Acute ataxia in a 4-year-old boy: a case of Lyme disease neuroborreliosis

Mark D Lopez, Cristin Wise

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

We present a case of a 4-year-old who presented to the emergency department with an unsteady gait for 2 days. Ataxia is a rare but known manifestation of cerebellar involvement in Lyme disease. A 4-year-old (17 kg) boy with no significant medical history presented to the emergency department (ED) with history of nonbloody emesis for 2 weeks and an unsteady gait for 2 days. Over the past 2 days, his gait had gotten progressively worse until he was unable to walk without assistance. The vomiting would usually occur 1 hour after eating meals. He had also complained of a single headache, which occurred approximately 10 days before admission. The headache did not occur in the early morning hours or wake him up from his sleep. His appetite for the weeks before admission had progressively decreased, and he had also become more irritable, especially when stimulated. He had increased fatigue for the week before presentation. His parents denied any fever, rhinorrhea, cough, diarrhea, rash, bruising, bleeding, or hematuria. The patient denied any abdominal pain or headache while in the ED. On arrival to the pediatric ED, the patient was awake but lying down in bed and in no acute distress. Vitals signs were temperature of 37.7°C, heart rate of 117 beats/min, respiratory rate of 24/min, blood pressure of 95/63, and oxygen saturation of 98% on room air. Physical examination was normal except for his ataxic gait. He was unable to sit up on the bed alone without falling to the side. When walking down the hallway, the patient stumbled to one side and almost ran into the wall before losing his balance. Complete blood count and serum chemistries were normal. Because of concern for an intracranial mass, a computed tomography of the head without contrast was ordered and was negative. Neurology was consulted, and it was their impression that the patient had either ataxia or diffuse weakness. They recommended the patient be admitted overnight for further workup. On admission to the inpatient ward, the patient had orthostatic blood pressures done, which were normal. A lumbar puncture was done the evening of admission, which showed colorless, clear fluid, no xanthochromia, 35 red blood cells, 93 white blood cells with 20% segs, 45% lymphs, 33% monos, 2% eosinophils. Cerebrospinal fluid (CSF) glucose was 62 mg/dL, and CSF protein was 22 mg/dL. C-reactive protein was 0.297 mg/dL, ESR 33 mm/h. The morning after admission, the patient was noted to have 3+ reflexes in the lower extremities, emotional lability, bilateral ankle clonus (left greater than right), and a positive Brudzinski and Kernig sign. On day 2 of hospitalization, neurology ordered a magnetic resonance imaging (MRI) of the brain with and without contrast. The MRI showed multifocal areas of subcortical white matter hyperintensity consistent with acute disseminated encephalitis. The patient was started on high-dose prednisone at 20 mg/kg, intravenously, for 3 days with 2 days of oral prednisone after completing the intravenous steroids. He was also started on azithromycin, gentamicin, and acyclovir, which were discontinued after negative blood cultures at 48 hours. The patient's gait improved shortly after steroid therapy was initiated. On hospital day number 3, serum was sent for Lyme disease, Bartonella, Epstein-Barr virus, Leptospira, measles, mumps, mycoplasma, Cryptococcus, and comprehensive viral and fungal culture. Cerebrospinal fluid herpes simplex virus and enteroviral polymerase chain reaction were negative. His gait continued to improve and was completely back to baseline after completing 3 days of intravenous steroids and 2 days of oral prednisone. Thus, the patient was discharge after 5 days of treatment and scheduled for a follow-up MRI study. One day after discharge, the patient's Lyme enzyme-linked immunosorbent assay came back positive and was confirmed with Western blot. Thus, the patient was readmitted to have a central line placement to receive intravenous Rocephin for a duration of 21 to 28 days. He was discharged the second day of his second admission and subsequently completed a full course of intravenous Rocephin. He had no repeated episodes of ataxia. Cerebellar involvement in Lyme disease is a known but rare manifestation of the illness with very few reports in the literature in children. More common causes of acute cerebellar ataxia include immune-mediated postviral