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Cervicomedullary Injury After Pneumococcal Meningitis With Brain Edema FREE

RajaNandini Muralidharan, MD; Alejandro A. Rabinstein, MD; Eelco F. M. Wijdicks, MD, PhD
[+] Author Affiliations

Author Affiliations: Department of Neurology, Mayo Clinic, Rochester, Minnesota.


Arch Neurol. 2011;68(4):513-516. doi:10.1001/archneurol.2011.61.
Text Size: A A A
Published online

ABSTRACT

Objectives  To demonstrate a rare but potential mechanism of quadriplegia in a patient with fulminant pneumococcal meningitis complicated by severe intracranial hypertension.

Design  Case report.

Setting  Intensive care unit.

Patient  A 21-year-old man who presented with 3 days of headache, combativeness, and fever.

Intervention  Antibiotics and steroids were initiated after lumbar puncture yielded purulent cerebrospinal fluid and streptococcus pneumoniae.

Results  The patient's course was complicated by severe cerebral edema necessitating intracranial pressure monitoring and intracranial pressure–targeted therapy. Within 5 days he developed quadriplegia and areflexia. Brain and cervical spine magnetic resonance imaging revealed patchy areas of T2 signal hyperintensity with associated gadolinium enhancement in the superior cervical spinal cord, cerebellar tonsils, and medulla.

Conclusions  Quadriplegia secondary to tonsillar herniation in fulminant meningitis is rare but should be considered in patients with acute quadriparesis after treatment of increased intracranial pressure. Magnetic resonance imaging signal changes and gadolinium enhancement may be demonstrated. Significant improvement of cord symptoms can be expected.

Figures in this Article

REPORT OF A CASE

A 21-year old man was brought into the hospital with 3 days of headache followed by acute stupor, combativeness, and fever. A noncontrast computed tomographic scan showed mild generalized cerebral edema, and his white blood cell count was 22 000. He was intubated for airway protection and then started receiving sedation with propofol and fentanyl. His temperature was 39.4°C; blood pressure, 105/57; and heart rate, 123 beats per minute. Findings of neurologic examination were normal except for positive Brudzinski sign. Lumbar puncture yielded markedly purulent cerebrospinal fluid, a protein level of 0.773 g/dL (to convert to grams per liter, multiply by 10.0), and a glucose level of less than 20 mg/dL (to convert to millimoles per liter, multiply by 0.0555). A Gram stain showed gram-positive cocci that was later proved to be streptococcus pneumoniae. He started receiving 10 mg of ceftriaxone, vancomycin, and dexamethasone intravenously every 6 hours. He was overbreathing the ventilator and was placed on spontaneous mode of ventilation overnight. The next morning, he did not awaken despite discontinued sedation and was found to be deeply comatose. Minimally reactive pupils without corneal, gag, or cough reflexes were observed. Oculocephalic reflexes were only present on one side. He had no motor response to pain and did not breathe during the set ventilatory rate. There were subtle rhythmic facial movements that were suspicious for seizure. A repeated computed tomographic scan showed global effacement of cisterns, marked increase in cerebral edema, low cortical density areas consistent with encephalitis, and early cerebellar tonsillar herniation (Figure 1A).

Place holder to copy figure label and caption
Figure 1.

A, A computed tomographic scan of the brain without contrast before the start of intracranial pressure–targeted therapy shows severe generalized edema, effacement of cisterns within the brainstem, and tonsillar herniation; notice the pseudosubarachnoid hemorrhage appearance due to the severity of brain swelling and purulent cerebrospinal fluid. B, A repeated scan 5 days later shows dramatic improvement in cerebral edema, reappearance of subarachnoid spaces, and retraction of cerebellar tonsils.

