Acute Cerebellar Ataxia Associated with Varicella Moshe Nussinovitch, MD; Galia Soen, MD; Benjamin Volovitz, MD; and Itzhak Varsano, MD Petach Tikvah, Israel

Brief Report

Between 1987 and 1990, seven children presented residual signs or symptoms during a 2- to 3-year fol with acute cerebellar symptoms and serologic evi low-up. dence suggestive of recent varicella infection. The neurological symptoms lasted for 1 to 3 weeks. Symp Key words. Chickenpox; cerebellar ataxia; child; disease. toms resolved spontaneously in all patients without (JFam Pract 1995; 40:494-496)

Neurological complications are relatively rare in chicken- nial nerves, sensory findings, muscle strength, and muscle pox.1-3 Acute cerebellar ataxia is the most common, oc tone were all normal. None of the patients had myoclonus curring in about one in 4000 varicella cases among chil or opsoclonus. Cerebellar symptoms lasted between 7 dren younger than 15 years of age.1 and 20 days, resolving spontaneously in all patients. No Acute varicella-associated cerebellar ataxia occurs residual neurological effects were observed during a 2- to primarily in children and is characterized by ataxia with 3-year follow-up. At the time of evaluation, all seven pa nystagmus, headache, nausea, vomiting, and nuchal rigid tients had serologic evidence of recent varicella infection, ity. The symptoms of varicella encephalitis may begin as as indicated by the results of an enzyme-linked immu early as 11 days before and up to 21 days after the onset of nosorbent assay for IgM antibodies. All patients exhibited the rash.4 at least a fourfold increase in complement fixation anti We describe seven children with acute cerebellar bodies (Table). ataxia associated with varicella virus infection and review Lumbar puncture performed in all patients revealed the pertinent literature. pleocytosis (9 to 28 white blood cells [WBC]/mm3)with lymphocytic predominance. The protein and glucose determinations on all cerebrospinal fluids (CSF) were Case Reports normal. Patient 4 (Table) presented with an unusual case of Between 1987 and 1990, seven children presented with varicella. He was brought to the hospital because ofvom- acute cerebellar ataxia. In one of the patients, ataxia pre iting, lethargy, slurred speech, and difficulty in walking. ceded the development of varicella by 10 days. In six The patient had been in excellent health until 2 days patients, the ataxia occurred 12 to 21 days after the onset before admission. His brother had had varicella 2 weeks of the varicella rash. Patient ages ranged from 2 to 6 years previously. The physical examination at admission re (Table). vealed an irritable but cooperative child. His neck was Cerebellar symptoms consisted of truncal ataxia, in supple. Brudzinski’s and Kernig’s signs were negative. A tention tremor, and abnormal results on finger-to-nose neurological examination revealed no abnormality' of cra and heel-to-shin tests. There was no clinical evidence of nial nerves except for nystagmus upon lateral gaze to meningeal irritation, and mental status was normal. Cra- either side. Finger-to-nose and heel-to-shin tests, as well as rapid Submitted, revised, December 20, 1994. alternating movements, suggested severe bilateral cere From the Department of Pediatrics “C, ” Children }s Medical Center of Israel, Petach bellar dysfunction. There seemed to be a decrease in tone Tikvah, Israel. Requests for reprints should be addressed to Moshe Nussinovitch, MD, in all four extremities. Deep tendon reflexes were pendu Department of Pediatrics “C, ” Children’s Medical Center o f Israel, Petach Tikvah, Israel. lar in quality but symmetrical in both the upper and lower

Table. Patients Experiencing Cerebellar Ataxia As a Complication of Varicella Infection (N =7)