infections, labyrinthitis, or cerebellar tumor [1]. A broad range of Lyme disease-associated neurologic disorders have been described in children. These include cranial neuropathies, headache, seizures, meningitis, meningoencephalitis, encephalopathy, focal neurologic signs, ataxia, vertigo, chorea, and transverse myelitis [2]. The most frequent of which are facial nerve palsy and meningitis [3]. Lyme disease occurs in the northeast of the United States (from Maine to Maryland), in the Midwest (Wisconsin and Minnesota), and on the west coast (northern California and Oregon) [4]. It is the most frequent vector-borne disease of the northern temperate zone [5]. Lyme borreliosis is an infection caused by spirochetes of the group Borrelia burgdorferi, which is generally transmitted by the symptomless bite of certain ticks of the species Ixodes scapularis. Lyme borreliosis occurs with similar frequencies in men and women and affects people of all ages; however, it affects children and elderly adults more frequently [6]. Birds, several strains of mice, dormice, deer, voles, as well as lizards are the major reservoirs of Borrelia. The tick has a 2-year, 3-stage life cycle (larval, nymphal, and adult) and feeds once during each stage. The tick may become infected at any stage of its life cycle by feeding on a natural reservoir for B burgdorferi. Larvae emerge in the following spring in the nymphal stage. The nymphal stage tick is most likely to transmit the infection, presumably because it is so small that it is difficult to identify the bite and to remove the tick in a timely manner. Moreover, nymphs are prevalent during spring and summer, when people often enter habitats in which ticks thrive. In each of the 3 stages the ticks require a blood meal [4]. Children account for a disproportionate number of Lyme borreliosis cases, presumably because of increased exposure and decreased attention to prevention. Manifestations are similar to those in adults. Lyme borreliosis manifestations in children include erythema migrans (solitary/multiple lesions) in 89%, arthritis in 6%, facial nerve palsy in 3%, meningitis in 2%, and carditis in 0% to 5% in 201 consecutive children with a median age of 7 years (range, 1-21 years) followed in Connecticut [4]. Younger children (<10 years) more frequently have fever with the onset of arthritis, a more acute course, lower antibody titers to B burgdorferi, a shorter arthritis duration after antibiotics are started, and a better 1 year prognosis [5]. Children present more frequently than adults with acute early neuroborreliosis [5]. Neuroborreliosis can manifest as a lymphocytic meningitis, cranial neuritis (particularly peripheral facial nerve palsy), radiculoneuropathy, and rarely, encephalomyelitis. There can also be headache, fatigue, paresthesia, or stiff neck without other objective symptoms of Lyme borreliosis. The symptoms of neuroborreliosis develop within weeks after the onset of erythema migrans. Peripheral neuropathy (chronic axonal neuropathy); paresthesias; less commonly, radicular pain; encephalopathy (typically subacute or chronic, subtle memory and cognitive dysfunction); and encephalomyelitis (unifocal or multifocal inflammatory disease) are neurological manifestations [4]. Patients with B burgdorferi infection have a unique multi isotype antibody response in the CSF not seen in other neurologic diseases. They demonstrate intrathecal IgM, IgG, and IgA antibody responses to the spirochete [1]. Definitive diagnosis requires CSF assessment for intrathecal antibody production or demonstration of organisms by culture, histopathology, or polymerase chain reaction [7]. Acute neuroborreliosis may resolve without antibiotic therapy; however, the duration of neurologic symptoms is longer, and the risk for sequelae is greater [5,8]. For patients with evidence of neurologic abnormalities, a 2- to 4-week course of intravenous ceftriaxone (75-100 mg/kg or 2 g/d) is most commonly administered. Intravenous therapies with cefotaxime (150-200 mg/kg or 6 g/d) or penicillin G (200-400 000 U/kg per day or 18-24 MU/day) are alternatives [2,5,7]. The signs and symptoms of acute neuroborreliosis usually resolve within weeks, but those of chronic neuroborreliosis improve slowly over a period of months [4]. Although neuroborreliosis is a rare presentation of Lyme disease, it is a more likely manifestation in a child with the disease. We recommend the emergency physicians that treat children in endemic areas become familiar with this unusual presentation of a common illness.

