Infectious Diseases

INFECTIOUS DISEASES



 


 


 







9.1


HIV Dementia Complex


Case History


A 45-year-old man with untreated HIV infection presented with 4 months of cognitive slowing. On examination, he was disheveled and sat passively in the chair. He did not know the date or location and was unperturbed by his failures.


Diagnosis: HIV Dementia Complex


Images


Images 9.1A–9.1D: Axial fluid-attenuated inversion recovery (FLAIR) images demonstrate extensive periventricular and subcortical white matter hyperintensities in a patient with HIV.


Introduction


Images  Direct infection of the central nervous system (CNS) with HIV presents with a dementia that progresses over several months, termed HIV dementia complex (HIVD). The likelihood of developing HIVD is inversely related to the CD4 count, and dementia is considered a late feature of HIV infection. It is now most commonly found in patients with resistance to or nonadherence to highly active antiretroviral therapy (HAART).


Images  It is part of a spectrum of cognitive disorders seen in patients with HIV, which are collectively termed HIV-associated neurocognitive disorders.


Clinical Presentation


Images  Patients present with personality changes, psychomotor retardation, and a subcortical dementia, characterized by difficulty sustaining attention, executive function abnormalities, and slow processing speed. Language and memory functions are relatively unimpaired. By definition, activities of daily living must be impaired.


Images  This presentation may mimic infections with cytomegalovirus, progressive multifocal leukoencephalopathy (PML), and neoplastic processes, lymphoma in particular. However, these are much more likely to present with focal neurological deficits compared to HIVD.


Radiographic Appearance and Diagnosis


Images  The radiographic appearance of HIVD typically shows symmetric, confluent white matter hyperintensities on T2-weighted images without mass effect or enhancement. Significant, diffuse atrophy is common.


Images  There is no specific test for HIVD, but it is important to evaluate patients for other opportunistic infections and other causes of dementia.


Treatment


Images  There is no specific treatment for HIVD beyond starting HAART therapy. The overall prognosis is poor.


References


1.  Brew BJ, Chan P. Update on HIV dementia and HIV-associated neurocognitive disorders. Curr Neurol Neurosci Rep. August 2014;14(8):468.


2.  Manji H, Jäger HR, Winston A. HIV, dementia and antiretroviral drugs: 30 years of an epidemic. J Neurol Neurosurg Psychiatry. October 2013;84(10):1126–1137.


3.  Kelly CM, Miller AR, Aji B, Solomon T. HIV dementia: a diagnosis to keep in mind. Br J Hosp Med (Lond). July 2012;73(7):410–411.


4.  Steinbrink F, Evers S, Buerke B, et al. Cognitive impairment in HIV infection is associated with MRI and CSF pattern of neurodegeneration. Eur J Neurol. March 2013;20(3):420–428.



 


 


 







9.2


HIV-Associated Vacuolar Myelopathy


Case History


A 56-year-old woman with a history of untreated HIV developed paraparesis with severe burning and tingling.


Diagnosis: HIV-Associated Vacuolar Myelopathy


Images


Images 9.2A and 9.2B: Sagittal and axial T2-weighted images demonstrate the characteristic findings of HIV-myelopathy. The red arrows point to lateral corticospinal tracts, the yellow and red arrows point to the dorsal columns, and the blue arrow points to the anterior corticospinal tract.


Introduction


Images  HIV-associated vacuolar myelopathy (VM) occurs during the late stages of HIV infection, when CD4+ counts are very low. It is thought that HIV impairs the vitamin-B12-dependent transmethylation pathway.


Clinical Presentation


Images  Patients present with gradual paraparesis, stiffness, sensory loss, imbalance, and bowel/bladder dysfunction. The arms are usually spared. Pain, due to involvement of the dorsal columns, is common. It often occurs in conjunction with other AIDS-defining illnesses.


Radiographic Appearance and Diagnosis


Images  MRI is the preferred imaging modality. Spinal cord atrophy is the most common finding. The thoracic cord is most commonly affected, but the entire cord is vulnerable. In some patients, there is vacuolization of the corticospinal tract and dorsal columns, resembling the myelopathy produced by B12 deficiency. Other patients may have more diffuse signal abnormality.


Treatment


Images  There is no known treatment, nor is there evidence that antiretroviral therapy can improve symptoms or slow progression. Supportive care includes antispasticity agents, management of sphincter dysfunction, pain management, and rehabilitation.


References


1.  Di Rocco A, Simpson DM. AIDS-associated vacuolar myelopathy. AIDS Patient Care STDS. June 1998;12(6):457–461.


2.  Di Rocco A. Diseases of the spinal cord in human immunodeficiency virus infection. Semin Neurol. 1999;19(2):151–155.


3.  Di Rocco A, Bottiglieri T, Werner P, et al. Abnormal cobalamin-dependent transmethylation in AIDS-associated myelopathy. Neurology. March 2002;58(5):730–735.



 


 


 







9.3


Herpes Simplex Encephalitis


Case History


A healthy 23-year-old man presented with confusion and agitation for 3 days. He was disoriented with weakness of his left arm, of which he was unaware. He had a seizure in the emergency department.


Diagnosis: Herpes Encephalitis


Images


Images 9.3A and 9.3B: Axial CT images demonstrate extensive edema with hemorrhage in the right temporal and frontal lobes in a patient with HSV encephalitis. Images 9.3C and 9.3D: Axial FLAIR images demonstrate hyperintensity and necrosis of the right temporal lobe.


Introduction


Images  The most common cause of viral encephalitis in the United States is infection with herpes simplex virus 1 (HSV-1). The presumed route of infection is reactivation of latent virus within the trigeminal ganglion.


Clinical Presentation


Images  Patients present with rapid-onset encephalitis. This includes seizures, headaches, and changes in cognition or personality. Fever is common.


Radiographic Appearance and Diagnosis


Images  HSV has a predilection for the limbic system. CT shows asymmetric hemorrhagic necrosis of the inferior frontal and temporal lobes. This finding is absent early in the disease course, however, and initial CT may be completely normal. On MRI T2-weighted images, there is hyperintensity of the affected areas, both cortex and white matter. With the addition of contrast there may be a variety of patterns, including enhancement of the meninges, cortex, or focal enhancement, often ring-shaped. Restricted diffusion is common as well.


