2014, 100th Anniversary for Anosagnosia

anosagnosia

Anosagnosia

Here’s an illustrative example from a conversation with FD, an elderly woman who had had a right hemispheric stroke one week before, leaving her paralyzed on the left side and confined to a wheelchair:

How are you feeling today?

FD: I’ve got a headache.  You know, doctor, I’ve had a stroke so they brought me to the hospital.

Can you walk?

FD: Yes (FD had been in a wheelchair for the past week, and cannot walk)

Mrs D, hold out your hands.  Can you move your hands?

FD: Yes

Can you use your right hand?

FD: Yes

Can you use your left hand?

FD: Yes

Are both hands equally strong?

FD: Yes, of course they are equally strong.

Mrs D, point to me with your left hand.

FD: (Her hand lays paralyzed in front of her).

Mrs D, are you pointing to my nose?

FD: Yes

Can you see it pointing?

FD: Yes, it is about 2 inches from your nose.

Mrs D, can you clap?

FD: Yes, of course I can clap.

Mrs D, will you clap for me?

FD: (She proceeded to make clapping movements with her right hand, as if clapping with an imaginary hand near the midline)

Are you clapping?

FD: Yes, I am clapping.

xx

xx

The term anosagnosia was first used by Joseph Babinksi on June 11, 1914 in a brief communication presented to the Neurological Society of Paris.

Babinski

He described two patients with left hemiplegia who didn’t know they were paralyzed.  The word comes from the Greek words nosos, “disease”, and gnosis, “knowledge”.

Affected patients deny their deficit, and overestimate their abilities, they state that they are capable of moving their paralyzed limb and that they are not different than normal people.

Their false belief of normality persists despite logical arguments and contradictory evidence – they may even produce bizarre explanations to defend their convictions.

If they admit any impairments, they will attribute them to other causes (i.e. arthritis, tiredness, etc.).

xx

xx
But, this syndrome is not only seen with hemiplegia.

Visual anosognosia or Anton-Babinski syndrome is a rare neurological condition related to cortical blindness.  Affected patients deny their blindness and affirm adamantly that they are capable of seeing. The clinical presentation includes confabulations – instead of admitting blindness, they will make up answers when asked about what they see.

Mr Magoo is a great TV example of Anton’s syndrome – unaware of his loss of vision, he misinterprets and confabulates his way into trouble.

xx

Anosognosia may occur as part of receptive or Wernicke’s aphasia – affected patients cannot monitor and correct their own speech  errors and may appear angry and frustrated when the person they are speaking to fails to understand them.

xx

Watch this lecture, by Dr V. S. Ramachandran for more information on this fascinating syndrome:

Christmas Neurolgy

Animated_Xmas-tree-animation

xx

xx

Christmas Ornament from Cafe-Press

christmasmind_oval_ornament

xx

The Christmas Tree Sign

Keyoumars Ashkan and Adrian Casey

J Neurol Neurosurg Psychiatry 1999;67:824

xmas tree

Back pain and sciatica in patients with neurofibromatosis may result from a multitude of pathological processes which include scoliosis, degenerative diseases of the spine, and spinal tumors. Neurofibroma, schwannoma, meningioma, and glioma can all occur. When specific tumors are responsible for the symptoms, surgical excision can offer effective treatment. Many patients, however, have widespread disease. We recently treated a patient with multiple neurofibromas which involved several nerve roots and the cauda equine (figure). The extensive distribution of the tumors produced an MRI picture resembling a Christmas tree. In patients with “the Christmas tree sign” the challenge is in the correct diagnosis of those tumors which produce the symptoms if surgery is to remain a realistic management option.

xx

xx

A Christmas Tree Cataract

E Obi and C Weir

BMJ 2010; 341, (Published 8 December 2010)

cataratc

A 73 year old woman referred for cataract surgery had on examination a left Christmas tree cataract (fig 1A). Highly reflective, iridescent, polychromatic crystals were noted within the lens. Their colours varied according to the angle of the incident light, (fig 1B, C). We postulate that Christmas tree cataracts result from the accelerated breakdown of membrane associated proteins. The peptides and amino acids accumulate in the lumen of the reticular meshwork, and cystine is concentrated beyond the level of crystallisation, giving rise to growing crystals.

xx

xx

A Christmas Carol: A memorable patient

Stephanie Davies

BMJ. 1998 December 19; 317(7174):1732.

