Gamma knife tremor patient comes back to get the other side done!

Our first tremor patient treated with gamma knife radiosurgery recently came back, one year later, to have the other side treated.
He has essential tremor, which was affecting both arms.
He had undergone treatment to the left brain for right sided tremor last year.
He was so pleased with his results, he recently came to have the right brain treated to address the left sided tremor.
Here is his most recent video.
Note the action and postural tremor on the left (untreated) side, and the fact that he has almost not residual tremor on the right (treated) side.

Click here to find out more about Gamma knife radiosurgery for tremor at the Monmouth Neuroscience Institute.

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Limbic Encephalitis

Post prepared by Precious Ramirez-Arao, Monmouth Medical Center PGY3

A 60 year-old female was found lethargic lying in a pool of feces by roommate.

EMS was called and was immediately brought to the hospital.

In the emergency department she had a witnessed generalized tonic-clonic seizure.

Her roommate relates she had episodes of confusion and short-term memory loss over the past few weeks.

She remained lethargic over the next 72 hours in the hospital.

48-hour EEG monitoring showed diffuse 2 to 3 Hz delta slowing with periodic lateralized epileptiform discharges emanating from the left frontal temporal region.

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T2 weighted image of the brain showed signal abnormality of the left mesial temporal lobe and the pulvinar with diffusion restriction in the left hippocampus consistent with limbic encephalitis.

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Limbic encephalitis (LE) is a subacute syndrome of seizures, personality change and cognitive dysfunction, typically evolving over days to weeks.

Autoimmune and paraneoplastic forms have been described. The most common neoplasms associated with paraneoplastic LE are lung cancer (usually small cell), thymoma, ovarian or testicular teratoma, breast cancer and Hodgkin lymphoma. The associated autoantibody depends on the tumor type. Lung cancer and thymomas are associated with anti-VGKC while ovarian or testicular teratomas are associated with antiNMDA antibodies.

Neurologic symptoms can precede oncologic diagnosis for several months to years and initial CT scans are typically unrevealing.

Nevertheless, prompt and thorough evaluation for malignancy including PET and CT scan of the chest, abdomen and pelvis should be initiated. Symptomatic treatment includes corticosteroids, plasmapharesis and intravenous immune globulin.

 

Diagnosis of Myasthenia Gravis Confirmed with Tensilon Test

Post prepared by Dr Mrugesh Panchani, PGYIII Medicine Resident, Monmouth Medical Center.

For patients with clinical features and signs suggestive of myasthenia gravis, the Tensilon test can serve as an easy bedside confirmatory test.

Edrophonium (Tensilon) is an acetylcholinesterase inhibitor and we have already blogged about how these drugs can improve conduction across the neuromuscular junction in myasthenics.

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Tensilon is preferable for diagnostic testing because of the rapid onset (30 s) and short duration (5 min) of its effect.

However, an objective end-point must be selected to evaluate the therapeutic effect, such as improvement in eye movements, ptosis, impairment of speech, or the length of time that the patient can maintain the arms in forward abduction.

Here’s a real life example (we have a signed consent form on file):

The first video shows our patient at her baseline with R>L ptosis and facial weakness:

Then the patient is given a low dose (2mg) of iv Tensilon (with telemetry monitoring and bedside atropine at the ready) is given and we see a definite improvement in ptosis and a more expressionful face:

This test was considered positive and hence terminated.

Had there been no change, the patient would have been given an additional 8 mg of iv Tensilon.

We typically start with a low dose at first because some patients react to edrophonium with side effects such as nausea, diarrhea, salivation, fasciculation, and (rarely) bradycardia. Atropine (0.6 mg) should be drawn up in a syringe, ready for IV administration if these symptoms occur.

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.

 

Neuroscience Research A Big Hit At 2013 Resident Research Day!

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PGY III resident Dr Mohamed Sheta won overall best prize, as well as the medicine and cardiology prizes, for his research project analyzing the 6-month outcome data from Monmouth’s innovative new TIA Rapid Evaluation Center.

Unfortunately, Dr Sheta was not able present his outstanding data in person, but here are pictures of him presenting some of the same data at the 2013 International Stroke Meeting in Hawaii earlier this year (left) and also at the 2013 Drexel University Research Day (right):

sheta posters

Congratulations Dr Sheta!

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PGY I-III residents Drs Martin Miguel Amor, Paavani Atluri, Alan Hamza (left and right), Michael Chan (right only) and Mohamed Sheta (above) won the best poster award.

One of their findings was that most acute stroke patients not given thrombolytic therapy because of too “mild” a neurologic deficit still needed in-patient rehabilitation, so we should probably lower our threshold for administering the drug.

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Stroke Poster Revised

Congratulations to them!!

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We also had a neuroscience poster by PGY II residents Drs Nagakrishnal Nachimuthu and Michael Chan on an acute stroke case we have already featured on this blog:

Neuro case report final

We’re very proud to see our medicine residents so engaged in neuroscience research.

Keep up the good work!

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Prosopagnosia

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Memo to Hollywood: Don’t take it personally if Brad Pitt has no memory of meeting you. The World War Z actor, 49, apparently has a difficult time recognizing people’s faces. In fact, he thinks he may suffer from prosopagnosia, or face blindness.

Speaking about the problem in his much-talked-about interview for Esquire magazine’s June/July issue, Pitt says that even if he’s had a “real conversation” with someone, he’ll forget what the person looks like almost as soon as he or she walks away. “So many people hate me because they think I’m disrespecting them,” the actor confesses to the mag.

Prosopagnosia is a rare brain disorder that impairs the ability to recognize faces without affecting other aspects of visual processing.  It is usually caused by a lesion affecting the fusiform gyrus such as stroke or head trauma, although there are even rarer congenital cases.

Perhaps the best known case is “Dr. P.” in Oliver Sacks‘ 1985 book The Man Who Mistook His Wife for a Hat:

Dr P. was a musician of distinction, well-known for many years as a singer, and then, at the local School of Music, as a teacher. It was here, in relation to his students, that certain strange problems were first observed. Sometimes a student would present himself, and Dr P. would not recognize him; or, specifically, would not recognize his face. The moment the student spoke, he would be recognized by his voice …….. At first these odd mistakes were laughed off as jokes, not least by Dr P. himself …….. His musical powers were as dazzling as ever; he did not feel ill—he had never felt better ……. The notion of there being ‘something the matter’ did not emerge until some three years later, when diabetes developed. Well aware that diabetes could affect his eyes, Dr P. consulted an ophthalmologist, who took a careful history and examined his eyes closely. ‘There’s nothing the matter with your eyes,’ the doctor concluded. ‘But there is trouble with the visual parts of your brain ……… ‘What seems to be the matter?’ I asked him at length. ‘Nothing that I know of,’ he replied with a smile, ‘but people seem to think there’s something wrong with my eyes.’ ‘But you don’t recognize any visual problems?’ ‘No, not directly, but I occasionally make mistakes.’

Dr P. illustrates another important symptom in cognitive neurology, anosagnosia. Dr P. is himself completely unaware that he has a problem, he compensates without even knowing it.  It is his wife and students that encouraged him to seek medical attention.