Original languageEnglish (US)
Pages (from-to)1069.e5-1069.e6
JournalAmerican Journal of Emergency Medicine
Volume26
Issue number9
DOIs
StatePublished - Jan 1 2008

Fingerprint

Lyme Neuroborreliosis
Lyme Disease
Ataxia
Ticks
Headache
Gait
Meningitis
Cerebrospinal Fluid
Ceftriaxone
Hospital Emergency Service
Facial Paralysis
Facial Nerve
Neurologic Manifestations
Prednisone
Neurologic Gait Disorders
Arthritis
Antibody Formation
Encephalomyelitis
Paresthesia
Patient Discharge

ASJC Scopus subject areas

  • Emergency Medicine

Cite this

Acute ataxia in a 4-year-old boy : a case of Lyme disease neuroborreliosis. / Lopez, Mark D; Wise, Cristin.

In: American Journal of Emergency Medicine, Vol. 26, No. 9, 01.01.2008, p. 1069.e5-1069.e6.

Research output: Contribution to journalArticle

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title = "Acute ataxia in a 4-year-old boy: a case of Lyme disease neuroborreliosis",
abstract = "We present a case of a 4-year-old who presented to the emergency department with an unsteady gait for 2 days. Ataxia is a rare but known manifestation of cerebellar involvement in Lyme disease. A 4-year-old (17 kg) boy with no significant medical history presented to the emergency department (ED) with history of nonbloody emesis for 2 weeks and an unsteady gait for 2 days. Over the past 2 days, his gait had gotten progressively worse until he was unable to walk without assistance. The vomiting would usually occur 1 hour after eating meals. He had also complained of a single headache, which occurred approximately 10 days before admission. The headache did not occur in the early morning hours or wake him up from his sleep. His appetite for the weeks before admission had progressively decreased, and he had also become more irritable, especially when stimulated. He had increased fatigue for the week before presentation. His parents denied any fever, rhinorrhea, cough, diarrhea, rash, bruising, bleeding, or hematuria. The patient denied any abdominal pain or headache while in the ED. On arrival to the pediatric ED, the patient was awake but lying down in bed and in no acute distress. Vitals signs were temperature of 37.7°C, heart rate of 117 beats/min, respiratory rate of 24/min, blood pressure of 95/63, and oxygen saturation of 98{\%} on room air. Physical examination was normal except for his ataxic gait. He was unable to sit up on the bed alone without falling to the side. When walking down the hallway, the patient stumbled to one side and almost ran into the wall before losing his balance. Complete blood count and serum chemistries were normal. Because of concern for an intracranial mass, a computed tomography of the head without contrast was ordered and was negative. Neurology was consulted, and it was their impression that the patient had either ataxia or diffuse weakness. They recommended the patient be admitted overnight for further workup. On admission to the inpatient ward, the patient had orthostatic blood pressures done, which were normal. A lumbar puncture was done the evening of admission, which showed colorless, clear fluid, no xanthochromia, 35 red blood cells, 93 white blood cells with 20{\%} segs, 45{\%} lymphs, 33{\%} monos, 2{\%} eosinophils. Cerebrospinal fluid (CSF) glucose was 62 mg/dL, and CSF protein was 22 mg/dL. C-reactive protein was 0.297 mg/dL, ESR 33 mm/h. The morning after admission, the patient was noted to have 3+ reflexes in the lower extremities, emotional lability, bilateral ankle clonus (left greater than right), and a positive Brudzinski and Kernig sign. On day 2 of hospitalization, neurology ordered a magnetic resonance imaging (MRI) of the brain with and without contrast. The MRI showed multifocal areas of subcortical white matter hyperintensity consistent with acute disseminated encephalitis. The patient was started on high-dose prednisone at 20 mg/kg, intravenously, for 3 days with 2 days of oral prednisone after completing the intravenous steroids. He was also started on azithromycin, gentamicin, and acyclovir, which were discontinued after negative blood cultures at 48 hours. The patient's gait improved shortly after steroid therapy was initiated. On hospital day number 3, serum was sent for Lyme disease, Bartonella, Epstein-Barr virus, Leptospira, measles, mumps, mycoplasma, Cryptococcus, and comprehensive viral and fungal culture. Cerebrospinal fluid herpes simplex virus and enteroviral polymerase chain reaction were negative. His gait continued to improve and was completely back to baseline after completing 3 days of intravenous steroids and 2 days of oral prednisone. Thus, the patient was discharge after 5 days of treatment and scheduled for a follow-up MRI study. One day after discharge, the patient's Lyme enzyme-linked immunosorbent assay came back positive and was confirmed with Western blot. Thus, the patient was readmitted to have a central line placement to receive intravenous Rocephin for a duration of 21 to 28 days. He was discharged the second