Graphic Jump Location

The patient started receiving high-dose dexamethasone (100 mg followed by 10 mg every 4 hours) and 20% mannitol (2 g/kg followed by 0.5 g/kg every 4 hours) in an effort to reduce cerebral edema. Levetiracetam was given after an electroencephalogram showed epileptogenic abnormalities with severe generalized slowing and little background variability. Severe intracranial hypertension was detected after placement of ICP monitor (80 mm Hg on insertion, stabilizing between 50-58 mm Hg) around the time the patient was receiving the bolus doses of dexamethasone and mannitol. Systemic cooling to 33°C was instituted. Dopamine intravenous infusion was implemented to maintain cerebral perfusion pressures between 60 and 70 mm Hg. Within a few hours, his ICP decreased to 18 to 20 mm Hg and he recovered cough and right corneal reflexes. By the fifth hospital day, he had regained all brainstem reflexes but had generalized areflexia with no response to noxious stimulation of his extremities. Repeated computed tomography of the head showed marked improvement with reappearance of subarachnoid spaces and global reduction in cerebral edema (Figure 1B). Mannitol and intracranial pressure (ICP) monitoring were discontinued and he was extubated on day 10. He mouthed responses to questions with normal facial strength but had brisk reflexes in the lower extremities with clonus and extensor plantar response. Diminished touch, temperature, and pinprick were present on his cheeks, mandible, and forehead. His joint position was recognized by the patient only at the level of his left shoulder and knee and reduced diffusely on his right side. Vibration was reduced in all extremities, though in the left more than the right. Patchy pinprick loss was detected in both extremities to his mid–upper arm. There was no definitive sensory level. Lesions were localized to the corticospinal tracts, spinal tract of trigeminal nerve, dorsal columns, and anterior columns. The “onion skin” pattern of facial sensory involvement was localized to spinal trigeminal tract located in the upper cervical cord and suggested pathology affecting the upper cervical cord and lower medulla. Magnetic resonance imaging of the brain with contrast showed scattered abnormal parenchymal signal and enhancement compatible with inflammatory changes of meningitis, without residual cerebral edema. Magnetic resonance imaging of the cervical spine showed abnormal patchy areas of T2 signal with associated enhancement in the superior cervical spinal cord, cerebellar tonsils, and medulla (Figure 2). There was no abnormal cord signal or enhancement below the level of C2. He was discharged to our inpatient rehabilitation service and, after 2 months, has regained most strength in his upper extremities and is walking with 2-person assistance.

Place holder to copy figure label and caption
Figure 2.

T1-weighted postgadolinium sagittal (A and B) and T2-weighted axial (C) images 1.5 weeks after the onset of illness show cerebellar tonsillar herniation below the foramen magnum with subsequent cervicomedullary compression. Patchy gadolinium enhancement and T2 signal changes are restricted to the cervicomedullary junction and cerebellar tonsils, as indicated by the arrows.

Graphic Jump Location

COMMENT

This is the first reported case of quadriplegia due to tonsillar herniation after pneumococcal meningitis with successfully treated brain edema since Ropper and Kanis' seminal case 10 years ago.1 Our case is also remarkable because of neurologic improvement after immediate intervention with high-dose steroids, ICP monitoring with aggressive ICP management, and antibiotics.

Pneumococcal meningitis is well known for its associated intracranial complications including cerebral edema reported in as many as 5.7% to 29% of patients with cerebrovascular complications.24 The benefit of steroid administration in pneumococcal meningitis is unproven5 but improved neurological outcome has been reported, presumably by mediating toxic inflammatory mediators released during bacterial lysis that can hasten cerebral edema and accelerate herniation.2,68 Edema tends to be an early feature of meningitis in adults, often presenting with stupor or coma on examination,2,8 and findings of CT may be normal.911 It was noted in a study by Lindvall et al11 that mean ICP was higher and cerebral perfusion pressure was lower in nonsurvivors of bacterial meningitis, with streptococcus pneumoniae being the most common pathogen identified. Results from The Dutch Cohort Study showed that death in younger patients (<60 years) was primarily secondary to neurological complications such as cerebral edema and herniation, while patients older than 60 years died of systemic complications.3

Treatment according to ICP-targeted therapy may help direct management in comatose or stuporous patients with meningitis, potentially leading to improved outcomes in this subset of patients, who already face higher mortality rates.2,8,1013 Although there is no proof of benefit of ICP monitoring in meningitis, ICP monitor placement and supportive therapies geared at lowering ICP may be beneficial in younger adults with symptoms of intracranial hypertension. There are also scattered case reports that describe successful use of ICP monitoring, Transcranial Doppler, craniectomy, and medical therapy based on the “Lund concept,” but none have been validated.12,1416

