Acute hemorrhagic encephalomyelitis (AHEM) is a severe form of encephalitis characterized by fulminant clinical course and presence of hemorrhagic necrosis of the white matter. Mycoplasma pneumoniae (M. pneumoniae) have severe central nervous syndrome complication with encephalitis as that most common pediatric manifestations, but have been extremely rare report with AHME. A 10-year-old boy was referred to emergency room because of drowsy mental status, weakness of left side extremities and truncal ataxia. His deep tendon reflexes were hyperactive, neck stiffness sign and Babinski sign were both positive. Motor power were decreased on the both left upper and lower extremities. The sequences of T2-weighted and gradient recalled echo (GRE) showed hyper-intense lesions on multifocal white natter areas with hemorrhagic signal. Cerebrospinal fluid (CSF) analysis showed the pleocytosis with neutrophil dominant. The results of deoxyribonucleic acid (DNA) test and culture of M. pneumoniae were reported negative in CSF, but immunoglobulin M (IgM) was positive in blood. He received intravenous high dose corticosteroid and macrolide. After discharge, his neurologic function gradually returned to normal including sitting and standing without support. We reported the previously healthy boy with M. pneumonia related AHEM. The early diagnosis with brain MRI and the aggressive immunosuppressive treatment may be beneficial for recovery.
Ataxia ; Brain ; Cerebrospinal Fluid ; Child ; DNA ; Early Diagnosis ; Emergency Service, Hospital ; Encephalitis ; Extremities ; Humans ; Immunoglobulin M ; Leukocytosis ; Leukoencephalitis, Acute Hemorrhagic* ; Lower Extremity ; Magnetic Resonance Imaging ; Male ; Mycoplasma pneumoniae* ; Mycoplasma* ; Neck ; Necrosis ; Neutrophils ; Pneumonia ; Pneumonia, Mycoplasma* ; Reflex, Babinski ; Reflex, Stretch ; White Matter

When the spinal cord is suddenly severed, all the fundamental functions of the spinal cord below the level of injury including the spinal cord reflexes are immediately depressed, which is referred to as spinal shock. The resolution of spinal shock occurs over a period of days to months, and spinal shock slowly transitions to spasticity. The definition of spinal shock and the pattern of reflex recovery or evolution remains as an issue of debate and controversy. The identification of clinical signs that determine the duration of spinal shock is controversial. The underlying mechanisms of spinal shock are also not clearly defined. Various authors have defined the termination of spinal shock as the appearance of the bulbocavernosus reflex, the recovery of deep tendon reflexes, or the return of reflexic detrusor activity. However, many questions remain to be answered, such as: When should we define spinal shock as the end? What types of reflexes appear first among polysynaptic cutaneous reflexes, monosynaptic deep tendon reflexes, and pathological reflexes? Should it include changes in autonomic reflexes such as a detrusor reflex?
Automatism ; Muscle Spasticity ; Reflex* ; Reflex, Stretch ; Shock* ; Spinal Cord ; Spinal Cord Injuries

Guillain-Barré syndrome (GBS) and acute disseminated encephalomyelitis (ADEM) are demyelinating neurologic disorders with different target organs. Although they share similar pathogenetic mechanism, reports of simultaneous occurrence of the 2 disorders are rare. A 2 year 6 month old girl visited our hospital for fever, cough, and general weakness. Although the muscle power of extremities showed mild weakness and voiding difficulty, initial deep tendon reflex of both knees and ankles was normal. A nerve conduction study to evaluate the weakness revealed the absence of F waves. Cerebrospinal fluid analysis demonstrated pleocytosis with lymphocyte predominance and elevated protein levels. Magnetic resonance imaging showed abnormal T2 hyperintensity in pons, medulla and spinal cord. Serum anti-GD1b antibody was positive. Based on clinical findings, laboratory findings, nerve conduction study, and neuroimaging, the diagnosis of GBS and ADEM was made. This is the first case of GBS accompanied by ADEM in Korea.
Ankle ; Cerebrospinal Fluid ; Cough ; Demyelinating Diseases* ; Diagnosis ; Encephalomyelitis ; Encephalomyelitis, Acute Disseminated ; Extremities ; Female ; Fever ; Guillain-Barre Syndrome ; Humans ; Knee ; Korea ; Leukocytosis ; Lymphocytes ; Magnetic Resonance Imaging ; Nervous System Diseases ; Neural Conduction ; Neuroimaging ; Peripheral Nervous System* ; Pons ; Reflex, Stretch ; Spinal Cord

