Drooping eyelids (ptosis)

Ptosis can affect one or both eyes and results from weakness affecting the muscles that raise the eyelid.

L ptosis

Left sided ptosis

Ptosis can be congenital (you are born with it), or acquired (it develops during life).

Acquired ptosis can result from a variety of problems affecting the nerves, muscles, neuromuscular junction or tendons involved in elevating the eye lids.

Neurogenic ptosis is usually unilateral, and can be caused by a lesion affecting either the oculomotor nerve or the sympathetic nerve fibers to the eye (Horner’s syndrome).

When ptosis is caused by an oculomotor nerve lesion, there is  generally also some degree of eye movement abnormality (ophthalmoparesis).  Oculomotor nerve palsy can be caused by something as simple as diabetes, but if the nerve fibers to the pupil are involved (causing a dilated pupil in addition to the ptosis and ophthalmoparesis), that is very suggestive of a compressive lesion such as an aneurysm (see below) and warrants immediate evaluation.

R ptosis, occulomotor palsy (eye is deviated down and outwards), with a dilated pupil, caused by nerve compression from aneurysm (red arrow)

R ptosis, oculomotor palsy (eye is deviated down and outwards), with a dilated unreactive pupil, caused by nerve compression from aneurysm (red arrow)

Horner’s syndrome causes mild ptosis associated with a small pupil (miosis) sometimes associated with lack of sweating (anhidrosis) on the face, and can be caused by trauma to the carotid artery, lung tumors, or strokes.

L Horner's syndrome with mild ptosis and miosis

L Horner’s syndrome with mild ptosis and miosis

Myasthenia gravis is an autoimmune disease that affects the neuromuscular junction, and frequently presents with fatiguable ptosis often associated with double vision and limb weakness.   The ptosis will usually get worse when the patients is tired at the end of the day (diurnal variation):

Fatiguable ptosis in myasthenia gravis

Fatiguable ptosis in myasthenia gravis

The ptosis of myasthenia can be temporarily improved with an acetylcholinesterase inhibitor medication, such as an injection of edrophonium (Tensilon), and this can used a diagnostic test.

Ptosis can also be seen in certain muscle diseases, including oculopharyngeal muscular dystrophy, mitochondrial myopathy and myotonic dystrophy.

Bilateral ptosis in a patient with myotonic muscular dystrophy

Bilateral ptosis in a patient with myotonic muscular dystrophy

However, acquired ptosis is most commonly caused by dehiscence or disinsertion of the levator aponeurosis, causing a disconnection between the eye lid and the elevating muscles.

This usually occurs in elderly patients, but can sometimes affect younger contact lens users.

L ptosis from levator dehiscence - Note that when the eye is closed, the lid crease is fainter and further away from the lid margin in the left eye, compared to the right eye

L ptosis from levator dehiscence – Note that when the eye is closed, the lid crease is fainter and further away from the lid margin in the left eye, compared to the right eye

Patients who notice a drooping eyelid, unequal pupils, or double vision should consult with a neurologist in order to establish the correct diagnosis.

After that, treatment might include medical therapy for an underlying disorder (such as diabetes or myasthenia), surgery or even eye lid crutches:

Myasthenic patient with isolated L ptosis, demonstrating improvement with the eye lid "crutch"

Myasthenic patient with isolated L ptosis, demonstrating improvement with the eye lid “crutch”

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Surgical options for trigeminal neuralgia

Trigeminal Neuralgia

Trigeminal neuralgia is characterized by recurrent short episodes of one-sided zapping, shooting, or excruciating spasms of facial pain. The pain may be so severe that the patient may cry out or visibly wince. The affected area of the face may become super-sensitive, with such minor stimulation as a light breeze, cold temperature, water from a shower, washing the face, shaving, or even eating can set off attacks of pain.

Medical Treatment of Trigeminal Neuralgia
The anticonvulsant carbamazepine is usually first line treatment.
Second line medications include baclofen, lamotrigine, oxcarbazepine, phenytoin, gabapentin, pregabalin, and antidepressants such as amitriptyline.
However these medications are not always effective and can cause side effects such as confusion and unsteady gait.

