prognosis.
This article describes the course of a young female diagnosed with acute disseminated encephalomy-elitis (ADEM) with an atypical presentation. TD is a 22-year-old female who presented to the emergency department (ED) with sudden onset of bilateral frontal and occipital headache, nausea, vomiting, unsteady gait, and numbness and tingling in all four extremities. A stat computed tomography (CT) scan of the brain was obtained to rule out acute subarachnoid hemorrhage, and the scan results were negative. TD was admitted to the hospital for a more thorough neurological workup because of the sudden onset of her symptoms.
On neurological examination, she was found to be in significant pain, moaning, and unable to answer detailed questions. She was mentally aware and alert; however, she appeared exhausted. According to her mother, who was at the bedside, TD had been experiencing generalized weakness and a 3-month history of "leg heaviness" for which she did not seek medical attention. Only in the past day and a half—when the headache began and increased in severity—did TD go to the ED. During the neurological examination, she was able to move her extremities distally and proximally with 4/5 power, and her deep tendon reflexes were 2+ on the left side and 3+ on the right side. Her gait was significantly abnormal with unsteady and wide-based stepping movements, which she explained was a result of the heaviness she felt in her legs. Her pupils were equal, round, and reactive bilaterally, and extraocular movements were intact. The only other significant finding was a bilateral dysmetria evidenced by grossly overshooting on the finger-to-finger test, more prominent on the right than left. Past medical history was not significant for a preceding infectious illness or recent immunizations. She denied any fevers or seizure activity. She also denied any respiratory or gastrointestinal illnesses during the past month. This was the first time the patient had ever been in a hospital. Family history was noncontributory. The patient denied smoking, drinking alcohol, or using any medication, including birth control pills. She had no known allergies and was up-to-date on all immunizations.
Magnetic resonance imaging (MRI) of the brain and spine was ordered, in addition to magnetic resonance angiography (MRA) and magnetic resonance venography (MRV). The MRA and MRV series results were negative. The MRI of the brain showed an enhancing lesion embedded in the right cerebellar hemisphere (Fig 1). The spine MRI showed extensively enhancing, multilevel, intramedullary, abnormal signals in the cervical (Fig 2) and thoracic cord seen on the T2-weighted images as well as on fluid attenuated inversion recovery (FLAIR) images. The findings prompted a neurology consult for further evaluation. The list of differential diagnoses at this point was extensive. A lumbar puncture was performed, and the cerebrospinal fluid (CSF) was sent to the laboratory for a multitude of tests to rule out conditions such as an inflammatory process, sexually transmitted diseases, Lyme disease, and West Nile virus. All of the blood work results were negative. Based on the clinical presentation and neuroimaging, the patient was diagnosed with ADEM. The patient was started on 1 gram intravenous (IV) infusion of methylprednisolone daily for 5 days. Within 1—2 days after the IV steroid infusion, she started to neurologically improve, especially in gait, and was able to ambulate without falling.
(Enlarge Image)
Figure 1.
Axial FLAIR MRI Highlighting a Lesion in the Cerebellum
(Enlarge Image)
Figure 2.
Sagittal MRI of the Cervical Spine Showing Consistencies with Demyelinating Disease
Abstract and Introduction
Abstract
Acute disseminated encephalomyelitis (ADEM) is a monophasic, demyelinating disease of the central nervous system that predominately affects prepubertal children. It is typically characterized by an abrupt onset of neurologic symptoms preceded by an infection or recent immunization. ADEM is a diagnosis of exclusion in many cases, though the differential diagnosis is extensive. An adult female diagnosed with ADEM represents an atypical presentation; she was subsequently treated successfully with high-dose steroids. Nursing considerations for patients with ADEM include teaching patients and families about the disease, its clinical course, treatment, and prognosis.
Introduction
This article describes the course of a young female diagnosed with acute disseminated encephalomy-elitis (ADEM) with an atypical presentation. TD is a 22-year-old female who presented to the emergency department (ED) with sudden onset of bilateral frontal and occipital headache, nausea, vomiting, unsteady gait, and numbness and tingling in all four extremities. A stat computed tomography (CT) scan of the brain was obtained to rule out acute subarachnoid hemorrhage, and the scan results were negative. TD was admitted to the hospital for a more thorough neurological workup because of the sudden onset of her symptoms.
On neurological examination, she was found to be in significant pain, moaning, and unable to answer detailed questions. She was mentally aware and alert; however, she appeared exhausted. According to her mother, who was at the bedside, TD had been experiencing generalized weakness and a 3-month history of "leg heaviness" for which she did not seek medical attention. Only in the past day and a half—when the headache began and increased in severity—did TD go to the ED. During the neurological examination, she was able to move her extremities distally and proximally with 4/5 power, and her deep tendon reflexes were 2+ on the left side and 3+ on the right side. Her gait was significantly abnormal with unsteady and wide-based stepping movements, which she explained was a result of the heaviness she felt in her legs. Her pupils were equal, round, and reactive bilaterally, and extraocular movements were intact. The only other significant finding was a bilateral dysmetria evidenced by grossly overshooting on the finger-to-finger test, more prominent on the right than left. Past medical history was not significant for a preceding infectious illness or recent immunizations. She denied any fevers or seizure activity. She also denied any respiratory or gastrointestinal illnesses during the past month. This was the first time the patient had ever been in a hospital. Family history was noncontributory. The patient denied smoking, drinking alcohol, or using any medication, including birth control pills. She had no known allergies and was up-to-date on all immunizations.
Magnetic resonance imaging (MRI) of the brain and spine was ordered, in addition to magnetic resonance angiography (MRA) and magnetic resonance venography (MRV). The MRA and MRV series results were negative. The MRI of the brain showed an enhancing lesion embedded in the right cerebellar hemisphere (Fig 1). The spine MRI showed extensively enhancing, multilevel, intramedullary, abnormal signals in the cervical (Fig 2) and thoracic cord seen on the T2-weighted images as well as on fluid attenuated inversion recovery (FLAIR) images. The findings prompted a neurology consult for further evaluation. The list of differential diagnoses at this point was extensive. A lumbar puncture was performed, and the cerebrospinal fluid (CSF) was sent to the laboratory for a multitude of tests to rule out conditions such as an inflammatory process, sexually transmitted diseases, Lyme disease, and West Nile virus. All of the blood work results were negative. Based on the clinical presentation and neuroimaging, the patient was diagnosed with ADEM. The patient was started on 1 gram intravenous (IV) infusion of methylprednisolone daily for 5 days. Within 1—2 days after the IV steroid infusion, she started to neurologically improve, especially in gait, and was able to ambulate without falling.
(Enlarge Image)
Figure 1.
Axial FLAIR MRI Highlighting a Lesion in the Cerebellum
(Enlarge Image)
Figure 2.
Sagittal MRI of the Cervical Spine Showing Consistencies with Demyelinating Disease
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