Ticked off at Neuro Lyme Disease

Human tick

Post Prepared by Dr. Mohammed Nasir Yousuf Shah,

PGY-3 Internal Medicine, Monmouth Medical Center

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Case Report

A 36 year old otherwise healthy male noticed a facial droop when he woke up one morning and looked in the mirror. There was associated pain at the angle of the right jaw like a “toothache” and also numbness to along right side of tongue.

He had been experiencing occipital headache with neck pain for the previous 3 days.  The headache was throbbing in character, worse when laying his head back on a pillow. He denied any other neurological symptoms.

Further questioning revealed that 2 months ago he suffered 2 tick bites on his thigh; but did not experience any fever, chills or rash at that time.

Physical examination showed prominent facial droop in the lower half of the right side of the face with inability to puff the cheek on the right and some mild weakness in the upper half of the right side of the face with reduced wrinkling of the forehead. He also had impaired taste sensation along the right side of the tongue. The rest of the neurological exam was normal.

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His brain imaging study was normal, but CSF analysis revealed low glucose and elevated protein and pleocytosis with increased lymphocytes indicating a diagnosis of aseptic meningitis.

Given the history of tick bite 2 months prior and the characteristic 7th cranial nerve palsy, a presumptive diagnosis of neurological Lyme disease was made and the patient was started on intravenous ceftriaxone.

Meanwhile Lyme serologies and antibodies to B. Burgdorferi in CSF were tested and the patient was discharged on IV ceftriaxone.

The results of the serological and CSF testing returned positive for Lyme disease a few days later.

Discussion

Background:

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Borrelia burgdorferi is the organism responsible for Lyme disease which affects several organ systems and is transmitted by the bite of infected ticks belonging to the genus Ixodes.

Skin, the site of inoculation, is involved in 80 percent or more of infected individuals followed by joint involvement.

The third most common site is the nervous system, which is involved in 10 to 15 percent.

Clinical manifestations:

Nervous system involvement begins during early disseminated Lyme disease, when spread of the spirochetes can result in meningeal seeding. Acute neurologic involvement usually occurs weeks to several months after the tick bite and may be the first manifestation of Lyme disease. In contrast, certain neurologic problems, such as a more indolent, disseminated polyneuropathy, may develop months to a few years after the initial infection.

Lymphocytic meningitis, cranial neuropathy and radiculoneuritis constitute the classic triad of acute, early neurologic Lyme disease.

Clinical findings of nervous system Lyme disease are divided into disorders of the peripheral vs. central nervous system.

Peripheral nervous system

In early disease, two peripheral nerve manifestations are particularly common and form part of the classic triad.

Cranial neuropathies: These tend to occur early in infection and are usually abrupt in onset. Virtually any cranial nerve can be involved, but the seventh (facial) is by far the most common, occurring in 8 percent of cases.

Since facial nerve palsy is uncommon in young children, Lyme disease should be strongly considered as the cause of facial nerve palsy affecting a child who has been in an endemic area. In adults in endemic areas, during spring through fall, a significant percentage of facial nerve palsies are attributable to Lyme disease. Involvement can be bilateral and because bilateral facial nerve palsies are generally uncommon, Lyme disease should be suspected in patients with potential recent exposure.

Radiculoneuritis: This is reported in 3 percent of cases of Lyme disease and is often missed. It can mimic a mechanical radiculopathy (eg, sciatica) with radicular pain in one or several dermatomes, accompanied by corresponding sensory, motor and reflex changes. This disorder should be considered in patients in endemic areas presenting in spring through autumn with severe limb or truncal radicular pain without an apparent mechanical precipitant.

Central nervous system

The most common form of CNS involvement is lymphocytic meningitis. Rarely, inflammation of the brain and/or spinal cord parenchyma (an encephalomyelitis) can occur.

Meningitis: Lymphocytic meningitis, alone or in combination with cranial nerve or spinal nerve root involvement, represents the most common form of central nervous system involvement. Clinically it is indistinguishable from viral meningitis, with headache, fever, other systemic symptoms, photosensitivity, and neck stiffness.

Encephalopathy: Patients may experience fatigue, cognitive slowing, and memory difficulty. However, these symptoms are nonspecific and are frequent concomitants of many inflammatory disorders.