Surgical Treatment of Trigeminal Neuralgia
Surgical treatment, known as microvascular decompression, involves sub occipital craniotomy, mobilizing the offending blood vessel off the nerve, and insertion of a sponge to separate these structures.

Partial lesioning of the trigeminal nerve, either by radiofrequency ablation using a percutaneous needle or gamma knife radiosurgery is less invasive and can also be very effective:

Gamma knife for trigeminal neuralgia patient story:
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Diabetic Neuropathy, You’d better be checking your blood sugars!

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Diabetes causes high blood sugar levels, either from lack of insulin (type I diabetes) or insulin resistance (type II diabetes).

Many diabetics develop numbness, tingling, neuropathic burning pain, or weakness that starts in the feet and progresses up the legs (“glove and stocking” pattern) from diabetic polyneuropathy.

Neuropathic pain in feet

Neuropathic pain in feet

Loss of protective sensibility from neuropathy can cause painless injuries that result in ulceration, infection and bony deformity “Charcot joints”.

Diabetic ulcer

Diabetic ulcer

Diabetic foot deformity, Charcot joint

Diabetic foot deformity, Charcot joint

There are many medications that can reduce the symptoms of neuropathic pain, such as gabapentin, pregabalin and duloxetine.

However, the only treatment that has been shown to be effective for diabetic neuropathy is improved control of blood sugar.

While many patients and their doctors rely on the glycosylated hemoglobin to measure how well their diabetes is controlled, it is important to recognize that this is an average measure of blood glucose levels, and patients with mild diabetes can have peaks (which are neurotoxic) and troughs (which can also be harmful), which average each other out, leading to a “normal” glycosylated hemoglobin level , and a false sense of security that everything is OK.

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Blood sugars – you can have a normal “average” level, but still get nerve damage from high peaks after you eat

If you have diabetic neuropathy, we suggest that you check your finger stick glucose regularly and write the numbers down in a book or on a computer spreadsheet, so that you can review them with your doctor.

We usually suggest the following schedule to our patients:

Pick the same 2 days each week (no matter what you have eaten those days) to do the test.  On those days, check a “fasting” sugar when you first wake up (should be <130, ideally <110), a second sugar test before dinner (should be <130, ideally <110), and a third test 2 hours after dinner (should be <180, ideally <140).

Remember, although improved blood sugar control will prevent progression of your neuropathy, it won’t make it better, so start today before things get too bad!

MMC Uses New Treatment for Brain Hemorrhage

Background:

Spontaneous (non-traumatic) intracerebral hemorrhage (ICH) is a type of stroke caused by bleeding into the brain.

ICH typically causes sudden headache with stroke like symptoms.

ICH accounts for 20% of all strokes.

The mortality rate of ICH is 40%, and more than 20% of survivors are left with significant disability. Death and disability is more common with larger or expanding bleeds.

ICH is more common in patients who are taking blood thinners like warfarin (Coumadin).

Warfarin associated ICH is more likely to expand from continued bleeding leading to a worse outcome or death, so it is very important to reverse the effects of warfarin as quickly as possible in these patients.
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An injection of Vitamin K will reverse the effects of warfarin, but it takes 12-24 hours to work.

Fresh frozen plasma (FFP) will reverse the effects of warfarin more quickly than vitamin K, and this has been the standard treatment for warfarin associated ICH, but FFP is a blood product that requires cross-matching and has to be thawed out and administered slowly.

Studies have shown that many patients treated with Vitamin K and FFP have not yet had the effects of warfarin completely reversed even by 24 hours after admission, and are still therefore at risk for hematoma expansion, death and disability.

Prothrombin Complex (PCC) is a new concentrated plasma protein product that can given by immediate intravenous injection, and along with Vitamin K and a small amount of FFP reverses the effects of warfarin within minutes, preventing ICH expansion and improving survival in ICH patients.

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We now have PCC (Profilnine) on formulary at Monmouth Medical Center for this situation.

Case Report
A 75 year old hypertensive woman who was taking warfarin because of previous stroke was brought to our emergency room with headache and confusion. A brain CT showed ICH and her INR was modestly elevated at 1.7.

Baseline brain CT showing intraventicular blood of different ages

Baseline brain CT showing intraventicular blood of different ages

She was appropriately given 10mg of Vitamin K, 25U/Kg of Profilnine and 2 units of FFP, her INR was normalized within hours, and she did very well clinically, with some evidence of ICH resolution on a follow-up CT scan 2 days later.