Images  Cerebrospinal fluid (CSF) is hemorrhagic with a leukocytosis ranging from 5 to 1,000 cells/mm3. Initially these are monocytes, but a lymphocytic predominance develops over time. There may be mild elevations of the protein and opening pressure and a normal or mildly decreased glucose. The diagnosis can be confirmed by HSV polymerase chain reaction (PCR).


Images  An electroencephalogram (EEG) may show periodic lateralizing epileptiform discharges, generalized slowing, or focal temporal lobe spikes.


Treatment


Images  It is treated with intravenous (IV) acyclovir. Treatment should begin as soon as the illness is suspected, as mortality approaches 75% in untreated patients. Survivors are often left with permanent deficits, with less than 5% of patients making a complete neurological recovery. Renal function must be carefully monitored in patients on acyclovir.


References


1.  Safain MG, Roguski M, Kryzanski JT, Weller SJ. A review of the combined medical and surgical management in patients with herpes simplex encephalitis. Clin Neurol Neurosurg. November 2014;128C:10–16.


2.  Kennedy PG, Steiner I. Recent issues in herpes simplex encephalitis. J Neurovirol. August 2013;19(4):346–350.



 


 


 







9.4


Ramsay Hunt Syndrome


Case History


A 45-year-old woman developed a painful rash on her right ear, followed by hearing loss and a facial palsy on that side.


Diagnosis: Herpes Zoster Oticus


Images


Images 9.4A and 9.4B: Axial FLAIR and postcontrast T1-weighted images demonstrate hyperintensity and enhancement of the seventh and eighth cranial nerves (red arrow) and geniculate ganglion (yellow arrow) on the right.


Introduction


Images  Herpes zoster oticus (Ramsay Hunt syndrome) is caused by reactivation of the varicella zoster virus (VZV) in the geniculate ganglion of the facial nerve.


Clinical Presentation


Images  It presents with a painful, vesicular rash of the external acoustic meatus and tympanic membrane, ipsilateral peripheral facial weakness, and loss of taste. The pain precedes the rash by several hours or days. There may be hearing loss, tinnitus, ataxia, or vertigo in some cases.


Radiographic Appearance and Diagnosis


Images  MRI may reveal hyperintensity and enhancement of the facial nerve, geniculate ganglion, and even the facial nerve nucleus in the pons. Similar findings may be seen in Bell’s palsy, though some enhancement of the facial nerve can be a normal finding.


Images  Patients will have red, oozing blisters typical of a shingles rash over their ears, within the ears, and in their ear canals.


Images


Images 9.4C: The typical rash of varicella zoster virus is shown on a thoracic dermatome.


Treatment


Images  Prednisone and acyclovir may hasten recovery if started within the first three days of symptom onset. Older patients and those with more severe facial weakness have a worse prognosis.


Images  Vaccination against VZV helps prevent the condition.


References


1.  Shin DH, Kim BR, Shin JE, Kim CH. Clinical manifestations in patients with herpes zoster oticus. Eur Arch Otorhinolaryngol. July 2016;273(7):1739–1743.


2.  Kuo CY, Lin YY, Wang CH. Painful rash of the auricle: herpes zoster oticus. Ear Nose Throat J. December 2014;93(12):E47.



 


 


 







9.5


Progressive Multifocal Leukoencephalopathy


Case History


A 54-year-old man with HIV presented with confusion, apathy, and weakness of his left arm for 3 months. He had not taken his HIV medications in several years.


Diagnosis: Progressive Multifocal Leukoencephalopathy


Images


Images 9.5A–9.5C: Axial FLAIR images demonstrate confluent white matter lesions in the frontal lobes bilaterally, and throughout the temporal and parietal lobes on the right, without mass effect. Image 9.5D: The lesions are hypointense on the T1-weighted image.


Introduction


Images  Progressive multifocal leukoencephalopathy (PML) is a devastating infection of oligodendrocytes caused by the JC virus, a papovavirus.


Images  PML most commonly occurs in patients with late-stage AIDS (CD4 < 100) or immunosuppressed patients, such as multiple sclerosis (MS) patients treated with the monoclonal antibody natalizumab.


Clinical Presentation


Images  PML of the CNS presents with subacute onset of focal neurological defects, such as weakness, sensory or visual loss, aphasia, personality changes, or cognitive decline. Most patients experience significant decline over several months. Less commonly, patients may present with a seizure.


Radiographic Appearance and Diagnosis


Images  The MRI typically shows asymmetric involvement of the white matter, often involving the subcortical U-fibers. The lesions are hyperintense on T2-weighted images and hypointense on T1-weighted images, without mass effect. Although enhancement may occur with the administration of contrast, it is not typical. Diffusion restriction is common.


Images  In patients treated with natalizumab, the lesions of PML can initially be indistinguishable from MS lesions. Over time, the lesions of PML grow to be more confluent or may develop a “milky-way” pattern of multiple punctate lesions. There may be uneven restricted diffusion on diffusion-weighted images (DWIs). The diagnosis is confirmed by detecting the JC virus via PCR in the spinal fluid, though the result may be negative early in the disease course depending on the sensitivity of the test used. There is no direct treatment for PML, but with early detection patients with MS may have a favorable outcome.


Images


Images 9.5E–9.5H: Axial FLAIR and postcontrast T1-weighted images demonstrate multifocal white matter hyperintensities in the cerebellum, thalami, insula, and cortex characteristic of PML in a patient treated with natalizumab. The lesions enhance with contrast, and the characteristic “milky-way” appearance (red arrows) is seen in the cerebellum.


Images  It is confirmed by detection of the JC virus via PCR in the CSF, though false negatives may occur. In certain cases, a biopsy is required to definitively make the diagnosis.


Treatment


Images  There is no direct treatment, but reconstitution of the immune system with HAART therapy in AIDS patients may be helpful. Immune reconstitution inflammatory syndrome (IRIS) is an inflammatory responsive that can occur in HIV patients with reconstitution of the immune system after the initiation of HAART. Patients with low CD4 counts who have not received prior HAART therapy are at the greatest risk. It occurs in about 20% of patients. Patients present with visual deficits, confusion, personality changes, cognitive deficits, weakness, ataxia, and seizures. Even with the initiation of HAART, the outcome is generally poor, and the disease is often fatal within 1 year.


