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Postinfectious Cerebellitis: An Unusual Presentation of Acute Brigid Hallinan DO Lehigh Valley Health Network

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Published In/Presented At Hallinan, B. (2010). Postinfectious Cerebellitis: An Unusual Presentation of Acute Infectious Mononucleosis. LVHN Scholarly Works. Retrieved from http://scholarlyworks.lvhn.org/medicine/73

This Poster is brought to you for free and open access by LVHN Scholarly Works. It has been accepted for inclusion in LVHN Scholarly Works by an authorized administrator. For more information, please contact [email protected]. Postinfectious Cerebellitis: An Unusual Presentation of Acute Infectious Mononucleosis Brigid Hallinan, DO • Internal Medicine Residency Program • Lehigh Valley Health Network • Allentown, Pennsylvania

TABLE 1.8 TABLE 2 TABLE 41 Introduction Clinical Course express both positive serum EBV VCA IgM and IgG, EBV Epstein-Barr (EBV), the causative agent of Based on the above history, physical exam, and consistent with acute EBV . Acute Neurologic Microbiologic Agents Associated with Complications of Heterophile Positive infectious mononucleosis, has been linked to the diagnostic studies, our patient was diagnosed CT and MRI are classically normal. There Postinfectious Cerebellitis Epstein-Barr Virus VCM IgM antibody Positive Legionella secondary development of several neurologic with postinfectious cerebellitis secondary to is suggestion that abnormalities in cerebellar Infection VCA IgG antibody Positive Epstein-Barr virus Neisseria meningitidis disorders (Table 1). Here, I present the case of an acute infectious mononucleosis. She was treated enhancement may be seen on MRI prior to the onset EA antibody Positive Varicella-zoster virus Salmonella typhi 18-year-old female with acute cerebellar dysfunction supportively but remained symptomatic over the next of neurologic symptoms with near normalization by Cerebellitis EBNA antibody Negative Enterovirus Bordatella pertussis consistent with postinfectious cerebellitis secondary eight days with no significant change in her clinical the time the patient is symptomatic (Figures 1 & 2).4 Cranial neuritis Parvovirus pyogenes Transverse to a previously undiagnosed EBV infection. picture. On the ninth day of her illness, she was Thus, it may be possible to detect mild cerebellar virus Corynebacterium diphtheriae Peripheral neuritis TABLE 3 discharged home in stable condition with neurology changes on MRI if obtained very early in the course of A virus Autonomic neuropathy Cerebrospinal fluid analysis Parasites follow-up. She experienced complete symptom the illness; however, this is not thought to be typical. Human herpes virus 6 Guillan-Barre syndrome Plasmodium falciparum Case Presentation resolution within four weeks. perfusion single photon emission photography RBC (/cmm) 0 Alice in Wonderland syndrome History (SPECT) examination using 99mTc-HMPAO, on the WBC (/cmm) 4 Bacteria Varicella-zoster virus An 18-year-old Caucasian female presented to other hand, may be useful during the symptomatic Protein (mg/dL) 69 Borrelia burgdorferi Hepatitis Discussion Glucose (mg/dL) 28 the emergency department with a two-day history phase, demonstrating cerebellar hyperperfusion Coxiella burnetti virus of vertigo, slurred speech, ataxia, poor hand-eye Introduction (Figure 3).5 EBV by PCR (copies/mL) <200 (negative) Mycoplasma pneumonia coordination, and impaired depth perception. The Postinfectious cerebellitis (PIC) is a rare, immune- The CSF cell count is frequently normal, with a CMV by PCR (copies/mL) <500 (negative) patient mentioned that a few weeks earlier she mediated of Epstein-Barr virus, mild lymphocytic pleocytosis seen in only 25-50% VZV by PCR (copies/mL) <100 (negative) had experienced a prolonged upper respiratory characterized by rapid development of cerebellar of patients.1 CSF protein may be normal or slightly Oligoclonal bands No bands tract infection with , , and nasal dysfunction. The condition has been linked to several elevated. No direct evidence of CNS infection is found