Follow-up brain CT, NOT WORSE, actually showing interval improvement

Follow-up brain CT, NOT WORSE, actually showing interval improvement

This favorable outcome was the direct result of great communication and cooperation between our emergency room, ER and ICU nursing staff, pharmacy and physicians with regard to this new approach for treating warfarin associated ICH.

Click here for further information about Monmouth’s fully accredited stroke program.

A Comprehensive Sleep Medicine Evaluation

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Sleep disorders are common and underdiagnosed. Studies have shown that initial consultation and follow-up with a fellowship trained sleep medicine physician is more likely to resolve symptoms than direct referral for a polysomnogram by a primary care physician.

If you think you might have a sleep disorder, ask your doctor for a referral to a sleep medicine specialist!

The Importance of Correct Positioning During Anesthesia

This post is provided courtesy of K. T. Weber, Drexel University College of Medicine Class of 2013:

When a patient goes under anesthesia, a highly trained team of people take over and monitor the body. This team controls breathing, circulation and many other processes, filling in very well for the patient’s brain… however, there is one function that cannot be replicated by the anesthesia team: movement. In order to undergo surgery, a patient must have their muscles relaxed, and therefore will not move away from sore or painful spots. It is normal for people to fidget, rearranging their weight to more comfortable positions, which helps to prevent bed sores (decubitus ulcers) as well as preventing minor nerve compression.

Decubitus (Pressure) Ulcer

Decubitus (Pressure) Ulcer

Decubitus (or “pressure”) ulcers can be a devastating complication for patients who undergo very prolonged surgeries – Superman actor Christopher Reeve actually died from complications associated with an infected pressure ulcer. Fortunately, these wounds are very rare in patients who are only temporarily relaxed for surgery, and there are many precautions in place to prevent this from occurring.

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Nerve Injury

However, minor nerve injury is still possible! One of the less commonly discussed side effects of having surgery, this minor nerve injury can be very disconcerting and affects a surprisingly large number of people

Nerve damage can occur in several forms based on the type and cause of injury:

The mildest form of nerve injury is neurapraxia, where the myelin sheath and support cells around the nerve are damaged (frequently by excessive pressure or ischemia), leading to poor signal conduction along an otherwise unharmed nerve. Neurapraxia improves on it’s own with time as the myelin sheath regenerates, and does not directly involve injury to the axon of the nerve.

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Neurotmesis, the most severe type of injury, occurs when a nerve is lacerated, over-stretched or very badly crushed, and the two ends of nerve are actually separated from each other.  These nerve injuries will not get better on their own without nerve graft repair surgery.

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Axonotmesis is an intermediate type of nerve injury, where the nerve is damaged (often by crushing) but the support cells around the nerve are intact and aid regeneration, so (slow) spontaneous recovery is possible.

The most common symptom of compressive nerve injury is numbness or pain. The tingling, pins-and-needles feeling of hitting one’s funny bone is a classic example of nerve pain.  Many patients who have undergone surgery and are held in one position for an extended period of time may wake up with areas on their skin that feel extra sensitive, like a sunburn. It is understandable to be concerned by waking up with hyperaesthesia, or pain in response to something that wouldn’t normally be painful! Weakness and uncoordination is less common, and only occurs after more severe or prolonged nerve compression during surgery.

The good news for patients who wake up with an unusually sensitive area or some numbness after having surgery is that this minor nerve damage tends to improve rapidly. Neurapraxia resolves as the swelling and bruise around the nerve decreases and the cells that help conduction recover, sometimes as quickly as a few hours, sometimes as slowly as several months. If necessary, non-steroidal anti-inflammatories, other medications for neuropathic pain and splinting can help treat the symptoms of mild nerve damage. Even more severe forms of nerve  injury can recover with time – peripheral nerves grow back around 1mm per day! In the most extreme cases, without the potential to regenerate on their own with time, nerve graft repair can be discussed as an option.

Watch a video presentation on nerve injury and repair:

Of course, an ounce of prevention is worth a pound of cure. The skilled members of the operating room staff take extensive measures to protect their patients with adequate padding and careful positioning.