Pretreatment Images


Images  MS patients treated with natalizumab have an improved prognosis depending on how far advanced their disease is at the time of diagnosis. Of the approximately 600 cases thus far, 20% have been fatal, and some have made a full recovery.


Images


Images 9.5I and 9.5J: Postcontrast axial T1-weighted and FLAIR images showing white matter lesions consistent with PML in the right occipitoparietal and left frontal lobes. Images 9.5K and 9.5L: Postcontrast axial T1-weighted and FLAIR images demonstrating large areas of enhancement in the right frontal, parietal, and occipital lobes with significant edema after the initiation of HAART therapy.


References


1.  Berger JR. Progressive multifocal leukoencephalopathy. Handb Clin Neurol. 2014;123:357–376.


2.  Ferenczy MW, Marshall LJ, Nelson CD, et al. Molecular biology, epidemiology, and pathogenesis of progressive multifocal leukoencephalopathy, the JC virus-induced demyelinating disease of the human brain. Clin Microbiol Rev. July 2012;25(3):471–506.


3.  Serana F, Chiarini M, Sottini A, et al. Immunological biomarkers identifying natalizumab-treated multiple sclerosis patients at risk of progressive multifocal leukoencephalopathy. J Neuroimmunol. December 2014;277(1–2):6–12.


4.  Baldwin KJ, Hogg JP. Progressive multifocal leukoencephalopathy in patients with multiple sclerosis. Curr Opin Neurol. June 2013;26(3):318–323.



 


 


 







9.6


Tuberculous Meningitis


Case History


A 28-year-old man from India developed cranial nerve deficits and soon became encephalopathic.


Diagnosis: Tuberculous Meningitis


Images


Images 9.6A–9.6C: Postcontrast axial and sagittal T1-weighted images demonstrate extensive ring-enhancing lesions in the basal meninges in a patient with tuberculosis, representing tuberculomas. Images 9.6D–9.6F: Axial FLAIR images show significant edema and mass effect associated with the lesions.


Introduction


Images  Infection with Mycobacterium tuberculosis occurs from person to person by infected respiratory droplets. It is not common in Western countries except in the setting of an immunocompromised state, especially in patients with AIDS. The most common route of infection of the CNS is hematogenous spread from a systemic source. Approximately 10% of patients develop neurological complications.


Clinical Presentation


Images  It most commonly affects the basal meninges, manifesting with cranial neuropathies, primarily of the abducens nerve, and altered mental status. Other presentations include seizures, vasculitis with subsequent infarction, hydrocephalus, and focal neurological deficits if there are tuberculomas within the brain.


Images  Patients with tuberculous meningitis are categorized by stage on presentation, based upon mental status and focal neurological signs as follows:


       1.  Stage I: Patients are lucid with no focal neurological signs or evidence of hydrocephalus.


       2.  Stage II: Patients exhibit lethargy and confusion. They may have mild focal signs, such as cranial nerve palsy or hemiparesis.


       3.  Stage III: Represents advanced illness with delirium, stupor, coma, seizures, multiple cranial nerve palsies, and/or dense hemiplegia.


Images  Myelopathy can occur due to infection of the vertebral body (Pott disease) or due to direct infection of the spinal canal and spinal cord with tuberculomas in the absence of any lesion to the bone. There can be both intraspinal granulomatous tissue, which compresses the cord from the outside, or intramedullary tuberculomas. Pott disease arises from the vertebral body, usually in the thoracic or lumbar spine, which then invades the epidural space. Patients present with the subacute onset of severe, localized pain and fever. Myelopathic signs develop if there is compression of the spinal cord.


Radiographic Appearance and Diagnosis


Images  CNS tuberculosis most commonly causes a basal meningitis with enhancing lesions and resultant mass effect and edema in the brain, as seen in Images 9.6A–9.6F.


Images  Tuberculomas may be found throughout the brain parenchyma as well.


Images  Within the spine, there can be both intraspinal granulomatous tissue that compresses the cord from the outside, as well as intramedullary tuberculomas.


Images  In Pott disease, the thoracolumbar spine is involved in 80% to 90% of cases, but any area of the spinal axis may be affected.


Images  The CSF typically reveals highly elevated protein, often greater than 1,000 mg/dL, a low glucose, and a lymphocytic pleocytosis, though this may not be evident in patients with compromised immune systems. The elevation of the protein may be high enough to cause obstruction of CSF flow and hydrocephalus. Acid-fast bacilli and PCR are usually positive in the CSF.


Images  The diagnosis is often evident due to pulmonary symptoms or tuberculosis elsewhere in the body.


Treatment


Images  Treatment begins with an “intensive phase,” which includes isoniazid, rifampin, pyrazinamide, and a fourth drug, either a fluoroquinolone or an injectable aminoglycoside, given daily for 2 months. This is followed by a regimen of isoniazid and rifampin alone. For HIV-negative patients glucocorticoid therapy is often added. Patients with hydrocephalus or symptoms of increased intracranial pressure (ICP) may require surgical decompression of the ventricular system.


Images


Image 9.6G: Gross pathology demonstrates a thick basal exudate in a patient with tuberculosis (image credit Yale Rosen).


Images


Images 9.6H–9.6K: Postcontrast axial T1-weighted and FLAIR images demonstrate innumerable ring-enhancing lesions throughout the brainstem and brain with surrounding edema and mass effect from tuberculomas.


Images


Images 9.6L and 9.6M: Postcontrast axial and sagittal T1-weighted images of the cervical spine demonstrate thin linear enhancement of the meninges (red arrow) and a larger lesion at C2 with cord compression. On the axial image, the spinal cord is indicated by the yellow arrow, while the large tubercular lesion is indicated by the blue arrow. Image 9.6N: Sagittal T2-weighted image demonstrates hyperintensity throughout the cervical cord.


Images


Images 9.6O and 9.6P: Sagittal T2-weighted and postcontrast TI-weighted images demonstrate a severe compression fracture of the L1 vertebral body with retropulsion of bone into the spinal canal indenting the tip of the conus medullaris in a patient with Pott disease. Image 9.6Q: Gross pathology of Pott disease (image credit Dr. Yale Rosen).