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Diagnosis

The diagnosis of nervous system Lyme disease rests on three elements:

  • Since the disease is transmitted exclusively by bites of Ixodes ticks, there must be the possibility of exposure
  • The clinical disorder should include objective evidence of nervous system Lyme disease
  • Laboratory testing (positive two tier Lyme serologies with or without positive CSF Lyme antibodies)

Serologic testing: With the exception of the first 4 to 6 weeks of infection, when the specific immune response may not yet have developed sufficiently to provide a measurable antibody response, serologic testing for antibodies to B. burgdorferi is highly sensitive and specific for the diagnosis of Lyme disease and thus in such cases the absence of detectable antibodies in the serum is strong evidence against the diagnosis.

The two-tier strategy, which is recommended by the US Centers for Disease Control and Prevention, uses a sensitive enzyme-linked immunosorbent assay (ELISA) followed by a Western blot. If the ELISA is positive or equivocal, then the same serum sample should be tested by Western blot. If the ELISA is negative, the sample needs no further testing.

2 tier

CSF analysis: In Lyme meningitis the CSF typically has a modest pleocytosis of up to several hundred lymphocytes and/or monocytes per microL. The CSF protein concentration is usually moderately elevated, and is typically no greater than about 200 to 300 mg/dL (2 to 3 g/L).

CSF antibodies: The sensitivity for testing the CSF for intrathecal production of antibodies to B. burgdorferi is poor and a negative test does not exclude CNS Lyme disease if clinical circumstances support the diagnosis.

Imaging:  Since Lyme encephalomyelitis is so rare, MRI of the brain and spine is only rarely abnormal in Lyme disease. When present, the MRI reveals areas of increased signal on T2 and FLAIR sequences.

Electrophysiologic testing: In patients with a peripheral neuropathy, electrophysiologic assessment (electromyography and nerve conduction studies) can be helpful and typically reveal findings consistent with a patchy axonal polyneuropathy (ie, a mononeuropathy multiplex).

Approach to diagnostic testing

Assessment of the patient with possible nervous system Lyme disease must be tailored to the specific presentation. It can be sufficient to simply administer oral antibiotics to patients with recent exposure, a positive serology and an appropriate clinical syndrome.  However a lumbar puncture may still be necessary if there is a strong clinical suspicion of meningitis, primarily to exclude other, potentially more dangerous pathogens.

CSF studies should include cell count, protein and glucose concentrations, and gram stain and bacterial cultures.

CSF and serum should both be sent for anti-B. burgdorferi antibodies and VDRL should be measured.

Neuroimaging, preferably by MRI, should precede the lumbar puncture if the patient has clinical evidence of parenchymal brain involvement. Depending on the findings on imaging,

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Treatment

Lyme meningitis is generally self-limited, even without treatment.

Oral doxycycline is effective for early disseminated Lyme disease with neurologic manifestations, including meningitis. Doxycycline has moderately good penetration into the CSF and has oral bioavailability >98 percent, making oral dosing equivalent to intravenous dosing.

Lyme patients with isolated facial nerve palsy are treated with oral doxycycline (100 mg orally twice daily). Antibiotic therapy does not have a major impact on the outcome of facial palsy. However, treatment is recommended to prevent other complications of disseminated Lyme disease. The majority of patients with Lyme facial palsy recover. The prognosis is worse for patients with bilateral facial palsy compared with unilateral palsy.

Lyme patients with radiculoneuritis, meningitis or other neurologic complications are typically treated using parenteral therapy with ceftriaxone (2 g intravenously once daily) for at least 14 days.

There are no diagnostic tests that can determine clearance of infection or predict the success of therapy. Resolution of neurological symptoms is often delayed and persistence of symptoms is not necessarily indicative of treatment failure.

Treatment recommendations are the same for both the early and late neurologic manifestations of Lyme disease.

 

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Osteoporosis from epidural steroid injections

epidural

Epidural steroid injections are often offered to patients as a conservative treatment for back or leg pain from herniated discs.

We have already highlighted the lack of outcome studies to support this intervention in an earlier post on radiculopathy.