day of his second admission and subsequently completed a full course of intravenous Rocephin. He had no repeated episodes of ataxia. Cerebellar involvement in Lyme disease is a known but rare manifestation of the illness with very few reports in the literature in children. More common causes of acute cerebellar ataxia include immune-mediated postviral infections, labyrinthitis, or cerebellar tumor [1]. A broad range of Lyme disease-associated neurologic disorders have been described in children. These include cranial neuropathies, headache, seizures, meningitis, meningoencephalitis, encephalopathy, focal neurologic signs, ataxia, vertigo, chorea, and transverse myelitis [2]. The most frequent of which are facial nerve palsy and meningitis [3]. Lyme disease occurs in the northeast of the United States (from Maine to Maryland), in the Midwest (Wisconsin and Minnesota), and on the west coast (northern California and Oregon) [4]. It is the most frequent vector-borne disease of the northern temperate zone [5]. Lyme borreliosis is an infection caused by spirochetes of the group Borrelia burgdorferi, which is generally transmitted by the symptomless bite of certain ticks of the species Ixodes scapularis. Lyme borreliosis occurs with similar frequencies in men and women and affects people of all ages; however, it affects children and elderly adults more frequently [6]. Birds, several strains of mice, dormice, deer, voles, as well as lizards are the major reservoirs of Borrelia. The tick has a 2-year, 3-stage life cycle (larval, nymphal, and adult) and feeds once during each stage. The tick may become infected at any stage of its life cycle by feeding on a natural reservoir for B burgdorferi. Larvae emerge in the following spring in the nymphal stage. The nymphal stage tick is most likely to transmit the infection, presumably because it is so small that it is difficult to identify the bite and to remove the tick in a timely manner. Moreover, nymphs are prevalent during spring and summer, when people often enter habitats in which ticks thrive. In each of the 3 stages the ticks require a blood meal [4]. Children account for a disproportionate number of Lyme borreliosis cases, presumably because of increased exposure and decreased attention to prevention. Manifestations are similar to those in adults. Lyme borreliosis manifestations in children include erythema migrans (solitary/multiple lesions) in 89{\%}, arthritis in 6{\%}, facial nerve palsy in 3{\%}, meningitis in 2{\%}, and carditis in 0{\%} to 5{\%} in 201 consecutive children with a median age of 7 years (range, 1-21 years) followed in Connecticut [4]. Younger children (<10 years) more frequently have fever with the onset of arthritis, a more acute course, lower antibody titers to B burgdorferi, a shorter arthritis duration after antibiotics are started, and a better 1 year prognosis [5]. Children present more frequently than adults with acute early neuroborreliosis [5]. Neuroborreliosis can manifest as a lymphocytic meningitis, cranial neuritis (particularly peripheral facial nerve palsy), radiculoneuropathy, and rarely, encephalomyelitis. There can also be headache, fatigue, paresthesia, or stiff neck without other objective symptoms of Lyme borreliosis. The symptoms of neuroborreliosis develop within weeks after the onset of erythema migrans. Peripheral neuropathy (chronic axonal neuropathy); paresthesias; less commonly, radicular pain; encephalopathy (typically subacute or chronic, subtle memory and cognitive dysfunction); and encephalomyelitis (unifocal or multifocal inflammatory disease) are neurological manifestations [4]. Patients with B burgdorferi infection have a unique multi isotype antibody response in the CSF not seen in other neurologic diseases. They demonstrate intrathecal IgM, IgG, and IgA antibody responses to the spirochete [1]. Definitive diagnosis requires CSF assessment for intrathecal antibody production or demonstration of organisms by culture, histopathology, or polymerase chain reaction [7]. Acute neuroborreliosis may resolve without antibiotic therapy; however, the duration of neurologic symptoms is longer, and the risk for sequelae is greater [5,8]. For patients with evidence of neurologic abnormalities, a 2- to 4-week course of intravenous ceftriaxone (75-100 mg/kg or 2 g/d) is most commonly administered. Intravenous therapies with cefotaxime (150-200 mg/kg or 6 g/d) or penicillin G (200-400 000 U/kg per day or 18-24 MU/day) are alternatives [2,5,7]. The signs and symptoms of acute neuroborreliosis usually resolve within weeks, but those of chronic neuroborreliosis improve slowly over a period of months [4]. Although neuroborreliosis is a rare presentation of Lyme disease, it is a more likely manifestation in a child with the disease. We recommend the emergency physicians that treat children in endemic areas become familiar with this unusual presentation of a common illness.",
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TY - JOUR