Images


Images 9.6R and 9.6S: Chest radiograph and axial CT image demonstrate miliary tuberculosis.


Images  The illness is universally fatal in 1 to 2 months without treatment, and fatal in about 25% of those with treatment. Immunocompromised patients fare worse.


Images  For patients with Pott disease, the same antitubercular treatment is indicated as for tuberculous meningitis, though neurosurgical debridement and stabilization of the spine may be required in patients with acute neurological deterioration, spinal deformity with instability, resistance to drug therapy, or paravertebral abscess.


References


1.  Bernaerts A, Vanhoenacker FM, Parizel PM, et al. Tuberculosis of the central nervous system: overview of neuroradiological findings. Eur Radiol. August 2003;13(8):1876–1890.


2.  Sahu SK, Giri S, Gupta N. Longitudinal extensive transverse myelitis due to tuberculosis: a report of four cases. Postgrad Med. October–December 2014;60(4):409–412.


3.  Sanei Taheri M, Karimi MA, Haghighatkhah H, Pourghorban R, Samadian M, Delavar Kasmaei H. Central nervous system tuberculosis: an imaging-focused review of a reemerging disease. Radiol Res Pract. 2015;2015:202806.


4.  Mondol BA, Siddiqui MR, Mohammad QD, Saha NC, Hoque MA, Uddin MJ. Tuberculosis of the central nervous system. Mymensingh Med J. April 2010;19(2):312–322.



 


 


 







9.7


Bacterial Meningitis


Case History


A 4-week-old infant presented with lethargy and a fever.


Diagnosis: Infant With Meningitis


Images


Images 9.7A–9.7C: Postcontrast axial T1-weighted and T2-weighted images demonstrate diffuse pial enhancement and bilateral, frontal, subdural empyemas in an infant with a group B streptococcal meningitis. Image 9.7D: Gross pathology of bacterial meningitis (image credit Centers for Disease Control and Prevention [CDC]).


Introduction


Images  Most cases of bacterial meningitis result from the hematogenous spread of bacteria from an upper respiratory tract infection to the choroid plexus (Table 9.7.1). Blood cultures reveal the causative organism in many cases. Alternatively, bacteria can directly enter the subarachnoid space from infections of the nasopharynx, dental abscesses, or following trauma or neurosurgical manipulation. The extension of an infectious agent into the ependymal cells or ventricles is termed ventriculitis.


Clinical Presentation


Images  It most commonly presents with the rapid onset of stiff neck, fever, headache, and altered mental status. Focal neurological deficits, primarily hearing loss and seizures, occur as well. Infants may present with lethargy, poor feeding, and hypothermia as opposed to fever.


Table 9.7.1 Causes of Meningitis by Risk Group

























Risk and/or Predisposing Factor


Bacterial Pathogen


Neonate


Group B streptococci
E coli
L monocytogenes


Childhood–50 years


S pneumoniae
N meningitidis
H influenzae → Vaccine now exists


Age older than 50 years


S pneumoniae
N meningitidis
L monocytogenes
Aerobic gram-negative bacilli


Immunocompromised state


S pneumoniae
N meningitidis
L monocytogenes
Aerobic gram-negative bacilli


CSF shunts/neurosurgery


Staphylococcus aureus
Coagulase-negative Staphylococci
Aerobic gram-negative bacilli, including
Pseudomonas aeruginosa


Skull fracture


S pneumoniae
H influenzae
Group A streptococci


Radiographic Appearance and Diagnosis


Images  Imaging studies are not of paramount importance in the acute situation unless a mass lesion is suspected that may preclude a lumbar puncture. However, an MRI will typically show meningeal enhancement, and in cases of cerebral edema, there will be sulcal effacement. Other later complications may include hydrocephalus, subdural empyema, and infarction.


Images  The typical CSF findings are a leukocytosis (100 to 10,000 WBCs/mL), lowered glucose (<40% of serum glucose), an elevated protein (100–500 mg/dL), and an elevated opening pressure. CSF studies should be sent for gram stain, which is positive in about 60% of cases, and culture, which is positive in about 75% of cases.


Treatment


Images  Treatment for bacterial meningitis should begin as soon as the illness is suspected, even before a lumbar puncture is performed. All patients should be treated empirically with a third-generation cephalosporin with the addition of ampicillin in neonates and patients older than 50. Corticosteroid therapy has also been shown to reduce morbidity from meningitis, such as deafness, though primarily in high-income countries.


Images  With prompt treatment, the mortality rate is approximately 20%, though many survivors still have significant morbidity.


References


1.  Brouwer MC, McIntyre P, Prasad K, van de Beek D. Corticosteroids for acute bacterial meningitis. Cochrane Database Syst Rev. September 2015;9:CD004405.


2.  Oliveira CR, Morriss MC, Mistrot JG, Cantey JB, Doern CD, Sánchez PJ. Brain magnetic resonance imaging of infants with bacterial meningitis. J Pediatr. July 2014;165(1):134–139.



 


 


 







9.8


Intracerebral Abscess


Case History


A 54-year-old man presented with headaches and left-sided weakness.


Diagnosis: Intracerebral Abscess


Images


Images 9.8A and 9.8B: Axial FLAIR and postcontrast T1-weighted images demonstrate a large ring-enhancing lesion in the right frontal lobe with mass effect on the lateral ventricle and significant edema. Images 9.8C and 9.8D: DWI and apparent diffusion coefficient map demonstrate restricted diffusion of the lesion. The abscess is in the early encapsulation stage.


Introduction


Images  Brain abscesses most commonly result from direct infection from an adjacent site. Frontal lobe abscess typically result from a primary infection in the sinuses, while temporal lobe abscess commonly result from a middle ear infection or mastoiditis. In these cases of contiguous spread, the result is a single abscess.


Images  In cases of hematogenous spread, the most common primary infection is pulmonary, followed by endocarditis. In such cases, multiple abscesses may result.


Images  Most abscesses contain multiple organisms, and the most common pathogens are Streptococcus species, Bacteroides, Enterobacteriaceae, and other anaerobic bacteria. In cases of head trauma, Staphylococcal species are most commonly implicated.


Clinical Presentation


Images  Abscesses present with headaches, seizures, nausea and vomiting, and focal neurological deficits. Fever and neck pain are not common. Abscesses may rupture into the ventricles, causing a sudden and drastic deterioration.