OBJECTIVE: Craniovertebral junctional anomalies constitute a technical challenge. Surgical opening of atlantoaxial joint region is a complex procedure especially in patients with nuchal deformity like basilar invagination. This region has actually very complicated anatomical and functional characteristics, including multiple joints providing extension, flexion, and wide rotation. In fact, it is also a bottleneck region where bones, neural structures, and blood vessels are located. Stabilization surgery regarding this region should consider the fact that the area exposes excessive and life-long stress due to complex movements and human posture. Therefore, all options should be considered for surgical stabilization, and they could be interchanged during the surgery, if required.METHODS: A 53-year-old male patient applied to outpatients’ clinic with complaints of head and neck pain persisting for a long time. Physical examination was normal except increased deep tendon reflexes. The patient was on long-term corticosteroid due to an allergic disease. Magnetic resonance imaging and computed tomography findings indicated basilar invagination and atlantoaxial dislocation. The patient underwent C0–C3–C4 (lateral mass) and additional C0–C2 (translaminar) stabilization surgery.RESULTS: In routine practice, the sites where rods are bound to occipital plates were placed as paramedian. Instead, we inserted lateral mass screw to the sites where occipital screws were inserted on the occipital plate, thereby creating a site where extra rod could be bound. When C2 translaminar screw is inserted, screw caps remain on the median plane, which makes them difficult to bind to contralateral system. These bind directly to occipital plate without any connection from this region to the contralateral system. Advantages of this technique include easy insertion of C2 translaminar screws, presence of increased screw sizes, and exclusion of pullout forces onto the screw from neck movements. Another advantage of the technique is the median placement of the rod; i.e., thick part of the occipital bone is in alignment with axial loading.CONCLUSION: We believe that this technique, which could be easily performed as adjuvant to classical stabilization surgery with no need for special screw and rod, may improve distraction force in patients with low bone density.
Atlanto-Axial Joint ; Blood Vessels ; Bone Density ; Congenital Abnormalities ; Dislocations ; Head ; Humans ; Joints ; Magnetic Resonance Imaging ; Male ; Methods ; Middle Aged ; Neck ; Neck Pain ; Occipital Bone ; Physical Examination ; Posture ; Reflex, Stretch ; Spine

A 19-year-old girl with immunosuppressive agents of tacrolimus and mychophenolate mofetil following liver transplantation due to glycogen storage disease visited hospital due to lower extremity motor weakness and blurred vision. Motor power was checked as grade II in the upper extremities and grade 0 in the lower extremities with absence of deep tendon reflexes and anal sphincter dysfunction. The magnetic resonance imaging (MRI) showed increased T2 high signal intensity lesions from C4 to L2 level of spinal cord, cerebral cortex, and the left optic nerve. The cerebrospinal fluid (CSF) analysis showed pleocytosis. Epstein-Barr virus (EBV) deoxyribonucleic acid (DNA) was detected as 5,954 copies/mL in CSF whereas all other microbiologic tests were negative. Anti-aquaporin 4 antibody and oligoclonal band were not detected. Intravenous immunoglobulin, methylprednisolone pulse therapy and 3-week course of acyclovir were administered. Although motor power in the upper extremities recovered to grade V, motor power in the lower extremities did not show any improvement. The EBV viral load was not detected in the follow-up CSF examination. EBV infection in an immune-compromised patient could cause extensive demyelinating diseases in central nervous system and result in severe disability.
Acyclovir ; Anal Canal ; Brain* ; Central Nervous System ; Cerebral Cortex ; Cerebrospinal Fluid ; Demyelinating Diseases ; DNA ; Epstein-Barr Virus Infections ; Female ; Follow-Up Studies ; Glycogen Storage Disease ; Herpesvirus 4, Human* ; Humans ; Immunocompromised Host ; Immunoglobulins ; Immunosuppressive Agents ; Leukocytosis ; Liver Transplantation ; Lower Extremity ; Magnetic Resonance Imaging ; Methylprednisolone ; Myelitis, Transverse* ; Optic Nerve ; Reflex, Stretch ; Spinal Cord ; Tacrolimus ; Upper Extremity ; Viral Load ; Young Adult