T1 - Acute ataxia in a 4-year-old boy

T2 - a case of Lyme disease neuroborreliosis

AU - Lopez, Mark D

AU - Wise, Cristin

PY - 2008/1/1

Y1 - 2008/1/1

N2 - We present a case of a 4-year-old who presented to the emergency department with an unsteady gait for 2 days. Ataxia is a rare but known manifestation of cerebellar involvement in Lyme disease. A 4-year-old (17 kg) boy with no significant medical history presented to the emergency department (ED) with history of nonbloody emesis for 2 weeks and an unsteady gait for 2 days. Over the past 2 days, his gait had gotten progressively worse until he was unable to walk without assistance. The vomiting would usually occur 1 hour after eating meals. He had also complained of a single headache, which occurred approximately 10 days before admission. The headache did not occur in the early morning hours or wake him up from his sleep. His appetite for the weeks before admission had progressively decreased, and he had also become more irritable, especially when stimulated. He had increased fatigue for the week before presentation. His parents denied any fever, rhinorrhea, cough, diarrhea, rash, bruising, bleeding, or hematuria. The patient denied any abdominal pain or headache while in the ED. On arrival to the pediatric ED, the patient was awake but lying down in bed and in no acute distress. Vitals signs were temperature of 37.7°C, heart rate of 117 beats/min, respiratory rate of 24/min, blood pressure of 95/63, and oxygen saturation of 98% on room air. Physical examination was normal except for his ataxic gait. He was unable to sit up on the bed alone without falling to the side. When walking down the hallway, the patient stumbled to one side and almost ran into the wall before losing his balance. Complete blood count and serum chemistries were normal. Because of concern for an intracranial mass, a computed tomography of the head without contrast was ordered and was negative. Neurology was consulted, and it was their impression that the patient had either ataxia or diffuse weakness. They recommended the patient be admitted overnight for further workup. On admission to the inpatient ward, the patient had orthostatic blood pressures done, which were normal. A lumbar puncture was done the evening of admission, which showed colorless, clear fluid, no xanthochromia, 35 red blood cells, 93 white blood cells with 20% segs, 45% lymphs, 33% monos, 2% eosinophils. Cerebrospinal fluid (CSF) glucose was 62 mg/dL, and CSF protein was 22 mg/dL. C-reactive protein was 0.297 mg/dL, ESR 33 mm/h. The morning after admission, the patient was noted to have 3+ reflexes in the lower extremities, emotional lability, bilateral ankle clonus (left greater than right), and a positive Brudzinski and Kernig sign. On day 2 of hospitalization, neurology ordered a magnetic resonance imaging (MRI) of the brain with and without contrast. The MRI showed multifocal areas of subcortical white matter hyperintensity consistent with acute disseminated encephalitis. The patient was started on high-dose prednisone at 20 mg/kg, intravenously, for 3 days with 2 days of oral prednisone after completing the intravenous steroids. He was also started on azithromycin, gentamicin, and acyclovir, which were discontinued after negative blood cultures at 48 hours. The patient's gait improved shortly after steroid therapy was initiated. On hospital day number 3, serum was sent for Lyme disease, Bartonella, Epstein-Barr virus, Leptospira, measles, mumps, mycoplasma, Cryptococcus, and comprehensive viral and fungal culture. Cerebrospinal fluid herpes simplex virus and enteroviral polymerase chain reaction were negative. His gait continued to improve and was completely back to baseline after completing 3 days of intravenous steroids and 2 days of oral prednisone. Thus, the patient was discharge after 5 days of treatment and scheduled for a follow-up MRI study. One day after discharge, the patient's Lyme enzyme-linked immunosorbent assay came back positive and was confirmed with Western blot. Thus, the patient was readmitted to have a central line placement to receive intravenous Rocephin for a duration of 21 to 28 days. He was discharged the second day of his second admission and subsequently completed a full course of intravenous Rocephin. He had no repeated episodes of ataxia. Cerebellar involvement in Lyme disease is a known but rare manifestation of the illness with very few reports in the literature in children. More common causes of acute cerebellar ataxia include immune-mediated postviral