Radiographic Appearance and Diagnosis


Images  T1-weighted images typically show central hypodensity with ring enhancement. T2-weighted images may show edema around the lesion, depending on its stage. There is often intense restricted diffusion on DWI.


Images  There are four stages to abscess formation:


       1.  Early cerebritis: There is little mass effect and minimal enhancement.


       2.  Late cerebritis: Characterized by the development of a necrotic center. Early abscesses, as shown in Images 9.8E–9.8H, are associated with a significant amount of edema and intense enhancement.


       3.  Early encapsulation: There is a well-defined mass with intense ring enhancement. This is shown in Images 9.8A–9.8D.


       4.  Late encapsulation: Mature abscesses have a collagen capsule, with less cerebritis and edema. The length of time required to form a mature abscess varies from weeks to months.


Images


Images 9.8E and 9.8F: Postcontrast axial T1-weighted images demonstrate innumerable ring-enhancing lesions in the white matters. The presence of multiple abscesses suggests hematogenous spread from a systemic source. Images 9.8G and 9.8H: Axial FLAIR images demonstrate edema representing an intense inflammatory response. The intense edema and inflammatory response indicate that these are early in their formation.


Images


Images 9.8I and 9.8J: Postcontrast axial T1-weighted images demonstrate ring-enhancing lesions in the right thalamus and pons, with characteristic findings of late encapsulation. Images 9.8K and 9.8L: Axial FLAIR images demonstrate minimal hyperintensity of these later stage abscesses.


Images  The diagnosis is made based on clinical and radiographic features. It is not always possible to distinguish abscesses from neoplasms, and a biopsy may be required to make the diagnosis. CSF analysis and blood cultures are typically of little diagnostic value.


Treatment


Images  Empiric therapy is with vancomycin, metronidazole, and a third-generation cephalosporin for 6 to 8 weeks. Antibiotics are most effective if given prior to encapsulation. In mature abscesses, cases with increased ICP, or abscesses near the ventricles, surgical drainage may be indicated to prevent rupture of the abscess into the ventricle. Clinical improvement precedes radiographic improvement.


References


1.  Sáez-Llorens X, Nieto-Guevara J. Brain abscess. Handb Clin Neurol. 2013;112:1127–1134.


2.  Muzumdar D, Jhawar S, Goel A. Brain abscess: an overview. Int J Surg. 2011;9(2):136–144.


3.  Hakan T. Management of bacterial brain abscesses. Neurosurg Focus. 2008;24(6):E4.



 


 


 







9.9


Spinal Epidural Abscess


Case History


A 36-year-old man with a history of IV heroin use presented with leg weakness, fever, and low back pain. On examination he was weak in both legs with saddle anesthesia.


Diagnosis: Spinal Epidural Abscess


Images


Images 9.9A–9.9D: Postcontrast sagittal and axial T1-weighted images demonstrate an enhancing lesion of the lumbar spine (red arrows) with compression of the cauda equina.


Introduction


Images  Spinal epidural abscess (SEA) is an infection in the spinal epidural space leading to injury of the spinal cord or cauda equina directly by mechanical compression and indirectly by vascular compromise. Most SEAs occur in the thoracic area.


Images  The infectious agent in SEAs is Staphylococcus aureus about 50% of the time. However, Streptococcus, gram-negative bacilli, anaerobes, fungi, and tuberculosis (in endemic areas) can also present with epidural abscess. The most common route of infection is hematogenous spread from infection elsewhere in the body, though about one-third of cases are due to direct spread from an adjacent infection. About one-third of cases have a history of trauma to the back, sometimes quite minor in nature. Patients with compromised immune systems, IV drug use, and skin infections are at the highest risk.


Clinical Presentation


Images  The classic presentation is a triad of fever, back pain/tenderness, and neurological deficits. The following staging system has been described:


       1.  Stage 1: Focal neck or back pain at the level of the spine affected


       2.  Stage 2: Radicular pain and paresthesias


       3.  Stage 3: Motor weakness, sensory deficits, and bladder/bowel dysfunction


       4.  Stage 4: Progression to paralysis


Radiographic Appearance and Diagnosis


Images  MRI best delineates both the longitudinal and paraspinal extension of the abscess and may help differentiate infection from other etiologies. It will reveal an enhancing lesion with compression of the underlying neural tissue. Pus will be hyperintense on T2-weighted images and hypointense on T1-weighted images with restricted diffusion. Diskitis and osteomyelitis occur in 80% of patients and may be demonstrated on CT images as well as MRI.


Images  The responsible pathogen is commonly identified in blood cultures. Serum studies reveal a leukocytosis in two-thirds of patients and typically an elevated erythrocyte sedimentation rate (ESR), though these findings are not specific for SEA. A lumbar puncture is relatively contraindicated if an SEA is suspected, but may be essential to exclude meningitis.


Treatment


Images  Emergency surgical decompression of the spinal cord with drainage of the abscess is the mainstay of treatment. Neurological deficits may progress rapidly and are permanent if they last over 24 hours.


Images  Empiric antibiotic coverage effective against methicillin-resistant staphylococcus aureus (MRSA) is recommended. The combination of antistaphylococcal penicillin, third-generation cephalosporin, and aminoglycoside is prescribed for postsurgical infection. Culture results guide definitive therapy.


References


1.  Connor DE Jr, Chittiboina P, Caldito G, Nanda A. Comparison of operative and nonoperative management of spinal epidural abscess: a retrospective review of clinical and laboratory predictors of neurological outcome. J Neurosurg Spine. July 2013;19(1):119–1127.


2.  Patel AR, Alton TB, Bransford RJ, Lee MJ, Bellabarba CB, Chapman JR. Spinal epidural abscesses: risk factors, medical versus surgical management, a retrospective review of 128 cases. Spine J. February 2014;14(2):326–330.


3.  Arko L, Quach E, Nguyen V, Chang D, Sukul V, Kim BS. Medical and surgical management of spinal epidural abscess: a systematic review. Neurosurg Focus. August 2014;37(2):E4.



 


 


 







9.10


Neurocysticercosis


Case History


A 40-year-old man from India presented with two seizures over the course of one day. The patient denied any prior seizure history as well as any head trauma or family history of seizures. He had a normal neurological exam.


