2. Case : History
- 54 y/o F previously healthy presented Oct 12, 2017 with progressive parasthesia (tingling and
numbness) and weakness over 3 weeks.
- Parasthesia started in left arm then progressed to right arm and bilateral lower extremities.
- Progressed left arm weakness and bilateral lower extremities weakness.
- History of unsteadiness
- The presentation preceded by flu like syndrome.
- Travel history to Austria (endemic of lyme) a month prior to her symptoms. No history of
hiking/tick bite
- No bowel/ bladder symptoms, hiccups, nausea, vomiting, visual symptoms, headache, spasms,
bulbar symptoms
3. Case : Exam
- CN: Right RAPD, normal VA, and optic disc. Other cranial nerves exam: unremarkable.
- Motor: normal tone, weakness 2-3/5 in Left UE, and 3-4/5 in bilateral LE. Mild weakness in R
UE 4+. Brisky reflexes, up going toes, no clonus.
- Sensory: impaired pin prick and vibration
- Coordination: dysmetria, impaired RAM, ataxia in UE and LE.
- Skin: no rash.
5. Introduction
• Lyme disease: Systemic illness caused by the spirochete
Borrelia burgdorferi and the most common tick-borne illness in
the United States and Europe.
•B. burgdorferi sensu lato group includes at least 20
genospecies, the majority of human infections are caused by
three genospecies:
•B. burgdorferi sensu stricto, which causes disease in North
America and Europe,
• Borrelia afzelii and Borrelia garinii, which cause disease in
Europe and Asia.
6. Life cycle
• Transmission: by infected ticks of the Ixodes ricinus complex.
Ixodes scapularis, the deer or black-legged tick, is the main
vector in the United States.
• Most patients do not recall a tick bite.
• Patients are most likely to have illness in June, July, or August
and less likely from December to March.
10. The numbers of probable, confirmed and incidence of reported
Lyme disease cases by province of residence in Canada, 2015
Available from: https://www.canada.ca/en/public-health/services/diseases/lyme-disease/surveillance-
lyme-disease.html
11. The numbers and incidence of reported Lyme disease cases (confirmed and
probable) by year in Canada, 2009-2015
Available from: https://www.canada.ca/en/public-health/services/diseases/lyme-disease/surveillance-lyme-
disease.html
14. Clinical manifestation of Neuroborreliosis
Early vs Chronic (>6 mo)
Early clinical features:
Occurs in about 15% of patients with untreated erythema migrans,
Median interval between erythema migrans and neurologic disease of 4 weeks.
1. Cranial neuropathy (particularly facial palsy)
2. Lymphocytic meningitis
3. Radiculoneuritis
1. Facial paralysis is a common manifestation of Lyme neuroborreliosis.
- Misdiagnosed as Bell’s palsy
- Bilateral in 25% of the patients.
- Patients with bilateral involvement often have CSF pleocytosis.
15. Early manifestation of Neuroborreliosis
2. Lyme meningitis
- May begin acutely or sub-acutely and may continue for weeks to months if not treated
-Headache.
- Mild neck stiffness occurs in 20% to 30% of patients
- CSF:
- lymphocytic pleocytosis (usually around 100 cells/2L, but can range from 8 to more than 1000)
- moderate increase of protein level
- normal glucose level.
16. Early manifestation of Neuroborreliosis
3- Lyme radiculoneuritis
- Pain is severe, deep in the musculature, and worse at night and does not respond to
common analgesics.
- Distribution: multifocal and asymmetric.
- Frequently misdiagnosed as mechanical radiculopathy and, depending of the
distribution (eg, truncal, abdominal), may lead to investigation of visceral causes for
the pain. –
- Acute mononeuropathies and plexopathies may occur.
17. Early manifestation of Neuroborreliosis
Subacute painful meningoradiculitis, (Bannwarth syndrome or Garin-Bujadoux-Bannwarth
syndrome):
- Most common manifestation of early Lyme neuroborreliosis in Europe,
- Associated with B. garinii.
18. Early manifestation of Neuroborreliosis
When suspecting early Lyme neuroborreliosis, patients should have a complete skin
examination
Clues to the diagnosis include:
- History of tick bite;
- Rash, malaise, or febrile illness within the past 2months
- The disease occurring in the summer or early autumn;
- Endemic area visit.
19. Chronic manifestation of Neuroborreliosis
1. Subtle encephalopathic syndrome affecting memory and cognition has been reported in the
United States1
2. Progressive encephalitis, myelitis, or encephalomyelitis has been reported in Europe2.
3. Chronic mononeuritis or polyneuritis may occur.
- EMG: primarily axonal neuropathy.