Data from a new study now indicates that epidural steroids may actually be harmful, and increase the risk of osteoporosis with spinal compression fractures.

compression fracuture

Making Sure Pedicle Screws are Correctly Placed During Spine Surgery

spinal hardware

 During a spinal fusion, two or more vertebra are fused together in orrder to eliminate abnormal motion caused by degenerative conditions.

A spinal fusion may require stabilization of the lumbar spine using artificial devices (known as “instrumentation”) including wires, rods, plates and vertebral cages.

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This instrumentation is usually fixed to the vertebral body with a pedicle screw, as can be seen in the adjacent lateral radiograph.

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screw

The pedicle screw is inserted through the bony lumbar pedicle, into the anterior vertebral body.

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These screws are inserted blindly from the back, similar to nailing the back panel on a book case, and just like with the book case, it’s easy to get off track:

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Remember the last time you put a book case together – you nailed the back panel onto the frame (or where you thought the wood frame was), then flipped the whole thing over and found that many nails had missed.

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Obviously, a misplaced screw can end up inside the spinal canal, where it could injure the adjacent nerve roots, a potential cause of post operative deficit:

misplaced screw

Various degrees of misplaced pedicle screws, and then (right) a pathologic specimen showing a pedicle wall that has been perforated by a pedicle screw

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bad screw

As many as 70% of patients undergoing spinal fusion with instrumentation may have a misplaced screw, although most are just misplaced by a millimeter or two, and only 5-10% of those misplaced screws are cause for concern.

However, the incidence of an actual new neurologic deficits from a misplaced screw is much lower, estimated at less than 2 per 1000 screws in a recent study.

Nevertheless, this is still cause for concern, because it may be difficult to detect a misplaced screw during surgery.   Pedicle screw placement may be checked by:  Direct inspection and palpation, Fluoroscopy, Electrical testing, Computerized navigation or the Pediguard system.

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laminectomy

If the surgery involves a laminectomy, then the spinal canal will be open, and the surgeon will either see the misplaced screw, or feel it when they swipe a finger along the medial pedicle wall.

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However, in most cases, there is no laminectomy required, and doing so would prolong surgery time unnecessarily, so misplaced screws can go unrecognized.

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intraop fluoro

Intraoperative fluoroscopy (live X-rays taken during the operation) can detect most pedicle wall perforations and misplaced screws, but is only about 75% accurate because of limited available two dimensional viewing planes.  Furthermore, excessive use can expose the patient to excessive radiation.

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Real time electrophysiologic testing has been used in the operating room to confirm correct placement of pedicle holes and screws during surgery.

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The premise here is that a pedicle screw or hole that is correctly placed within the wall of the bony pedicle (b, above), will be separated from the adjacent nerve root by a layer of cortical bone which has a high impedance (resistance) to the passage of electrical current.

However, a pedicle hole or screw that has perforated the medial bony wall of the pedicle (a, above), will lie directly adjacent to the nerve root without that intervening layer of cortical bone.

Hence electrical stimulation of that perforated hole or screw (a) is more likely to activate the adjacent nerve root and evoke a recordable muscle twitch in the innervated muscle (a) at a lower stimulus intensity (threshold)  than in case of the correctly placed hole or screw (b).

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This electrical threshold testing has become very popular, but requires the presence of specialized equipment and personal in the operating room.

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New “O-arm” technology allows computed tomographic images to be fused with a computerized navigation system, allowing 3 dimensional visualization of pedicle screw tracks as they are inserted in the operating room:

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However, this technology is expensive, and may not be widely available.

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And finally, the Pediguard, a simple, cheaper and widely available technique that uses a disposable hand held drill that emits a signal based on the thickness of surrounding bone, and can be used by any surgeon in any operating room to ensure correct placement of pedicle screws in real time without the need for extra specialized equipment or personnel.

pediguard

Sciatic Neuropathy

The terms lumbar radiculopathy and sciatica are used interchangeably to indicate radiating pain, numbness and weakness in a leg from a pinched nerve root in the back.

However, it is important to recognize that similar symptoms and signs can be caused by injury or compression of the sciatic nerve outside the spine, either in the buttock or thigh.