Diagnosis: Neurocysticercosis


Images


Images 9.10A–9.10C: Postcontrast axial T1-weighted images demonstrate round, peripherally enhancing lesions with a central hyperintensity (red arrows) in the brain parenchyma and the Sylvian fissure on the right (yellow arrow). Images 9.10D–9.10F: Axial FLAIR images demonstrate cystic lesions with a central hyperintensity (red arrows) representing the scolex, the “hole with dot” sign of the vesicular stage of neurocysticercosis.


Introduction


Images  Neurocysticercosis is caused by infection with Taenia solium, the pork tapeworm. It is the most common parasitic infection of the CNS and is common in India, Mexico, and South America, as well as parts of Asia, Africa, and Eastern Europe.


Images  Initial human infection occurs due to the consumption of infected pork. The cystericerci embed in the stomach and the eggs are excreted in the feces. Ingestion of T. solium eggs then occurs via the fecal–oral route. The larvae attach to and invade the intestine where they migrate throughout the body (Illustration 9.10.1).


Images


Image 9.10G: The scolex (head) of Taenia solium with its four suckers and two rows of hooks (image credit CDC).


Clinical Presentation


Images  Symptoms most frequently arise when the cyst dies within the brain parenchyma, triggering an inflammatory reaction. It is the most common cause of acquired epilepsy in endemic areas, and seizures are the most common presentation (70%–90% of patients). It is common in the United States in immigrants from endemic areas.


Images  With a large lesion burden, there may be significant mass effect and edema, producing focal neurological deficits, headache, encephalopathy, hydrocephalus, and meningismus.


Images


Illustration 9.10.1: Life cycle of Taenia solium (Illustration credit CDC).


Images


Images 9.10H–9.10J: Postcontrast axial T1-weighted, FLAIR, and noncontrast axial CT images demonstrate a ring-enhancing lesion in the right frontal lobe with an intense inflammatory reaction from neurocysticercosis in the colloidal stage. On the noncontrast axial CT image, an older, calcified lesion is also seen (red arrow).


Imaging Characteristics and Diagnosis


Images  There are four stages to the life cycle, though more than one state may exist in a single patient at the same time.


       1.  Vesicular stage: There is a thin vesicular wall and a viable scolex in the middle. As seen in the aforementioned case, a visible scolex in the center of a hole produces the “hole with dot” sign. There is minimal if any enhancement with the addition of contrast. Lesions are most common at the gray–white junction. Images 9.10A–9.10F are from the vesicular stage.


       2.  Colloidal stage: There is a thick vesicular wall with a degenerating scolex and an intense inflammatory reaction with edema and marked enhancement of the cyst wall.


       3.  Granular stage: There is a thick vesicular wall and the scolex has degenerated. The edema and enhancement have decreased.


       4.  Calcified stage: The parasite has transformed into calcified nodules. At this stage, the cysts are dead. There will be no edema or enhancement on postcontrast imaging. On CT, the dead parasites will present as small, punctate calcifications.


Images


Images 9.10K and 9.10L: Axial CT images show multiple calcifications consistent with neurocysticercosis in the calcified state.


Images


Images 9.10M–9.10O: Postcontrast T1-weighted, precontrast T1-weighted, and coronal FLAIR images demonstrate innumerable cysts in a patient with neurocysticercosis. On postcontrast imaging the lesions have a “starry-night” appearance, and on other sequences the brain has a “Swiss-cheese” appearance (images courtesy of Dr. Rajan Jain). Image 9.10P: Gross pathology of neurocysticercosis.


Source: Image 9.10P from Andrea Sylvia Winkler. Epilepsy and neurocysticercosis in sub-Saharan Africa. In: Sibat HF, ed. Novel Aspects on Cysticercosis and Neurocysticercosis; 2013. ISBN: 978-953-51-0956-3. doi:10.5772/53289. www.intechopen.com/books/novel-aspects-on-cysticercosis-and-neurocysticercosis/epilepsy-and-neurocysticercosis-in-sub-saharan-africa


 


Images  With a large lesion burden, the brain is said to resemble “Swiss cheese,” with a so-called “starry-night” appearance on postcontrast images.


Images  Cysts can form within the ventricular system as well, most commonly within the fourth ventricle or the subarachnoid space. This pattern is termed racemose (grape-like) neurocysticercosis. This can lead to ventricular obstruction and hydrocephalus. The cysts are isodense to CSF and there may be enhancement of the cyst wall and edema of the adjacent brain tissue. The scolex is typically not visible.


Images  Involvement of the spinal canal and cord may cause a myelopathy. As with the brain, lesions can be either intramedullary or extramedullary, compressing the cord from the outside.


Treatment


Images  Albendazole is used to kill the parasite, and corticosteroids are often used to minimize the inflammatory reaction. Not all patients need treatment as the majority of cysts show spontaneous resolution. Treatment should be initiated only once a patient is medically stable in terms of seizures, edema, and intracranial hypertension. Once the calcified state is reached, no antiparasitic treatment is needed, though patients may still suffer from seizures.


Images


Images 9.10Q and 9.10R: Axial constructive interference in steady state and postcontrast sagittal T2-weighted images demonstrate racemose (“grape-like”) neurocysticercosis (red arrow) of the fourth ventricle. The fourth ventricle is significantly dilated. Images 9.10S and 9.10T: Postcontrast axial and sagittal T1-weighted images demonstrate a ring-enhancing cyst in the fourth ventricle.


Images


Images 9.10U–9.10W: Sagittal and axial T2-weighted images demonstrate multiple cysts of neurocysticercosis within the spinal cord (red arrow) and within the spinal canal (yellow arrow) compressing the spinal cord.


Images  In patients with intraventricular cysts, surgical removal of the cyst may be required. Shunting of the ventricular system may be required to treat hydrocephalus.


References


1.  Garcia HH, Nash TE, Del Brutto OH. Clinical symptoms, diagnosis, and treatment of neurocysticercosis. Lancet Neurol. December 2014;13(12):1202–1215.


2.  Lerner A, Shiroishi MS, Zee CS, Law M, Go JL. Imaging of neurocysticercosis. Neuroimaging Clin N Am. November 2012;22(4):659–676.


