Parsonage-Turner Syndrome Revisited

Posted by Daniel Rubio, Drexel University College of Medicine Class of 2014

Parsonage-Turner Syndrome (PTS) is an inflammatory disorder that affects the brachial plexus an important network of nerves which lies deep in the armpit (axilla) giving off nerve brachnes including the axillary, radial, musculocutaneous, ulnar and median nerves which supply power to the shoulder and entire upper extremity.

What does PTS look like?

Unlike other brachial plexopathies, PTS begins spontaneously, without any prior injury to the arm, neck, or axilla.  The classical presentation is severe pain followed by patchy weakness in the shoulder, biceps, and the muscles controlling the thumb and first two fingers (index and middle).  It may also present with a finding known as winged scapula: the shoulder blade sticks out more from the back especially when pushing yourself off a wall.  Weakness may be so severe that the muscles may actually shrink (atrophy).  Pain may be found in the shoulder and along the outside of the upper arm and the thumb-side (lateral) of the forearm and hand.  Pain symptoms usually occur before the weakness and may last up to 4 weeks.  Patients may experience alteration in sensations in the upper extremity, specifically increased sensitivity to touch and temperature and/or tingling.  Symptoms may affect one or both sides, but they usually are asymmetric if they both sides.

 

What causes PTS?

Approximately 50% of patients describe some type of stressful event or illness prior to the onset of symptoms: infection, exercise, surgery, pregnancy, or vaccination.

Diagnosing PTS

Your neurologist can make the diagnosis based on signs and symptoms (especially if you do the above dance); however, sometimes further testing is required to ensure accurate diagnosis.  Suspicion of PTS should occur based upon pattern of initial sudden and severe pain followed by weakness in the upper extremity and slow recovery.  The neurologist may use nerve conduction studies and needle electromyography to document denervation to support clinical suspicion.  Blood tests and imaging rarely help make the diagnosis of PTS.

Help doctor! Fix me?

There is currently no specific treatment for PTS and management usually involves symptom relief.  Pain relief with short course of narcotics may be necessary.  A short course of steroids may be given, which may or may not help relieve symptoms or hasten recovery.  Physical therapy may be prescribed to maintain range of motion and decrease risk of atrophy.  Despite the above measures, there is no treatment to quicken recovery.

When will I be cured?

Recovery of symptoms begins 1-3 months following onset of symptoms; however, maximal recovery may take up to 1-3 years and some patients may be left with residual symptoms.

New Interactive eLearning Format

Take a tour of a new eLearning format we’re working on, and learn the basics of neurologic localization:

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.

face

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:

tick

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.

 

Acute Back Pain, What Not To Do!

back pain

70-80% adults have experienced acute back pain, almost 30% seek medical attention, and this problem is one of the commonest reasons for a doctors’ office visit.

Most cases are caused by sprains or tears in one of the numerous muscles or ligaments in the back triggered by twisting or lifting something heavy.

back muscles

These “soft tissue” injuries will usually improve on their own within a few weeks with anti-inflammatory medications and physical therapy.

However a recent study showed that more and more such patients are getting unnecessary imaging studies right away leading to surgeries and other invasive procedures that they don’t need.

Possible reasons cited for the necessary procedures include patient expectations and financial incentives for doctors.

flag_status_redDoctors shouldn’t immediately order an MRI or CT scan to determine the cause of back pain if a patient doesn’t have any red flags such as tingling in the legs — a sign of a nerve problem such as spinal stenosis — or a previous history of cancer.

Otherwise, imaging studies ordered for nonspecific back pain may reveal incidental disk problems, the result of aging, and not the cause of the symptoms.

disc bulge

This will then often lead to unnecessary and unproven interventional pain management procedures.

Most back pain patients simply need to be told that their pain will improve with antiinflammatory medications, physical therapy, massage therapy, and/or supervised exercise programs.

However, in the words of Dr. John Mafi, one of the study’s authors, “it takes longer to sit and reassure patients that their pain will likely resolve on its own than it does to order an MRI.”

Prescription Pain Meds, Sometimes A Necessary Evil

pain meds

NPR recently aired an interview between NY Times reporter Barry Meier and Fresh Air’s Terry Gross regarding prescription pain medicine abuse and his new ebook – “A World of Hurt: Fixing Pain Medicine’s Biggest Mistake”.

Mr Meier suggested that physicians should be treating the actual cause of pain rather than just prescribing pain medications, that patients can reduce chronic pain by exercise, and questioned the role of drugs like Oxycontin for treating chronic pain.

In fact this is just one of many recent backlashes against doctors for prescribing narcotic medications. Doctors who prescribe narcotic pain medications now face multiple telephone calls from individual and insurance company pharmacists urging them to taper narcotic medications or try something else.

It is true that there has been a marked increase in the use of prescription narcotic medications for chronic non-cancer pain over the last 2 yrs, and this has led to many cases of prescription medication abuse, including unintentional overdose deaths:

narcotic1

Source: Stanos, Phys Sportsmed. 2012 Nov;40(4):12-20.