Pseudoparalysis is defined as immobility of the extremity due to painful limb infection. However, such conditions may be misdiagnosed as nerve palsy when abnormal neurologic findings are present. We report herein a case with femoral osteomyelitis that was misdiagnosed as lumbosacral plexitis. A 7-month-old male infant was brought by his parents to the clinic who noticed that he did not move his left leg since 4 days prior to admission. Four days prior to admission, the parents noticed that he was not moving his left leg. There were no signs of inflammation in the involved limb. The left leg showed a motor weakness with grade 4/5, and it was hypotonic with decreased deep tendon reflexes. No abnormal findings were shown in studies which included lumbar spine magnetic resonance imaging (MRI), abdominal computed tomography (CT), and nerve conduction studies. Lumbosacral plexitis was diagnosed presumptively and intravenous immunoglobulin (IVIG) was given. The patient's symptom did not improve with the IVIG. After the orthopedic consultation, a hip MRI was performed and it showed acute osteomyelitis involving the proximal metaphysis of the left femur. Surgical debridement and intravenous antibiotics were administered. The patient's symptoms improved with treatment and he was fully recovered by the time of discharge. During the 4-year follow-up, the patient is doing well without any complication in the bone growth. During infancy, limb infection may present with nerve palsy that is characterized by hypotonic weakness with hyporeflexia. Pseudoparalysis may be the sole sign of osteomyelitis. In a unilateral limb weakness, musculoskeletal infection should be an initial diagnosis of exclusion with a thorough evaluation.
Anti-Bacterial Agents ; Bone Development ; Debridement ; Diagnosis ; Extremities ; Femur ; Follow-Up Studies ; Hip ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Infant* ; Inflammation ; Leg ; Magnetic Resonance Imaging ; Male ; Neural Conduction ; Neurologic Manifestations ; Orthopedics ; Osteomyelitis* ; Paralysis ; Parents ; Reflex, Abnormal ; Reflex, Stretch ; Spine

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.
Administration, Intravenous ; Axons ; Central Nervous System ; Cerebellum ; Child ; Demyelinating Diseases ; Diffusion Tensor Imaging* ; Diffusion* ; Emergency Service, Hospital ; Female ; Follow-Up Studies* ; Humans ; Immunoglobulins ; Infant ; Leukoencephalitis, Acute Hemorrhagic* ; Magnetic Resonance Imaging ; Methylprednisolone ; Mortality ; Neurologic Manifestations ; Parents ; Pupil ; Rare Diseases ; Reflex, Stretch ; Rehabilitation ; Seizures ; Seizures, Febrile ; Stupor ; Survivors ; Thalamus ; White Matter