3.  Sotelo J. Clinical manifestations, diagnosis, and treatment of neurocysticercosis. Curr Neurol Neurosci Rep. December 2011;11(6):529–535.


4.  Andrea Sylvia Winkler. Epilepsy and neurocysticercosis in sub-Saharan Africa. In: Sibat HF, ed. Novel Aspects on Cysticercosis and Neurocysticercosis; 2013. ISBN: 978-953-51-0956-3. doi:10.5772/53289. http://www.intechopen.com/books/novel-aspects-on-cysticercosis-and-neurocysticercosis/epilepsy-and-neurocysticercosis-in-sub-saharan-africa



 


 


 







9.11


Toxoplasmosis


Case History


A 46-year-old woman with HIV presented with headaches, generalized weakness, and visual disturbances. The patient had been noncompliant with HAART therapy and had a CD4 count of 88. On exam, she was confused, diffusely weak, and had trouble visually identifying common objects.


Diagnosis: Toxoplasmosis


Images


Images 9.11A and 9.11B: Postcontrast axial T1-weighted and FLAIR images demonstrate ring-enhancing lesions with significant edema in the frontal and parietal lobes bilaterally. They demonstrate the “eccentric target” sign characteristic of toxoplasmosis.


Introduction


Images  Toxoplasmosis is caused by the intracellular parasite Toxoplasma gondii. Cysts are ingested through undercooked meat, soil, cat feces, or contaminated water. It is a common parasite, though only symptomatic in immunocompromised patients. In some areas, over 95% of people have shown evidence of infection, and over 60 million people in the United States are infected.


Images  Cats are its definitive hosts, and humans can be infected by consuming food contaminated with cat feces or eating undercooked meat of animals with tissue cysts. It can also be transmitted transplacentally, from the mother to her fetus.


Images  CNS toxoplasmosis is seen most commonly in late-stage AIDS (CD4 < 200) or other immunosuppressed patients. In patients with AIDS, toxoplasmosis is the most frequent cause of an intracranial mass lesion.


Clinical Presentation


Images  Patients present with fever, decreased mental status, focal neurological deficits, headaches, or seizures. Cysts can also form in skeletal and heart muscle and in the eyes.


Radiographic Appearance and Diagnosis


Images  The lesions are hyperintense on T2-weighted images, often with a large amount of mass effect and edema. They enhance with the administration of contrast in a solid or ring-shaped pattern. The lesions often have an “eccentric target” appearance.


Images  The majority of effected AIDS patients have multiple lesions, most commonly in the basal ganglia, thalami, or gray–white junction.


Images  Distinguishing between toxoplasmosis and primary CNS lymphoma (PCNSL) is difficult. Both present with multiple masses that may have solid or ring-like enhancement. CT scans can be helpful in differentiating toxoplasmosis from PCNSL. The lesions of toxoplasmosis are typically hypodense, while lymphomas are typically hyperdense due to increased cellularity.


Images


Images 9.11C and 9.11D: Postcontrast axial T1-weighted and FLAIR images demonstrate a ring-enhancing lesion in the right basal ganglia with significant edema and resultant mass effect. Image 9.11E: Axial CT image demonstrates hypodensity in the area of the lesion. This hypodensity on CT supports the diagnosis of toxoplasmosis.


Images


Image 9.11F: Histology of a pseudocyst containing bradyzoites (image credit Yale Rosen).


Images  Toxoplasma IgG should be sent when evaluating any intracranial mass in an HIV positive patient. In patients who test negative for toxoplasma IgG antibodies, the diagnosis of PCNSL is favored, though toxoplasmosis is not ruled out. In patients who test positive for toxoplasma IgG antibodies, both toxoplasmosis and PCNSL are possibilities.


Treatment


Images  Patients with suspected toxoplasmosis should receive empiric treatment with trimethoprim–sulfamethoxazole (TMP–SMX) and folinic acid. Treatment should continue for 6 weeks or until there is no enhancement on MRI. Over 90% of patients with toxoplasmosis respond to treatment. If the lesions do not diminish on repeat imaging within 1 to 2 weeks, a brain biopsy to evaluate for PCNSL is indicated.


Pretreatment Images


Images


Images 9.11G–9.11I: Postcontrast axial T1-weighted images demonstrate ring-enhancing lesions in the right basal ganglia, right frontal lobe, and in the pons. Images 9.11J–9.11L: Axial FLAIR images demonstrate significant edema and mass effect.


Posttreatment Images


Images


Images 9.11M–9.11O: Postcontrast axial T1-weighted images demonstrate decreased enhancement and size of the lesion after treatment.


Images


Images 9.11P–9.11R: Axial FLAIR images demonstrate marked decline in edema and mass effect.


References


1.  Saadatnia G, Golkar M. A review on human toxoplasmosis. Scand J Infect Dis. November 2012;44(11):805–814.


2.  Masamed R, Meleis A, Lee EW, Hathout GM. Cerebral toxoplasmosis: case review and description of a new imaging sign. Clin Radiol. May 2009;64(5):560–563.


3.  Ramsey RG, Gean AD. Neuroimaging of AIDS. I. Central nervous system toxoplasmosis. Neuroimaging Clin N Am. May 1997;7(2):171–186.


4.  Offiah CE, Turnbull IW. The imaging appearances of intracranial CNS infections in adult HIV and AIDS patients. Clin Radiol. May 2006;61(5):393–401.



 


 


 







9.12


Aspergillosis


Case History


A 45-year-old man with untreated HIV infection presented after a seizure. His friend reported 4 weeks of cognitive slowing and generalized malaise.


Diagnosis: Aspergillosis


Images


Images 9.12A and 9.12B: Postcontrast axial T1-weighted images demonstrate enhancing lesions (red arrows) in the left occipital and right frontal lobes. Images 9.12C and 9.12D: Axial FLAIR images demonstrate significant edema associated with the lesions.


Introduction


Images  Aspergillosis is an infection caused by Aspergillus, a ubiquitous fungus. CNS infection can cause a necrotizing vasculitis, which presents with hemorrhagic abscesses in the ependymal region.


Clinical Presentation


Images  It most commonly presents with pulmonary symptoms, such as cough or dyspnea. In the CNS, the most common features are cognitive changes, focal neurological deficits, and seizures. The symptom onset is usually dramatic and rapidly progressive. Affected patients are typically immunocompromised.