infections, labyrinthitis, or cerebellar tumor [1]. A broad range of Lyme disease-associated neurologic disorders have been described in children. These include cranial neuropathies, headache, seizures, meningitis, meningoencephalitis, encephalopathy, focal neurologic signs, ataxia, vertigo, chorea, and transverse myelitis [2]. The most frequent of which are facial nerve palsy and meningitis [3]. Lyme disease occurs in the northeast of the United States (from Maine to Maryland), in the Midwest (Wisconsin and Minnesota), and on the west coast (northern California and Oregon) [4]. It is the most frequent vector-borne disease of the northern temperate zone [5]. Lyme borreliosis is an infection caused by spirochetes of the group Borrelia burgdorferi, which is generally transmitted by the symptomless bite of certain ticks of the species Ixodes scapularis. Lyme borreliosis occurs with similar frequencies in men and women and affects people of all ages; however, it affects children and elderly adults more frequently [6]. Birds, several strains of mice, dormice, deer, voles, as well as lizards are the major reservoirs of Borrelia. The tick has a 2-year, 3-stage life cycle (larval, nymphal, and adult) and feeds once during each stage. The tick may become infected at any stage of its life cycle by feeding on a natural reservoir for B burgdorferi. Larvae emerge in the following spring in the nymphal stage. The nymphal stage tick is most likely to transmit the infection, presumably because it is so small that it is difficult to identify the bite and to remove the tick in a timely manner. Moreover, nymphs are prevalent during spring and summer, when people often enter habitats in which ticks thrive. In each of the 3 stages the ticks require a blood meal [4]. Children account for a disproportionate number of Lyme borreliosis cases, presumably because of increased exposure and decreased attention to prevention. Manifestations are similar to those in adults. Lyme borreliosis manifestations in children include erythema migrans (solitary/multiple lesions) in 89%, arthritis in 6%, facial nerve palsy in 3%, meningitis in 2%, and carditis in 0% to 5% in 201 consecutive children with a median age of 7 years (range, 1-21 years) followed in Connecticut [4]. Younger children (<10 years) more frequently have fever with the onset of arthritis, a more acute course, lower antibody titers to B burgdorferi, a shorter arthritis duration after antibiotics are started, and a better 1 year prognosis [5]. Children present more frequently than adults with acute early neuroborreliosis [5]. Neuroborreliosis can manifest as a lymphocytic meningitis, cranial neuritis (particularly peripheral facial nerve palsy), radiculoneuropathy, and rarely, encephalomyelitis. There can also be headache, fatigue, paresthesia, or stiff neck without other objective symptoms of Lyme borreliosis. The symptoms of neuroborreliosis develop within weeks after the onset of erythema migrans. Peripheral neuropathy (chronic axonal neuropathy); paresthesias; less commonly, radicular pain; encephalopathy (typically subacute or chronic, subtle memory and cognitive dysfunction); and encephalomyelitis (unifocal or multifocal inflammatory disease) are neurological manifestations [4]. Patients with B burgdorferi infection have a unique multi isotype antibody response in the CSF not seen in other neurologic diseases. They demonstrate intrathecal IgM, IgG, and IgA antibody responses to the spirochete [1]. Definitive diagnosis requires CSF assessment for intrathecal antibody production or demonstration of organisms by culture, histopathology, or polymerase chain reaction [7]. Acute neuroborreliosis may resolve without antibiotic therapy; however, the duration of neurologic symptoms is longer, and the risk for sequelae is greater [5,8]. For patients with evidence of neurologic abnormalities, a 2- to 4-week course of intravenous ceftriaxone (75-100 mg/kg or 2 g/d) is most commonly administered. Intravenous therapies with cefotaxime (150-200 mg/kg or 6 g/d) or penicillin G (200-400 000 U/kg per day or 18-24 MU/day) are alternatives [2,5,7]. The signs and symptoms of acute neuroborreliosis usually resolve within weeks, but those of chronic neuroborreliosis improve slowly over a period of months [4]. Although neuroborreliosis is a rare presentation of Lyme disease, it is a more likely manifestation in a child with the disease. We recommend the emergency physicians that treat children in endemic areas become familiar with this unusual presentation of a common illness.