There have certainly been many instances of rogue physicians writing narcotic prescriptions for cash during walk-in visits in so-called pain pill mills.

Critics of prescription narcotics have cited a concern about the lack of rigorous scientific outcome data showing any long term benefit from the use of narcotics for non-cancer related chronic pain.

Obviously, physicians should be encouraged to investigate the cause of chronic pain and look into treatments other than chronic pain medications.   There are now many “pain management physicians” who will only treat back pain patients with interventional procedures and won’t prescribe narcotics.  However, there iis little to no data demonstrating any long term outcome benefit from epidural injections for back pain.  Furthermore, each of these procedures cost health care carriers up to a thousand of dollars.  There can be no doubt that there are unethical physicians seeking insurance reimbursed for unnecessary procedures, just as their are unethical physicians writing narcotic prescriptions for cash.

There are studies that show chronic pain is under-treated in the community leading to poor quality of life, disability, and healthcare over-utilization.

It is clear that there are many patients currently being treated by ethical physicians for chronic pain with long-acting narcotic medications, and we need to be more careful that increased regulation does not deprive these legitimate pain patients of a necessary evil.  There are many anecdotal examples of physicians who won’t prescribe narcotics or take on new patients who are already taking these medications for fear of state medical board scrutinization or action.

Clearly, we need some kind of a compromise here:

Yes, physicians should investigate the cause of pain, and if possible treat that underlying problem rather than just prescribing pain medications.

Nevertheless, it is not good medicine to let patients suffer needlessly while this is being accomplished.

Physicians need to know it’s OK to prescribe pain meds as long as they take steps to be sure they are doing their best to only prescribe narcotics to legitimate patients without causing drug addiction and overdose:

Ten steps of universal precautions in pain medicine management, from Brennan J: Multidiscip Healthc. 2013 Jul 23;6:265-80.

The media and regulatory authorities needs to do a better job distinguishing appropriate from inappropriate prescription narcotic use.

One wonders if reporters who write about prescription drug abuse have ever been unfortunate enough to experience severe pain themselves and then encountered physicians too afraid to prescribe pain medications?

A less invasive surgical treatment for spinal stenosis?

…..The jury is still out!….

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First, the problem:

Lumbar spinal stenosis is an abnormal narrowing (stenosis) of the spinal canal that can lead to compression of the cauda equina, resulting in pain, numbness, and weakness in the legs.

spinal stenosis

Affected patients complain of back pain, leg pain and weakness, worse with prolonged standing and walking, better with sitting or flexing the spin forward (such as pushing a supermarket trolley).

stenosis2

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Next, the solution(s):

This stenosis (or narrowing) is caused by degenerative bone spurs, disc disease and ligamental hypetrophy.  While many patients try conservative measures including physical therapy and epidural injections, spinal stenosis is a mechanical problem, and usually necessitates a mechanical (surgical) solution to alleviate symptoms.  Consider the analogy of a blocked sewer pipe – you can try pouring chemicals down the sink, but ultimately you know you are going to need to call a plumber  (Rota-Rooter)!

blocked pipe

Traditional surgical management calls for a lumbar laminectomy to decompress the spinal canal and alleviate symptoms.

In some cases,  laminectomy can leave the spine unstable necessitating a spinal fusion using bone garft, rods and screws (“hardware”) done at the same time, and this major surgery can lead to an extended recovery period of months to a year.

Implantation of a interspinous spacer has been proposed as a less-invasive alternative surgical option than spinal fusion:

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Which is better?

A recent study compared the outcomes of these techniques in 99,000 Medicare patients who underwent surgery for spinal stenosis from 2006 to 2009.

6,000 patients who underwent implantation of new interspinous spacers were compared with 76,000 who underwent laminectomy, and 17,000 patients who underwent laminectomy with spinal fusion.

The results showed that patients treated with spacers had the lowest rate of major medical complications: 1.2%, compared to 1.8%with laminectomy and 3.3% with spinal fusion. Patients receiving spacers also spent less time in the hospital: average 1.4 days versus 2.7 days in the decompression group.

However, patients receiving spacers had the highest rate of revision surgery – about 17% of patients receiving spacers needed a second operation on the lumbar spine, compared to only 8.5%  in the laminectomy group and about 10% in the fusion group.

Patients receiving spacers tended to be older and to have other medical problems. With adjustment for these and other factors, patients in the spacer group were more than twice as likely to require repeat surgery.

Hospital payments for spacer surgery were greater for decompression alone, but less than for fusion procedures

Other than the need for repeat surgery (presumably because initial surgery was ineffective), the study did not look at pain control or functional outcome between the groups.  More data is needed.

Nevertheless, so far it looks as if the higher cost and higher re-operation rate with spacers may argue in favor of conventional decompression surgery, except in higher risk older patients with medical problems.