Radiographic Appearance and Diagnosis


Images  Lesions are commonly seen in the cerebral hemispheres, thalami, basal ganglia, and corpus callosum. The lesions are hypointense on T2-weighted images, although there may be hyperintense edema surrounding the lesions. There is frequently minimal or no contrast enhancement or edema, due to the immunosuppressed status of the patient. Enhancement is more prominent in immunocompetent patients and is often ring-shaped.


Images  Hemorrhage is seen in about 25% to 50% of patients, and this is best visualized on CT scans.


Images


Images 9.12E and 9.12F: Axial CT scans demonstrate small areas of hemorrhage (red arrows) in hypodense lesions.


Images  It can also cause a necrotizing vasculitis, which can lead to subsequent infarction.


Images  The distinction between this fungal infection and metastatic disease may be difficult, but the combination of punctate calcification and ring-enhancing lesions favors infections.


Images  The diagnosis is confirmed via a biopsy, showing the characteristic Aspergillus hyphae branching at a 45 degree angle. As many patients with suspected aspergillosis are too ill to tolerate such an invasive procedure, detecting Aspergillus via PCR in the CSF is highly suggestive.


Images


Images 9.12G–9.12I: Axial DWIs demonstrate multiple infarctions in the brainstem, thalami, and basal ganglia bilaterally in a patient with aspergillosis.


Images


Image 9.12J: Luxol fast blue hematoxylin and eosin (H&E) stain shows Aspergillus hyphae invading a blood vessel wall (red arrows). Image 9.12K: Grocott methenamine silver stain demonstrates the characteristic branching at a 45 degree angle.


Treatment


Images  Voriconazole is more effective than amphotericin B in the treatment of invasive aspergillosis, though only about 35% of patients respond. Without treatment, the mortality rate is near 100%.


References


1.  DeLone DR, Goldstein RA, Petermann G, et al. Disseminated aspergillosis involving the brain: distribution and imaging characteristics. AJNR Am J Neuroradiol. October 1999;20(9):1597–1604.


2.  Yamada K, Shrier DA, Rubio A, et al. Imaging findings in intracranial aspergillosis. Acad Radiol. February 2002;9(2):163–1671.


3.  Ruhnke M, Kofla G, Otto K, Schwartz S. CNS aspergillosis: recognition, diagnosis and management. CNS Drugs. 2007;21(8):659–676.


4.  Reinwald M, Buchheidt D, Hummel M, et al. Diagnostic performance of an Aspergillus-specific nested PCR assay in cerebrospinal fluid samples of immunocompromised patients for detection of central nervous system aspergillosis. PLoS One. 2013;8(2):e56706.



 


 


 







9.13


Creutzfeldt–Jakob Disease


Case History


A 65-year-old man presented with rapid-onset dementia. On exam, he knew his name, but not his age or the location. He thought he was in his office, though he had not worked in several months. He had a profound startle response to any unexpected noise.


Diagnosis: Creutzfeldt–Jakob Disease


Images


Images 9.13A and 9.13B: Diffusion-weighted and FLAIR axial images demonstrate restricted diffusion and hyperintensity in the caudate, putamen, and thalamus in a patient with CJD. The hyperintensity of the pulvinar and dorsomedial thalamic nuclei of the thalamus (red arrow) creates the “hockey stick” sign.


Introduction


Images  Creutzfeldt–Jakob disease (CJD) is due to a conformational change of a prion protein that leads to a toxic gain of function. It is sporadic 90% of the time and typically occurs in patients over the age of 60. There are familial forms (fatal familial insomnia), infectious forms (mad cow disease or variant CJD), and iatrogenic forms, via infected surgical equipment or corneal transplants. Kuru is a spongiform encephalopathy formerly seen in New Guinea due to consumption of human brain tissue.


Images  It is very rare, affecting only 1 in 1 million people.


Clinical Presentation


Images  It presents as a rapid-onset dementia with dramatic personality changes and psychiatric symptoms usually over the course of 3 to 4 months. It is associated with startle myoclonus, ataxia, weakness, and sensory deficits.


Diagnosis and Radiographic Appearance


Images  As seen in Images 9.13A and 9.13B, hyperintensity on T2-weighted images of the pulvinar and dorsomedial thalamic nuclei creates the “hockey stick” sign. The “pulvinar” sign refers to bilateral hyperintensities of the pulvinar nuclei of the thalamus. Hyperintensity and diffusion restriction of the cortical ribbon are also common. This is an early sign and initially asymmetric. In more advanced disease, the white matter will be affected as well.


Images  The CSF may show 14-3-3 protein, though this is not specific for CJD. The EEG may show periodic, generalized, high-amplitude sharp waves.


Images


Images 9.13C–9.13E: Axial DWIs demonstrate restricted diffusion in the cortex of a patient with CJD. Images 9.13F–9.13H: Axial FLAIR images demonstrate diffuse hyperintensity of the cortex (red arrows), mostly on the right, and bilateral hippocampi (yellow arrow).


Images


Image 9.13I: H&E stain showing spongiform changes in the cortex and loss of neurons in a case of variant CJD (image credit Sherif Zaki, MD, PhD; Wun-Ju Shieh, MD, PhD, MPH).


Images  Neuronal death leads to a spongiform appearance of the brain on pathological examination.


Treatment


Images  There is no treatment, and it is universally fatal within a year.


References


1.  Vitali P, Maccagnano E, Geschwind MD, et al. Diffusion-weighted MRI hyperintensity patterns differentiate CJD from other rapid dementias. Neurology. 2011;76:1711–1719.


2.  Lee J, Hyeon JW, Kim SY, Hwang KJ, Ju YR, Ryou C. Review: Laboratory diagnosis and surveillance of Creutzfeldt-Jakob disease. J Med Virol. January 2015;87(1):175–186.


3.  Wang LH, Bucelli RC, Patrick E, et al. Role of magnetic resonance imaging, cerebrospinal fluid, and electroencephalogram in diagnosis of sporadic Creutzfeldt-Jakob disease. J Neurol. February 2013;260(2):498–506.

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Apr 19, 2018 | Posted by in NEUROLOGY | Comments Off on Infectious Diseases

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