AB - We present a case of a 4-year-old who presented to the emergency department with an unsteady gait for 2 days. Ataxia is a rare but known manifestation of cerebellar involvement in Lyme disease. A 4-year-old (17 kg) boy with no significant medical history presented to the emergency department (ED) with history of nonbloody emesis for 2 weeks and an unsteady gait for 2 days. Over the past 2 days, his gait had gotten progressively worse until he was unable to walk without assistance. The vomiting would usually occur 1 hour after eating meals. He had also complained of a single headache, which occurred approximately 10 days before admission. The headache did not occur in the early morning hours or wake him up from his sleep. His appetite for the weeks before admission had progressively decreased, and he had also become more irritable, especially when stimulated. He had increased fatigue for the week before presentation. His parents denied any fever, rhinorrhea, cough, diarrhea, rash, bruising, bleeding, or hematuria. The patient denied any abdominal pain or headache while in the ED. On arrival to the pediatric ED, the patient was awake but lying down in bed and in no acute distress. Vitals signs were temperature of 37.7°C, heart rate of 117 beats/min, respiratory rate of 24/min, blood pressure of 95/63, and oxygen saturation of 98% on room air. Physical examination was normal except for his ataxic gait. He was unable to sit up on the bed alone without falling to the side. When walking down the hallway, the patient stumbled to one side and almost ran into the wall before losing his balance. Complete blood count and serum chemistries were normal. Because of concern for an intracranial mass, a computed tomography of the head without contrast was ordered and was negative. Neurology was consulted, and it was their impression that the patient had either ataxia or diffuse weakness. They recommended the patient be admitted overnight for further workup. On admission to the inpatient ward, the patient had orthostatic blood pressures done, which were normal. A lumbar puncture was done the evening of admission, which showed colorless, clear fluid, no xanthochromia, 35 red blood cells, 93 white blood cells with 20% segs, 45% lymphs, 33% monos, 2% eosinophils. Cerebrospinal fluid (CSF) glucose was 62 mg/dL, and CSF protein was 22 mg/dL. C-reactive protein was 0.297 mg/dL, ESR 33 mm/h. The morning after admission, the patient was noted to have 3+ reflexes in the lower extremities, emotional lability, bilateral ankle clonus (left greater than right), and a positive Brudzinski and Kernig sign. On day 2 of hospitalization, neurology ordered a magnetic resonance imaging (MRI) of the brain with and without contrast. The MRI showed multifocal areas of subcortical white matter hyperintensity consistent with acute disseminated encephalitis. The patient was started on high-dose prednisone at 20 mg/kg, intravenously, for 3 days with 2 days of oral prednisone after completing the intravenous steroids. He was also started on azithromycin, gentamicin, and acyclovir, which were discontinued after negative blood cultures at 48 hours. The patient's gait improved shortly after steroid therapy was initiated. On hospital day number 3, serum was sent for Lyme disease, Bartonella, Epstein-Barr virus, Leptospira, measles, mumps, mycoplasma, Cryptococcus, and comprehensive viral and fungal culture. Cerebrospinal fluid herpes simplex virus and enteroviral polymerase chain reaction were negative. His gait continued to improve and was completely back to baseline after completing 3 days of intravenous steroids and 2 days of oral prednisone. Thus, the patient was discharge after 5 days of treatment and scheduled for a follow-up MRI study. One day after discharge, the patient's Lyme enzyme-linked immunosorbent assay came back positive and was confirmed with Western blot. Thus, the patient was readmitted to have a central line placement to receive intravenous Rocephin for a duration of 21 to 28 days. He was discharged the second day of his second admission and subsequently completed a full course of intravenous Rocephin. He had no repeated episodes of ataxia. Cerebellar involvement in Lyme disease is a known but rare manifestation of the illness with very few reports in the literature in children. More common causes of acute cerebellar ataxia include immune-mediated postviral infections, labyrinthitis, or cerebellar tumor [1]. A broad range of Lyme disease-associated neurologic disorders have been described in children. These include cranial neuropathies, headache, seizures, meningitis, meningoencephalitis, encephalopathy, focal neurologic