The sciatic nerve is the longest and widest nerve in the body, extending from the spine all the way to the foot, and contributes most of the nerve supply to the leg:
Sciatic-Nerve-Anatomy

Sciatic nerve injury presents with:

1. Numbness affecting the entire leg, aside from the front of the thigh.

2. Weakness of the hamstrings, and all movement at the ankle.

3. Absent ankle jerk.

http://www.youtube.com/watch?v=z2YrjAGChBw&feature=youtu.be&rel=0

Sciatic Nerve Injury in the Buttock:

The nerve can be injured by misplaced buttock injections, gunshot wounds and knife injury. Buttock injections should be given in the upper outer quadrant to avoid the sciatic nerve

Buttock injections should be given in the upper outer quadrant to avoid the sciatic nerve

The sciatic nerve injury can also be injured by prolonged sitting on a toilet seat, either from direct nerve compression or hemorrhage and compartment syndrome into the gluteal muscles.  This has been reported in cases of  severe prolonged diarrhea, or drug induced coma on the toilet, so called toilet seat neuropathy.

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Sciatic Nerve Injury at the Hip:

The sciatic nerve runs behind the hip joint as it travels through the buttock.
The sciatic nerve is frequently injured by a posterior dislocation of the hip:
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Sciatic nerve injury occurs in as many as 1%–3% of patients who undergo total hip replacement surgery, usually from a stretch injury to the nerves, but occasionally from a misplaced crew, broken piece of wire, fragment of bone or cement pressing on the nerve:

Sciatic nerve injury after hip arthroplasty. (a) The skin incision for the transgluteal approach is in a continuous line. The cross on the left shows the ischium and the one on the right shows the trochanter. Between them, the skin projection of the sciatic nerve is seen. (b) The sciatic nerve was freed from all attachments. The arrows identify acrylic material from the hip arthroplasty, which was damaging the nerve

Sciatic nerve injury after hip arthroplasty. (a) The skin incision for the transgluteal approach is in a continuous line. The cross on the left shows the ischium and the one on the right shows the trochanter. Between them, the skin projection of the sciatic nerve is seen. (b) The sciatic nerve was freed from all attachments. The arrows identify acrylic material from the hip arthroplasty, which was damaging the nerve

Piriformis Syndrome:

However, symptoms of sciatic neuropathy most often result from nerve compression by the piriformis muscle at the level of the sciatic notch, so-called piriformis syndrome.

piriformis

This presents with buttock tenderness and pain, radiate down the posterior thigh.  Symptoms are made worse by prolonged sitting, bending at the waist, and activities involving hip adduction and internal rotation.  The pain can be reproduced by deep palpation over the sciatic notch.

Diagnostic modalities such as CT, MRI, ultrasound, and EMG may all be normal in piriformis syndrome, but are still useful for excluding other conditions.

Magnetic resonance neurography is a specialized imaging technique which can confirm the presence of sciatic nerve irritation or injury of the sciatic nerve in the piriformis muscle.

Magnetic resonance neurography findings in piriformis syndrome. A: Axial T1-weighted image of piriformis muscle size asymmetry (arrows indicate piriformis muscles). The left muscle is enlarged. B and C: Coronal and axial images of the pelvis (arrows indicate sciatic nerves). The left nerve exhibited hyperintensity. D: Curved reformatted neurography image demonstrating left sciatic nerve hyperintensity and loss of fascicular detail at the sciatic notch (arrows). Filler AG, Haynes J, Jordan SE, et al, "Sciatica of nondisc origin and piriformis syndrome: Diagnosis by magnetic resonance neurography and interventional magnetic resonance imaging with outcome study of resulting treatment," J Neurosurg Spine 2: 99-

MRN findings in piriformis syndrome. A: Axial T1-weighted image of piriformis muscle size asymmetry (arrows indicate piriformis muscles). The left muscle is enlarged. B and C: Coronal and axial images of the pelvis (arrows indicate sciatic nerves). The left nerve exhibited hyperintensity. D: Curved reformatted neurography image demonstrating left sciatic nerve hyperintensity and loss of fascicular detail at the sciatic notch (arrows).

Conservative treatment can include medications, physical therapy and stretching, or injection of a paralysing agent such as botulinum toxin into the piriformis muscle under ultrasound or CT control. Surgery may be necessary for recalcitrant cases.