Quadriplegia after meningitis has been reported to occur in 2% of patients with meningitis and can be associated with tonsillar herniation, myelitis, vasculitis, cord infarction, arachnoiditis, systemic hypotension, and epidural abscess.2,10,17,18 Cervicomedullary injury due to the unwanted consequence of tonsillar compression within the foramen magnum following severe brain edema with resultant elevated intracranial pressures is the most likely mechanism in our patient. We suspect the prevalence of this injury is rare because few comatose patients with early brainstem involvement recover after treatment of severe brain edema. The high-dose steroids given to these patients might indicate a diagnosis of steroid myopathy. Spinal shock resulting in flaccid paraparesis immediately following tonsillar herniation may also be mistaken for a critical illness polyneuropathy. Myelitis associated with the primary infection has also been reported to account for 2.3% of quadriparesis in these patients.4,19 However, careful inspection of the magnetic resonance images of the spine in patients with myelitis reveals that the T2 signal hyperintensity in the spinal cord and leptomeningeal gadolinium enhancement are usually most pronounced in the gray matter and are longitudinally extensive, spanning from the cervical to thoracolumbar cord,4 whereas in patients with cervicomedullary injury secondary to tonsillar herniation, the intramedullary and meningeal changes are patchy and limited to the upper cervical cord and cerebellar tonsils. Though herniation may also lead to vascular compromise with resultant cord infarction,18 the pattern of T2 signal changes in our patient did not conform to a spinal arterial distribution.

In conclusion, quadriplegia secondary to cervicomedullary injury arising from tonsillar herniation due to severe intracranial hypertension is a rare complication of pneumococcal meningitis. Magnetic resonance imaging signal changes and gadolinium enhancement are restricted to the upper cervical cord and cerebellar tonsils. Prognosis is generally poor but favorable recovery with aggressive corticosteroid treatment and ICP-targeted therapy can be achieved, as demonstrated in our patient.

ARTICLE INFORMATION

Correspondence: Eelco F. M. Wijdicks, MD, PhD, Department of Neurology, Mayo Clinic, W8B, 200 First St SW, Rochester, MN 55905 (wijde@mayo.edu).

Accepted for Publication: September 7, 2010.

Author Contributions:Study concept and design: Muralidharan and Wijdicks. Acquisition of data: Muralidharan and Wijdicks. Analysis and interpretation of data: Muralidharan, Rabinstein, and Wijdicks. Drafting of the manuscript: Muralidharan and Wijdicks. Critical revision of the manuscript for important intellectual content: Muralidharan and Rabinstein. Administrative, technical, and material support: Muralidharan. Study supervision: Rabinstein and Wijdicks.

Financial Disclosure: Dr Rabinstein reports receiving research support from CardioNet for an investigator-initiated project.