signs, ataxia, vertigo, chorea, and transverse myelitis [2]. The most frequent of which are facial nerve palsy and meningitis [3]. Lyme disease occurs in the northeast of the United States (from Maine to Maryland), in the Midwest (Wisconsin and Minnesota), and on the west coast (northern California and Oregon) [4]. It is the most frequent vector-borne disease of the northern temperate zone [5]. Lyme borreliosis is an infection caused by spirochetes of the group Borrelia burgdorferi, which is generally transmitted by the symptomless bite of certain ticks of the species Ixodes scapularis. Lyme borreliosis occurs with similar frequencies in men and women and affects people of all ages; however, it affects children and elderly adults more frequently [6]. Birds, several strains of mice, dormice, deer, voles, as well as lizards are the major reservoirs of Borrelia. The tick has a 2-year, 3-stage life cycle (larval, nymphal, and adult) and feeds once during each stage. The tick may become infected at any stage of its life cycle by feeding on a natural reservoir for B burgdorferi. Larvae emerge in the following spring in the nymphal stage. The nymphal stage tick is most likely to transmit the infection, presumably because it is so small that it is difficult to identify the bite and to remove the tick in a timely manner. Moreover, nymphs are prevalent during spring and summer, when people often enter habitats in which ticks thrive. In each of the 3 stages the ticks require a blood meal [4]. Children account for a disproportionate number of Lyme borreliosis cases, presumably because of increased exposure and decreased attention to prevention. Manifestations are similar to those in adults. Lyme borreliosis manifestations in children include erythema migrans (solitary/multiple lesions) in 89%, arthritis in 6%, facial nerve palsy in 3%, meningitis in 2%, and carditis in 0% to 5% in 201 consecutive children with a median age of 7 years (range, 1-21 years) followed in Connecticut [4]. Younger children (<10 years) more frequently have fever with the onset of arthritis, a more acute course, lower antibody titers to B burgdorferi, a shorter arthritis duration after antibiotics are started, and a better 1 year prognosis [5]. Children present more frequently than adults with acute early neuroborreliosis [5]. Neuroborreliosis can manifest as a lymphocytic meningitis, cranial neuritis (particularly peripheral facial nerve palsy), radiculoneuropathy, and rarely, encephalomyelitis. There can also be headache, fatigue, paresthesia, or stiff neck without other objective symptoms of Lyme borreliosis. The symptoms of neuroborreliosis develop within weeks after the onset of erythema migrans. Peripheral neuropathy (chronic axonal neuropathy); paresthesias; less commonly, radicular pain; encephalopathy (typically subacute or chronic, subtle memory and cognitive dysfunction); and encephalomyelitis (unifocal or multifocal inflammatory disease) are neurological manifestations [4]. Patients with B burgdorferi infection have a unique multi isotype antibody response in the CSF not seen in other neurologic diseases. They demonstrate intrathecal IgM, IgG, and IgA antibody responses to the spirochete [1]. Definitive diagnosis requires CSF assessment for intrathecal antibody production or demonstration of organisms by culture, histopathology, or polymerase chain reaction [7]. Acute neuroborreliosis may resolve without antibiotic therapy; however, the duration of neurologic symptoms is longer, and the risk for sequelae is greater [5,8]. For patients with evidence of neurologic abnormalities, a 2- to 4-week course of intravenous ceftriaxone (75-100 mg/kg or 2 g/d) is most commonly administered. Intravenous therapies with cefotaxime (150-200 mg/kg or 6 g/d) or penicillin G (200-400 000 U/kg per day or 18-24 MU/day) are alternatives [2,5,7]. The signs and symptoms of acute neuroborreliosis usually resolve within weeks, but those of chronic neuroborreliosis improve slowly over a period of months [4]. Although neuroborreliosis is a rare presentation of Lyme disease, it is a more likely manifestation in a child with the disease. We recommend the emergency physicians that treat children in endemic areas become familiar with this unusual presentation of a common illness.

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UR - http://www.scopus.com/inward/citedby.url?scp=54349123532&partnerID=8YFLogxK

U2 - 10.1016/j.ajem.2008.03.029

DO - 10.1016/j.ajem.2008.03.029

M3 - Article

C2 - 19091290

AN - SCOPUS:54349123532

VL - 26

SP - 1069.e5-1069.e6

JO - American Journal of Emergency Medicine

JF - American Journal of Emergency Medicine

SN - 0735-6757

IS - 9

ER -