22. Lab test (Direct vs Indirect)
• Direct method:
• Difficult
• Require special media and expertise
• Seldom used in clinical diagnosis
• Sensitivity of culture and PCR in CSF samples of patients with early neuroborreliosis is low (10% to 30%)
and even lower in late disease
24. Serology test (Indirect tests)
- No current assays distinguish between active and inactive infection.
- patients may continue to be seropositive for years, including an IgM response, even after
adequate antibiotic treatment.
- Infection acquired in Europe and Asia requires testing and criteria that include other species
within the B. burgdorferi sensu lato complex.
- CSF antibody test should be performed with a paired serum sample to measure the intrathecal
antibody index.
- The majority of patients with early neurologic Lyme disease are seropositive (>90%)
28. Prognosis
1- Facial palsy caused by Lyme disease have an excellent prognosis, with complete or near-
complete recovery
2- Radiculitis and meningitis will improve within days to weeks
3- Late lyme disease: gradual improvement, usually starting a few months after completion of
therapy, and continue to slowly improve for up to 1 to 2 years
30. Post treatment lyme disease syndrome
(PTLDS)
- Definition:
1. Documented episode of Lyme disease,
2. Treatment with an accepted antibiotic regimen
3. Resolution or stabilization of the objective manifestation(s) of Lyme disease,
4. Persistent or relapsing nonspecific symptoms for at least a 6-month period after completion of
antibiotic therapy in patients who have no other condition that could explain their symptoms.
- Non specific symptoms: fatigue, sleep disorders, headache, memory and concentration
difficulties, myalgia, and arthralgia.
- Unknown etiology
- No benefit from antibiotic treatment.
31. Summary
- Nervous system involvement occurs in up to 15% of patients with untreated B. burgdorferi
infection.
- Patients with early Lyme neuroborreliosis present in the summer and early fall, with cranial
neuropathy (particularly seventh nerve palsy), lymphocyticmeningitis, or a painful
radiculoneuritis.
- Late Lyme neuroborreliosis is much less frequent.
- Antibiotic therapy is successful in the majority of patients, but recovery can take weeks to
months.
- The cause of persisting or relapsing nonspecific symptoms for more than 6months after
treatment of Lyme disease is unknown; however, no evidence exists that these patients benefit
from additional antibiotic therapy.
Editor's Notes
Geographic Extension of Lyme Disease (LD) activities. Figure 1shows that Lyme Disease (LD) has extended to many countries around the world beyond the endemic foci. Reported LD activities that were mapped include diagnosed cases as well as infected ticks, infected animals, and seropositive human samples. The dark gray shading signifies countries with (at least) some reported LD activity, and the presence of activity is known only at the country level. The lighter gray shading represents areas in which Lyme disease has been reported at the sub-national level in particular regions of some countries. The lightest gray represents counties with rare or unknown activity.
After several days or weeks, B.
burgdorferi may disseminate hematogenously
from the initial site of infection,
and patients may develop multiple
erythema migrans lesions as well as
neurologic, cardiac, and rheumatologic
involvement.9Y12 Multiple erythema migrans
lesions are similar in appearance
to the primary lesion but are usually
smaller, do not have a punctum, and
have no local symptoms. Some secondary
lesions may be short-lived but may
recur.4 Cardiac involvement is observed
in 4% to 8% of patients, the most common
feature being a fluctuating atrioventricular
block. Borrelial lymphocytoma
is a rare manifestation of Lyme disease,
predominantly seen in Europe. It presents
as a painless bluish-red nodule or
plaque, usually localized in the earlobe
or nipple. Late manifestations include
Lyme arthritis, late Lyme neuroborreliosis,
and acrodermatitis chronica atrophicans.
Lyme arthritis presents a mean
of 6months after the onset of disease as an asymmetric oligoarticular arthriti usually involving the knees and occurs in about 60% of untreated patients in the United States. Acrodermatitis chronica atrophicans, also predominantly seen in Europe associated with infection with B. afzelii, is characterized by slowly progressive skin lesions usually involving the extensor surfaces of the extremities.13
Erythema migrain can be presented as a dissaminated lesion
which often occur in combination.
Patients with
early Lyme neuroborreliosis presenting
with facial palsy are commonly misdiagnosed
as having Bell’s palsy. Systemic
symptoms, headache, radicular pain,
presentation in late summer and fall, a
history of tick bite, and/or erythema
migrans point toward Lyme neuroborreliosis.