CSF Examination Days from Appearance Duration of Convalescent Patient N o./ Protein, of Rash to Onset Cerebellar Titer for Age (y)/Sex W BC/m m 3 m g/dL of Ataxia Signs, d VZV by CF* 1 /6 /F 9 11 12 7 1:128 2 /2 /M 7 17 14 8 1:256 3 /5 /M 12 25 12 14 1:256 4 / 2 /M | 24 18 10 20 1:128 5 /6 /M 28 20 21 14 1:256 6 /4 /F 12 10 14 10 1:128 7 /3 /M 10 10 12 8 1:128 *Fourfold rise from acute-phase titer, f Patient with pre-emptive acute cerebellar ataxia. CSF denotes cerebrospinal fluid; WRC, white blood count; VZV, varicella-zoster virus; CF, complement fixation test. extremities. Fundi were normal. The rest of the physical ing from 0 to 68 cells/mm3. Lymphocytic pleocytosis and and neurological examination was normal. A lumbar elevated protein may occur in 20% to 30% of the cases. puncture performed at admission revealed clean, colorless The CSF glucose is normal, and polymorphonuclear cells spinal fluid under normal pressure, containing 24 W BC/ may be present in the early course of the disease.2’7’8 In mm3, of which 75% were lymphocytes. The protein con complicated cases, an electroencephalogram (EEC), a tent was 18 mg/dL (0.18 g/L) (Table), and glucose computed tomography (CT) scan, and on some occa content was 80 m g/dL (0.8 g/L). sions, magnetic resonance imaging (MRI) are necessary. Ten days after admission to the hospital, vesicles de On the EEC, slow, diffuse wave activity has been noted in veloped on the patient’s arms, legs, and trunk. He began 20% of the cases8; CT is usually normal, although MRI to improve neurologically as soon as the varicella skin studies have revealed abnormalities, even in benign cases. lesions appeared. He became less lethargic and his ataxia In mild cases, no further evaluation is recommended.8 diminished. A serum specimen drawn at admission had The neurological complications of varicella that pre shown a varicella titer of 1:16; the titer was 1:128 on the cede or accompany the exanthematous manifestation are day of his discharge 2 weeks later. At a follow-up exami likely to be directly related to viral invasion of the nervous nation 2 months later, he showed full neurological recov system. On the other hand, neurological complications ery. There was no serologic evidence of recent mumps, that occur after the appearance of cutaneous lesions are echovirus, coxsackievirus B, measles, rubella, or poliomy most likely the result of a nonspecific, postinfectious au elitis. toimmune process.6’9 Although pre-eruptive neurological complications may occur, cerebellar symptoms are re ported to precede the skin rash only in very rare cases. It is Discussion worth noting that most of these pre-eruptive patients Acute cerebellar ataxia is a relatively common neurologi were male and that the interval between the onset and the cal disorder among children. Its association with a large appearance of the eruption was relatively long (more than variety of infectious agents has been described. These 10 days).9-11 include varicella, echovirus type 9, Epstein-Barr virus, Peters et al2 were the first to demonstrate the pres coxsackievirus B, poliomyelitis, measles, mumps and ru ence of varicella antigen in CSF cells by means of an bella, and mycoplasma pneumonias. Of these, varicella immunofluorescent technique, suggesting direct invasion appears to be the pathogen most commonly identified.5"6 of the central nervous system in patients with acute cere Cerebellar ataxia accounts for more than one half of bellar ataxia. Antibodies to VZV also have been demon all the neurological complications of varicella-zoster virus strated in the CSF of patients with varicella.8 (VZV) infection.7 Acute varicella-associated cerebellar The cerebellar manifestations of varicella are usually ataxia occurs primarily in children and is characterized by self-limited, mortality is low, and the majority of patients ataxia with nystagmus, headache, nausea, vomiting, and recover without sequelae.8 It is a benign complication of nuchal rigidity.4'7 The symptoms of varicella encephalitis VZV infection in children and does not generally require may begin as early as 11 days before and up to 21 days hospitalization. No specific therapy, eg, acyclovir, is indi after the onset of the rash.2’7 cated at this time for patients with central nervous system Examination of CSF in varicella-associated cerebellar involvement caused by VZV.8-10 In some patients with ataxia reveals nonspecific findings, with a cell count rang pre-eruptive varicella cerebellitis, polymerase chain reac-

The Journal of Family Practice, Vol. 40, No. 5(May), 1995 495 Varicella Cerebellar Ataxia Nussinovitch, Socn, Volovitz, and Varsano

tion may be extremely useful for rapid confirmation of the 4. Straus SE, Ostrove JM, Inchauspe G, Felser JM, Freifeld A, Croat diagnosis.10 KD, Sawyer MH. Varicella-zoster virus infections: biology, natural history, treatment and prevention. Ann Intern Med 1988- 108' The cerebellar symptoms in our patients were fol 221-37. lowed by complete neurological recovery. No residual 5. Bell WE. Ataxia in childhood— clinical approach and differential neurological complications were noted during a 2- to diagnosis. Lancet 1965; 2:85. 3-year follow-up, suggesting that acute varicella-associ 6. Dreyfus PM, Senter TP. Acute cerebellar ataxia of childhood: an unusual case of varicella. West J Med 1974; 120:161-3. ated cerebellar ataxia follows a benign, self-limited course. 7. Johnson R, Milbourn PE. Central nervous system manifestations of chickenpox. Can Med Assoc J 1970; 102:831-4. 8. Scheld WM, Whitley RJ, Durack DT. Infections of the central nervous system. New York, NY: Raven Press, 1991:59. References 9. Goldston AS, Millichap JG, Miller RH. Cerebellar ataxia with pre 1. Guess HA, Broughton DD, Melton LJ, Kurland LT. Population- emptive varicella. Am J Dis Child 1963; 106:197-200. based studies of varicella complications. Pediatrics 1986; 78:723-7. 10. Dangond F, Engle E, Yessayan L, Sawyer MH. Pre-eriiptivt 2. Peters AC, Versteeg J, Lindeman J, Bots GT. Varicella and acute varicella cerebellitis confirmed by PCR. Pediatr Neurol 1993; cerebellar ataxia. Arch Neurol 1978; 35:769-71. 9:491-3. 3. Jackson MA, Burry VJ, Olson LC. Complications of varicella re 11. Takeuchi Y, Yoshihara T, Ishimuru K, Amano T, ICadomoto Y, quiring hospitalization in previously healthy children. Pediatr Infect Sawada T. Recurrent pre-eruptive acute cerebellar ataxia—a rare Dis 1992; 11:441-5. case of varicella. Pediatr Neurol 1987; 3:240-1.

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