REFERENCES

Ropper  AHKanis  KB Flaccid quadriplegia from tonsillar herniation in pneumococcal meningitis. J Clin Neurosci 2000;7 (4) 339- 341
PubMed Link to Article
Pfister  HWFeiden  WEinhäupl  KM Spectrum of complications during bacterial meningitis in adults: results of a prospective clinical study. Arch Neurol 1993;50 (6) 575- 581
PubMed Link to Article
Weisfelt  Mvan de Beek  DSpanjaard  LReitsma  JBde Gans  J Clinical features, complications, and outcome in adults with pneumococcal meningitis: a prospective case series. Lancet Neurol 2006;5 (2) 123- 129
PubMed Link to Article
Kastenbauer  SWinkler  FFesl  G  et al.  Acute severe spinal cord dysfunction in bacterial meningitis in adults: MRI findings suggest extensive myelitis. Arch Neurol 2001;58 (5) 806- 810
PubMed Link to Article
van de Beek  DFarrar  JJde Gans  J  et al.  Adjunctive dexamethasone in bacterial meningitis: a meta-analysis of individual patient data. Lancet Neurol 2010;9 (3) 254- 263
PubMed Link to Article
Weisfelt  Mvan de Beek  Dde Gans  J Dexamethasone treatment in adults with pneumococcal meningitis: risk factors for death. Eur J Clin Microbiol Infect Dis 2006;25 (2) 73- 78
PubMed Link to Article
Sala  FAbbruzzese  CGalli  D  et al.  Intracranial pressure monitoring in pediatric bacterial meningitis: a fancy or useful tool? a case report. Minerva Anestesiol 2009;75 (12) 746- 749
PubMed
van de Beek  DWeisfelt  Mde Gans  JTunkel  ARWijdicks  EF Drug insight: adjunctive therapies in adults with bacterial meningitis. Nat Clin Pract Neurol 2006;2 (9) 504- 516
PubMed Link to Article
Rennick  GShann  Fde Campo  J Cerebral herniation during bacterial meningitis in children. BMJ 1993;306 (6883) 953- 955
PubMed Link to Article
Grände  POMyhre  EBNordström  CHSchliamser  S Treatment of intracranial hypertension and aspects on lumbar dural puncture in severe bacterial meningitis. Acta Anaesthesiol Scand 2002;46 (3) 264- 270
PubMed Link to Article
Lindvall  PAhlm  CEricsson  MGothefors  LNaredi  SKoskinen  LO Reducing intracranial pressure may increase survival among patients with bacterial meningitis. Clin Infect Dis 2004;38 (3) 384- 390
PubMed Link to Article
Flores-Cordero  JMAmaya-Villar  RRincón-Ferrari  MD  et al.  Acute community-acquired bacterial meningitis in adults admitted to the intensive care unit: clinical manifestations, management and prognostic factors. Intensive Care Med 2003;29 (11) 1967- 1973
PubMed Link to Article
van de Beek  Dde Gans  JTunkel  ARWijdicks  EF Community-acquired bacterial meningitis in adults. N Engl J Med 2006;354 (1) 44- 53
PubMed Link to Article
Cuthbertson  BHDickson  RMackenzie  A Intracranial pressure measurement, induced hypothermia and barbiturate coma in meningitis associated with intractable raised intracranial pressure. Anaesthesia 2004;59 (9) 908- 911
PubMed Link to Article
Di Rienzo  AIacoangeli  MRychlicki  FVeccia  SScerrati  M Decompressive craniectomy for medically refractory intracranial hypertension due to meningoencephalitis: report of three patients. Acta Neurochir (Wien) 2008;150 (10) 1057- 1065
PubMed Link to Article
Baussart  BCheisson  GCompain  M  et al.  Multimodal cerebral monitoring and decompressive surgery for the treatment of severe bacterial meningitis with increased intracranial pressure. Acta Anaesthesiol Scand 2006;50 (6) 762- 765
PubMed Link to Article
Norman  MG Respiratory arrest and cervical spinal cord infarction following lumbar puncture in meningitis. Can J Neurol Sci 1982;9 (4) 443- 447
PubMed
Moffett  KSBerkowitz  FE Quadriplegia complicating Escherichia coli meningitis in a newborn infant: case report and review of 22 cases of spinal cord dysfunction in patients with acute bacterial meningitis. Clin Infect Dis 1997;25 (2) 211- 214
PubMed Link to Article
Kastenbauer  SPfister  HW Pneumococcal meningitis in adults: spectrum of complications and prognostic factors in a series of 87 cases. Brain 2003;126 (pt 5) 1015- 1025
PubMed Link to Article

Figures

Place holder to copy figure label and caption
Figure 1.

A, A computed tomographic scan of the brain without contrast before the start of intracranial pressure–targeted therapy shows severe generalized edema, effacement of cisterns within the brainstem, and tonsillar herniation; notice the pseudosubarachnoid hemorrhage appearance due to the severity of brain swelling and purulent cerebrospinal fluid. B, A repeated scan 5 days later shows dramatic improvement in cerebral edema, reappearance of subarachnoid spaces, and retraction of cerebellar tonsils.

Graphic Jump Location
Place holder to copy figure label and caption
Figure 2.

T1-weighted postgadolinium sagittal (A and B) and T2-weighted axial (C) images 1.5 weeks after the onset of illness show cerebellar tonsillar herniation below the foramen magnum with subsequent cervicomedullary compression. Patchy gadolinium enhancement and T2 signal changes are restricted to the cervicomedullary junction and cerebellar tonsils, as indicated by the arrows.