It was a few weeks before Christmas on a dull, chill November morning when Jason and his parents came to see me. As I listened to the, alas, too familiar tale of a young boy, difficult and unreasonable at home who was misbehaving at school, my heart sank and I began to feel as depressed and hopeless as everyone in the room. Jason sat quietly, certainly not demonstrating the hyperactivity suggested by his teacher. Assessment and examination showed that he was a normal and intelligent boy, so was this attention deficit hyperactivity disorder and would the magic pill help?

We discussed strategies to help based mainly on the premise of rewarding good behavior and ignoring the bad coupled with praise and appreciation. Unfortunately, at this time, there did not seem to be any good behavior to reward or any achievements to praise.

The review took place some time shortly before Christmas. I sat with prescription pad at the ready, prepared to offer methylphenidate if there was no other way. Jason came in like spring sunshine and his parents were glowing with pride and happiness. What could have wrought this transformation? It seemed unlikely that our previous consultation could have had such a dramatic effect.

I was correct. The magic had been woven by the muse of drama and Charles Dickens. Jason had starred as Scrooge in the school production of A Christmas Carol and was a smash hit. He had even saved the day by playing the part when a less intrepid thespian was incapacitated by stage fright.

Eight months passed before I saw Jason and his father again. With bated breath I inquired about progress. With delight I heard that it had been maintained, culminating in an excellent school report at the end of the academic year.

How important it is for us all to have some measure of success and how I wish all my patients could be a star for a day. I was certainly grateful that Jason and his parents allowed me to share his success. It has been one of my most memorable Christmas presents.

xx

xx

The Christmas “Clacker”

David Strachan

BMJ. 1994 December 24; 309(6970): 1747.

clacker

A 44 year old occasional drinker presented to the accident and emergency department at 5 30 am on New Year’s Day. A sensation at the back of his throat was causing a slight discomfort on breathing. On examination his uvula was found to be swollen. He had suffered the same problem on Christmas Day morning the previous year, the common factor being alcohol consumption during the previous evening. It is well known that alcohol consumption triggers or worsens snoring. Overindulgence causes muscle hypotonia and depression of the arousal mechanisms, leading to many people sleeping flat on their backs rather than on their sides. This narrows the airway and leads to a tendency to obstruction. If a kick in the back from a disgruntled partner does not rectify the situation and the “comatose” state persists then forced inspiration through a narrowed airway causes repeated trauma of the uvula and swelling.

xx

xx

Convulsions at Christmas

S Kirker

BMJ. 1992 December 19; 305(6868): 1580.

butcher

A healthy 26 year old butcher described convulsions without warning or focal onset between 11 am and noon on Christmas Eve in 1987, 1988, 1989, 1990, and 1991. He had no other convulsions or episodic symptoms that could be epileptic in origin and he had no relevant medical history. His usual working day was from 7 am to 6 pm but for the 14 days before Christmas he worked until at least 10 pm and as late as 1 am, seven days a week. He worked all through the night of 23 December. He did not miss any meals, and ate through the night as well. His usual alcohol consumption was 35 units a week but he drank less during these weeks. Results of examination and computed tomography were normal. Electroencephalograms in 1989 and 1992, each three months after the period of sleep deprivation, showed paroxysmal generalized spike and wave activity at 8 Hz, with a normal background rhythm. The patient was not prepared to change his working hours during the busiest time of year so we prescribed phenytoin over the second half of December. His electroencephalogram suggested he had an underlying tendency to have seizures, which only became apparent when his “seizure threshold” was lowered by sleep deprivation.