congestion but denied any other significant past other microbiologic agents and some vaccinations as on CSF analysis by cultures or PCR. Figure 1.4 Figure 2.4 Figure 3.2 medical history. well (Table 4). Prognosis Physical Exam Pathogenesis PIC is usually self-limited, with the majority of patients was remarkable for posterior PIC is believed to be an immune-mediated condition, experiencing complete resolution of symptoms within cervical , palpable , as neurologic symptoms frequently follow resolution 1-4 weeks. However, 10-30% of patients experience mild ataxia, and difficulty with tandem gait to either of the infectious . The inability to detect persistent cerebellar dysfunction manifested by side. Impaired depth perception was demonstrated the presence of infection in the cerebrospinal fluid dysarthria or ataxia.1 In severe cases, cerebellar when she walked into a wall. supports this theory. To date, there have been several edema may result in death by transtentorial or 1 4 Diagnostic Data autoantibodies isolated in patients with PIC. transforaminal herniation. Initial laboratory studies revealed a normal white Clinical features Treatment cell count with lymphocytic predominance Patients may present with sudden onset of ataxia, Treatment is primarily supportive, as most cases are and mild transaminitis. A later CBC with differential dysarthria, saccadic eye movements, dysmetria, self-limited. However, in rare cases with significant revealed the presence of atypical . vertigo, , and .2 Some patients exhibit disability or a prolonged course, total plasma T1-weighted MRI image demonstrating T2-weighted MRI image demonstrating a Axial single photon emission tomographic sections through the cerebellum (A) hypointensity of the cerebellar grey and hyperintense signal in the cerebellum.4 and cerebral hemispheres (B). Increased cerebellar perfusion is demonstrated 6 CBC and CMP were otherwise unremarkable. the cerebellar cognitive affective syndrome, which exchange or IV Ig may hasten recovery. Steroids white matter.4 by high photon flux in the superficial regions (arrow heads).2 Epstein-Barr virus serology was consistent with is characterized by cerebellar dysfunction, cognitive may be beneficial in moderate to severe cases, 3 an acute EBV infection (Table 2). HIV by ELISA impairment and affective changes. Cerebellar however, direct viral invasion should be ruled out References was negative. drug screen was negative. symptoms tend to appear within four weeks of the 1. Chong HT, Tan CT. Post-viral cerebellitis. In: Lisak RP, Truong DD, Carroll WM, Bhidayasiri R, eds. International Neurology: A Clinical Approach. Oxford, UK: Wiley-Blackwell; 2009:329-330. by negative CSF PCR prior to the initiation of any 2. Gruis KL, Moretti P, Gebarski SS, Mikol D. Cerebellitis in an adult with abnormal magnetic resonance imaging findings prior to the onset of ataxia. Arch Neurol. 2003;60:877-880. Cerebrospinal fluid analysis (Table 3) revealed no onset of the preceding infection. In cases of EBV- immunosuppressive treatment.7 3. Rosinski A, Goldman M, Cameron O. A case of cerebellar psychopathology. Psychosomatics. 2010;51:171-175. 4. Gamangatti S, Nayaz Z. A child with cerebellar ataxia. Br J Radiol. 2008;81(961):82-84. abnormalities, including negative EBV by PCR. associated PIC, the preceding infection may go 5. Daaboul Y, Vern BA, Blend MJ, Brain SPECT imaging and treatment with IV Ig in acute postinfectious cerebellar ataxia: case report. Neurol Res. 1998;20(1):85-88. Conclusion 6. Schmahmann JD. Plasmapheresis improves outcome in postinfectious cerebellitis induced by Epstein-Barr virus. Neurology. 2004;62(8):1443-1445. Neuroimaging was unremarkable, including CT of unrecognized or the patient may only recall a recent 7. Lierde AV, Righini A, Tremolati E. Acute cerebellitis with tonsillar herniation and hydrocephalus in Epstein-Barr virus infection. Eur J Pediatr. 2004;163:689-691. Clinicians should be aware of this condition so that 8. Jenson HB. Acute complications of Epstein-Barr virus infectious mononucleosis. Curr Opin Pediatr. 2000;12(3):263-268. the head without contrast, MRI of the brain with and viral-like illness, as was the case with our patient. they may avoid unnecessary diagnostic testing, provide without contrast, and MRA of the head and neck Diagnosis appropriate medical treatment when intervention is without contrast. An abdominal ultrasound confirmed Patients with PIC due to Epstein-Barr virus will warranted, and prevent erroneous diagnoses that may mild splenomegaly. typically have a positive heterophile antibody test and be attached to a poor prognosis or negative stigma.

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