Graphic Jump Location

Tables

References

Ropper  AHKanis  KB Flaccid quadriplegia from tonsillar herniation in pneumococcal meningitis. J Clin Neurosci 2000;7 (4) 339- 341
PubMed Link to Article
Pfister  HWFeiden  WEinhäupl  KM Spectrum of complications during bacterial meningitis in adults: results of a prospective clinical study. Arch Neurol 1993;50 (6) 575- 581
PubMed Link to Article
Weisfelt  Mvan de Beek  DSpanjaard  LReitsma  JBde Gans  J Clinical features, complications, and outcome in adults with pneumococcal meningitis: a prospective case series. Lancet Neurol 2006;5 (2) 123- 129
PubMed Link to Article
Kastenbauer  SWinkler  FFesl  G  et al.  Acute severe spinal cord dysfunction in bacterial meningitis in adults: MRI findings suggest extensive myelitis. Arch Neurol 2001;58 (5) 806- 810
PubMed Link to Article
van de Beek  DFarrar  JJde Gans  J  et al.  Adjunctive dexamethasone in bacterial meningitis: a meta-analysis of individual patient data. Lancet Neurol 2010;9 (3) 254- 263
PubMed Link to Article
Weisfelt  Mvan de Beek  Dde Gans  J Dexamethasone treatment in adults with pneumococcal meningitis: risk factors for death. Eur J Clin Microbiol Infect Dis 2006;25 (2) 73- 78
PubMed Link to Article
Sala  FAbbruzzese  CGalli  D  et al.  Intracranial pressure monitoring in pediatric bacterial meningitis: a fancy or useful tool? a case report. Minerva Anestesiol 2009;75 (12) 746- 749
PubMed
van de Beek  DWeisfelt  Mde Gans  JTunkel  ARWijdicks  EF Drug insight: adjunctive therapies in adults with bacterial meningitis. Nat Clin Pract Neurol 2006;2 (9) 504- 516
PubMed Link to Article
Rennick  GShann  Fde Campo  J Cerebral herniation during bacterial meningitis in children. BMJ 1993;306 (6883) 953- 955
PubMed Link to Article
Grände  POMyhre  EBNordström  CHSchliamser  S Treatment of intracranial hypertension and aspects on lumbar dural puncture in severe bacterial meningitis. Acta Anaesthesiol Scand 2002;46 (3) 264- 270
PubMed Link to Article
Lindvall  PAhlm  CEricsson  MGothefors  LNaredi  SKoskinen  LO Reducing intracranial pressure may increase survival among patients with bacterial meningitis. Clin Infect Dis 2004;38 (3) 384- 390
PubMed Link to Article
Flores-Cordero  JMAmaya-Villar  RRincón-Ferrari  MD  et al.  Acute community-acquired bacterial meningitis in adults admitted to the intensive care unit: clinical manifestations, management and prognostic factors. Intensive Care Med 2003;29 (11) 1967- 1973
PubMed Link to Article
van de Beek  Dde Gans  JTunkel  ARWijdicks  EF Community-acquired bacterial meningitis in adults. N Engl J Med 2006;354 (1) 44- 53
PubMed Link to Article
Cuthbertson  BHDickson  RMackenzie  A Intracranial pressure measurement, induced hypothermia and barbiturate coma in meningitis associated with intractable raised intracranial pressure. Anaesthesia 2004;59 (9) 908- 911
PubMed Link to Article
Di Rienzo  AIacoangeli  MRychlicki  FVeccia  SScerrati  M Decompressive craniectomy for medically refractory intracranial hypertension due to meningoencephalitis: report of three patients. Acta Neurochir (Wien) 2008;150 (10) 1057- 1065
PubMed Link to Article
Baussart  BCheisson  GCompain  M  et al.  Multimodal cerebral monitoring and decompressive surgery for the treatment of severe bacterial meningitis with increased intracranial pressure. Acta Anaesthesiol Scand 2006;50 (6) 762- 765
PubMed Link to Article
Norman  MG Respiratory arrest and cervical spinal cord infarction following lumbar puncture in meningitis. Can J Neurol Sci 1982;9 (4) 443- 447
PubMed
Moffett  KSBerkowitz  FE Quadriplegia complicating Escherichia coli meningitis in a newborn infant: case report and review of 22 cases of spinal cord dysfunction in patients with acute bacterial meningitis. Clin Infect Dis 1997;25 (2) 211- 214
PubMed Link to Article
Kastenbauer  SPfister  HW Pneumococcal meningitis in adults: spectrum of complications and prognostic factors in a series of 87 cases. Brain 2003;126 (pt 5) 1015- 1025
PubMed Link to Article

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