Pediatric Neurology 51 (2014) 589e590
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
Chronic Granulomatosis Disease With Meningitis and Multiple Brain Abscesses _ Bahadir Ozturk MD *, Lutfi Incesu MD, Ilkay Camlidag MD Department of Radiology, University of Ondokuz Mayis, Samsun, Turkey
Patient Description A 3-year-old boy with fever, fatigue, and vomiting was admitted to the hospital after failing to respond to antibiotics. His history included frequent throat infections. His physical examination and laboratory findings revealed high body temperature, hyperemic oropharynx with multiple crypts on the tonsils, and elevated acute phase reactants. During follow-up, his temperature normalized but his vomiting increased. With a suspected diagnosis of meningitis, magnetic resonance imaging (MRI) was performed. The MRI findings suggested multiple abscesses (Figs 1 and 2) and meningitis (Fig 1). At surgery the lesions were consistent with abscesses, but no organisms were identified. Additional studies revealed a positive nitroblue
tetrazolium test, leading to diagnosis of chronic granulomatous disease (CGD).
Discussion
Central nervous system involvement occurs in only 4-5% of patients with CGD.1-3 The central nervous system may be affected by encephalitis, meningitis, brain abscess and granulomatous events.1 Brain abscesses are characterized by their typical ring shaped enhancement, location at the junction of gray white matter, and surrounding vasogenic edema.1,2 However, conventional MRI can not reliably discriminate brain abscesses and necrotic tumors. Magnetic resonance spectroscopy may reveal a lactate peak within the lesion, and diffusion-weighted images may exhibit restricted diffusion in the central part of an abscess.3 Organisms are not usually
FIGURE 1. T1 coronal contrast enhanced magnetic resonance image coronal (A) axial (B); peripheral ring-like contrast enhanced lesions and midline shift observed because of the effects of these lesions (black arrow). Increased contrast enhancement on the dural surfaces (white arrow).
* Communications should be addressed to: Dr. Ozturk; Department of Radiology; Ondokuz Mayis University; 55139 Atakum; Samsun, Turkey. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.pediatrneurol.2014.06.014
FIGURE 2. (A) Diffusion-weighted image; hyperintense lesions observed in the subcortical white matter. (B) Apparent diffusion coefficient image; these lesions are hypointense, characterized by central diffusion restriction.
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isolated; however, when isolated the most common organism is Aspergillus. The most frequently identified cause of meningitis is Candida. Increased dural enhancement may be evident on contrast enhanced computed tomography and MRI.2 Central nervous system involvement in CGD is potentially fatal. Physicians should be aware of the potential central nervous system complications in patients with CGD.
References 1. Towbin AJ, Chaves I. Chronic granulomatous disease. Pediatr Radiol. 2010;40:657-668. 2. Khanna G, Kao SC, Kirby P, et al. Imaging of chronic granulomatous disease in children. RadioGraphics. 2005;25:1183-1195. 3. Patiroglu T, Unal E, Yikilmaz A, et al. Atypical presentation of chronic granulomatous disease in an adolescent boy with frontal lobe located Aspergillus abscess mimicking intracranial tumor. Childs Nerv Syst. 2010;26:149-154.
An opportunity of this kind presented itself to me in the person of a girl, aged 19, who, according to her father’s statement, had been born healthy, but was attacked with violent convulsions at the age of 3 months, followed by paralysis of the right extremities. She was small for her age, and imperfectly developed; the arm and leg of the right side were much emaciated and were only half the size of their fellows. An autopsy confirmed an old infarction in the left cerebral hemisphere. Moretz Heinrich Romberg Manual of the Nervous Diseases of Man, 1853
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Visual Diagnosis
Chronic Granulomatosis Disease With Meningitis and Multiple Brain Abscesses _ Bahadir Ozturk MD *, Lutfi Incesu MD, Ilkay Camlidag MD Department of Radiology, University of Ondokuz Mayis, Samsun, Turkey
Patient Description A 3-year-old boy with fever, fatigue, and vomiting was admitted to the hospital after failing to respond to antibiotics. His history included frequent throat infections. His physical examination and laboratory findings revealed high body temperature, hyperemic oropharynx with multiple crypts on the tonsils, and elevated acute phase reactants. During follow-up, his temperature normalized but his vomiting increased. With a suspected diagnosis of meningitis, magnetic resonance imaging (MRI) was performed. The MRI findings suggested multiple abscesses (Figs 1 and 2) and meningitis (Fig 1). At surgery the lesions were consistent with abscesses, but no organisms were identified. Additional studies revealed a positive nitroblue
tetrazolium test, leading to diagnosis of chronic granulomatous disease (CGD).
Discussion
Central nervous system involvement occurs in only 4-5% of patients with CGD.1-3 The central nervous system may be affected by encephalitis, meningitis, brain abscess and granulomatous events.1 Brain abscesses are characterized by their typical ring shaped enhancement, location at the junction of gray white matter, and surrounding vasogenic edema.1,2 However, conventional MRI can not reliably discriminate brain abscesses and necrotic tumors. Magnetic resonance spectroscopy may reveal a lactate peak within the lesion, and diffusion-weighted images may exhibit restricted diffusion in the central part of an abscess.3 Organisms are not usually
FIGURE 1. T1 coronal contrast enhanced magnetic resonance image coronal (A) axial (B); peripheral ring-like contrast enhanced lesions and midline shift observed because of the effects of these lesions (black arrow). Increased contrast enhancement on the dural surfaces (white arrow).
* Communications should be addressed to: Dr. Ozturk; Department of Radiology; Ondokuz Mayis University; 55139 Atakum; Samsun, Turkey. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2014 Published by Elsevier Inc. http://dx.doi.org/10.1016/j.pediatrneurol.2014.06.014
FIGURE 2. (A) Diffusion-weighted image; hyperintense lesions observed in the subcortical white matter. (B) Apparent diffusion coefficient image; these lesions are hypointense, characterized by central diffusion restriction.
590
B. Ozturk et al. / Pediatric Neurology 51 (2014) 589e590
isolated; however, when isolated the most common organism is Aspergillus. The most frequently identified cause of meningitis is Candida. Increased dural enhancement may be evident on contrast enhanced computed tomography and MRI.2 Central nervous system involvement in CGD is potentially fatal. Physicians should be aware of the potential central nervous system complications in patients with CGD.
References 1. Towbin AJ, Chaves I. Chronic granulomatous disease. Pediatr Radiol. 2010;40:657-668. 2. Khanna G, Kao SC, Kirby P, et al. Imaging of chronic granulomatous disease in children. RadioGraphics. 2005;25:1183-1195. 3. Patiroglu T, Unal E, Yikilmaz A, et al. Atypical presentation of chronic granulomatous disease in an adolescent boy with frontal lobe located Aspergillus abscess mimicking intracranial tumor. Childs Nerv Syst. 2010;26:149-154.
An opportunity of this kind presented itself to me in the person of a girl, aged 19, who, according to her father’s statement, had been born healthy, but was attacked with violent convulsions at the age of 3 months, followed by paralysis of the right extremities. She was small for her age, and imperfectly developed; the arm and leg of the right side were much emaciated and were only half the size of their fellows. An autopsy confirmed an old infarction in the left cerebral hemisphere. Moretz Heinrich Romberg Manual of the Nervous Diseases of Man, 1853