Pseudoparalysis is defined as immobility of the extremity due to painful limb infection. However, such conditions may be misdiagnosed as nerve palsy when abnormal neurologic findings are present. We report herein a case with femoral osteomyelitis that was misdiagnosed as lumbosacral plexitis. A 7-month-old male infant was brought by his parents to the clinic who noticed that he did not move his left leg since 4 days prior to admission. Four days prior to admission, the parents noticed that he was not moving his left leg. There were no signs of inflammation in the involved limb. The left leg showed a motor weakness with grade 4/5, and it was hypotonic with decreased deep tendon reflexes. No abnormal findings were shown in studies which included lumbar spine magnetic resonance imaging (MRI), abdominal computed tomography (CT), and nerve conduction studies. Lumbosacral plexitis was diagnosed presumptively and intravenous immunoglobulin (IVIG) was given. The patient's symptom did not improve with the IVIG. After the orthopedic consultation, a hip MRI was performed and it showed acute osteomyelitis involving the proximal metaphysis of the left femur. Surgical debridement and intravenous antibiotics were administered. The patient's symptoms improved with treatment and he was fully recovered by the time of discharge. During the 4-year follow-up, the patient is doing well without any complication in the bone growth. During infancy, limb infection may present with nerve palsy that is characterized by hypotonic weakness with hyporeflexia. Pseudoparalysis may be the sole sign of osteomyelitis. In a unilateral limb weakness, musculoskeletal infection should be an initial diagnosis of exclusion with a thorough evaluation.
Anti-Bacterial Agents ; Bone Development ; Debridement ; Diagnosis ; Extremities ; Femur ; Follow-Up Studies ; Hip ; Humans ; Immunoglobulins ; Immunoglobulins, Intravenous ; Infant* ; Inflammation ; Leg ; Magnetic Resonance Imaging ; Male ; Neural Conduction ; Neurologic Manifestations ; Orthopedics ; Osteomyelitis* ; Paralysis ; Parents ; Reflex, Abnormal ; Reflex, Stretch ; Spine

Acute hemorrhagic leukoencephalitis (AHLE) is an acute, rapidly progressing, fulminant demyelinating disease. It is a rare disease of the central nervous system with high mortality; survivors commonly present with significant neurological deficit. We report the case of a 16-month-old girl who survived AHLE and presented with the associated neurologic deficit. The patient came into the emergency department with febrile seizure. She showed bilateral pinpoint-sized pupils and hyperactive deep tendon reflexes. Her mental status was initially drowsy and rapidly progressed to stupor. Extensive demyelination and microbleeds were found in the cerebral white matter, thalamus and left cerebellum on Magnetic resonance imaging (MRI) scans. Her mental status was improved by intravenous administration of immunoglobulin and methylprednisolone. Five months after being discharged, increased white matter connectivity was found on color-coded follow-up MR diffusion tensor imaging (DTI) as compared to previous MRI. We therefore suggest adding the DTI technique when a follow-up MRI is performed in patients with AHLE. It could be useful to visualize the status of axonal injury and to encourage patients and their parents to continue the rehabilitation program.
Administration, Intravenous ; Axons ; Central Nervous System ; Cerebellum ; Child ; Demyelinating Diseases ; Diffusion Tensor Imaging* ; Diffusion* ; Emergency Service, Hospital ; Female ; Follow-Up Studies* ; Humans ; Immunoglobulins ; Infant ; Leukoencephalitis, Acute Hemorrhagic* ; Magnetic Resonance Imaging ; Methylprednisolone ; Mortality ; Neurologic Manifestations ; Parents ; Pupil ; Rare Diseases ; Reflex, Stretch ; Rehabilitation ; Seizures ; Seizures, Febrile ; Stupor ; Survivors ; Thalamus ; White Matter

Spinal cord infarction is a rare condition and is easily misdiagnosed owing to its initial non-specific manifestation. We report a case of a 77–year-old man who presented with chest pain and upper back pain initially, and was misdiagnosed with a myocardial infarction. Four hours after admission, he complained of numbness in his entire left leg below the knee, with rapid deterioration of neurological symptoms. After 9 hours, loss of sensation progressed up to the T4 dermatome, strength of both lower extremities deteriorated to grade 0, and decrease in anal tone and deep tendon reflex was observed. Initial magnetic resonance imaging findings were normal; however, a signal change occurred 3 days after symptom onset. When patients present with acute chest pain and neurologic symptoms, the possibility of ischemic cardiac disease as well as any neurological manifestations must be investigated. Emergency physicians must remember the value of serial physical examinations.
Back Pain ; Chest Pain ; Emergencies ; Heart Diseases ; Humans ; Hypesthesia ; Infarction* ; Knee ; Leg ; Lower Extremity ; Magnetic Resonance Imaging ; Myocardial Infarction ; Myocardial Ischemia* ; Neurologic Manifestations ; Physical Examination ; Reflex, Stretch ; Sensation ; Spinal Cord Ischemia ; Spinal Cord*