Phantom Limb Pain

This post is provided by Ilya Shnaydman, Drexel University College of Medicine Class of 2013:

Phantom Limb is the sensation that an amputated limb is still attached to the body. It may occur after removal of other organs such as breast, eye, teeth, etc. It can even occur after a hysterectomy, where patients may suffer “phantom menstrual cramps.”  Approximately half of patients with phantom limb feel that they can move the missing body part, and the other half feel that the phantom limb is there, but “paralyzed” and frozen in space. These patients feel that if they could only relax the body part they would feel a great deal of relief. This is especially common if the body part is in a contracted, or fixed state prior to the amputation.

In the case of a paralyzed leg for example, the patient’s brain is sending signals telling the (paralyzed) limb to move, but since the leg can not move, the patient does not get the visual feedback of a moving limb. This leads to a “learned” pattern of paralysis. After an amputation, often this learned paralysis can remain with the patient feeling a clenched spasm of the missing extremity. The limb often feels as if it is burning, aching, in a painful position, or having electric-type pain. Phantom Limb Pain can occur anywhere from just after the amputation to even years later.

Our brains are hardwired from birth with sensations reaching the brain through predefined pathways. When you touch an object with your finger, those sensations reach the brain through complex pathways ending in a specialized area of the brain responsible for perceiving sensation. If that finger tip was amputated, and the remaining finger were to touch an object, the brain may perceive it as being the fingertip due to the similarity in the sensory pathways.

There are several theories for why phantom limb pain occurs. One theory is that the pain is caused by irritation of the severed nerve endings. The nerve endings can form a neuroma, or an abnormal growth of nerves. Some believe that the brain perceives these ‘nonsense’ stimulations as pain. This theory led to many patients undergoing revisional surgeries hoping to remove the inflamed nerve endings. Unfortunately in most cases it rarely helped.

Research at the National Institutes for Health (NIH) showed that the area of the brain responsible for interpreting sensation (primary somatosensory cortex) underwent reorganization after the loss of sensory input, as occurs after an amputation.  Phantom Limb Pain results when a conflict between signals received from the limb and a lack of visual input from the missing limb.

Stump Pain can result from other causes such as ischemia (lack of blood flow to the stump), infection, or pressure points over bony spurs. Phantom limb can only be diagnosed if all other causes of stump pain are ruled out.

The incidence of phantom limb pain varies from 50-85% depending on the diagnostic criteria used to define the syndrome. A minority of patients have such severe pain that it interferes with work, sleep and daily social life. The pain can be worsened by stress, anxiety and even weather changes. Phantom limb pain can be quite severe, leading to depression and even suicide.

Treatment

As with other forms of neuropathic pain, the treatment of Phantom Limb Pain has included pain medication, antidepressants, anticonvulsants, spinal cord stimulation, vibration therapy, acupuncture, hypnosis and biofeedback under the guidance of an experienced neurologist.

Biofeedback helps teach amputees with burning/tingling pain to unconsciously keep their phantom limb as warm as the intact limb. For cramping pain, the goal is to teach patients to prevent the onset of muscle tension leading to pain. Patients are hooked up to a biofeedback machine which consists of electrodes places on the body. The patient is then shown the relationship between temperature or muscular activity and the onset of phantom pain. Once they are convinced of this relationship, they undergo various exercises to increase their temperature/muscle tension awareness. After some time patients are able to sense these changes and control them effectively.

Some studies have shown that calcitonin (a hormone naturally occurring in the body that regulated calcium metabolism) and ketamine (an anesthetic drug) are effective in treating Phantom Limb Pain. If all other methods have failed, surgical intervention may be indicated.

Mirror Box Therapy

A novel way of relieving the clenched pain that patients with phantom limb face was created by  Vilayanur S. Ramachandran. He proposed placing a mirror between the patient’s two limbs, tricking the eyes into seeing that the amputated limb is actually still there.

The ‘phantom limb’ is placed behind the mirror, and the normal limb on the other side. The patient then makes the same clenched position with their normal limb. This brings the visual impression that the phantom limb is still there. By relaxing their normal limb, patients can trick their brain into relieving the pain of the phantom limb. This shows how the theory of  ‘learned paralysis’ applies to phantom limb and how it can be overcome with a simple visual aid.

If you have any questions about this condition, please comment below!