Left world neglected

Lhvf

Posted by Sanya Naware MSIV, Drexel University College of Medicine

What would it feel like to only perceive one half of the world around you?

For patients with hemispatial neglect, this is an everyday reality.  Hemispatial neglect or hemineglect is a condition in which damage to one hemisphere of the brain causes a lack of awareness of one contralateral side of space.  It is most often a lesion of the right posterior parietal cortex affecting the contralateral side of the body.  The person is unable to recognize stimuli or process them on the affected side.  Left neglect is more common than right neglect because the right hemisphere is able to compensate for the loss of left hemispheric function.

Because these patients only perceive one side, they only draw what we know to be half of an image as seen in the video and image below:

naware

Lisa Genova, a neuroscientist, expertly describes the daily challenges of living with neglect in her book Left Neglected. It is a difficult condition to imagine and this book does a wonderful job of explaining the realities and frustrations of the patient and her family.

naware2

The main character, Sarah Nickerson, suffers a traumatic brain injury in a car crash.  When she wakes up, everyone around her realizes that she ignores the left side of everything.  Whether it is a clock, a painting, or a room around her, she is not able to recognize the left side of anything.  While she is able to feel the left side of her body, she has to focus on the fact that she has a left side in order to control her left leg and walk.  In fact, when she first sees her left arm, she states that it feels like it belongs to another person, a problem called somatoparaphrenia.  While eating, she only eats the food on the right side of her tray.  She frequently bumps into objects on the left side of her body because she is unaware of their presence.

Sarah’s story is optimistic as her therapist and family use certain tricks to help her adjust.  Some of these methods include placing bright orange tape on the left side of things around their home, using a ruler to guide her to the left side of the page, and wearing shiny jewelry on her left hand to attract attention to it.

Genova ends her book by endorsing the New England Handicapped Sports Association (NEHSA), an organization of volunteers who help people like Sarah find some independence and confidence.

Click here to find out more about right hemispheric brain damage from NEHSA.

References

  1. Genova, Lisa. Left Neglected: A Novel. New York: Gallery, 2011.
  2. Waxman SG. Chapter 21. Higher Cortical Functions. In: Waxman SG, ed.Clinical Neuroanatomy. 26th ed. New York: McGraw-Hill; 2010.

Memory misplaced

bourne1

Post written by Dr. Farida A. Malik , PGY3 Medical Resident, Monmouth Medical Center

Case Summary

This 69 year old lady had a remote history of breast cancer, hypertension and hypothyroidism.  She was brought to the Emergency Room by her husband because of abrupt onset confusion after waking up that morning. She was disoriented and was noted to ask the same questions over and over again. She had no difficulty walking, talking or dressing herself. She denied having headache or visual problems. There was no history of head trauma, seizures or any prior similar episodes.

When she was seen in the in the ER she knew her name and recognized her husband.  She was able to follow simple commands.  She had no recollection of events since morning or the day before. She repeatedly asked how she got to the hospital, despite being told several times that her husband brought her. Neurological examination otherwise was unremarkable.

CT scan of head, MRI of the brain and EEG were all normal.

She was diagnosed with TRANSIENT GLOBAL AMNESIA.

xx
The family was reassured about the benign nature of the condition and she was discharged home the next day still with memory lag.

Discussion

Transient global amnesia (TGA) is a clinical syndrome of reversible anterograde amnesia accompanied by repetitive questioning that occurs in middle-aged and elderly individuals.

The incidence of TGA is 5.2 to 10 per 100,000 per year overall, but 23.5 to 32 per 100,000 per year in adults aged 50 and over.

During a TGA episode recall of recent events simply vanishes. One may also draw a blank when asked to remember things that happened a day, a month or even a year ago.  Unlike “soap opera amnesia” (Jason Bourne) affected patients do remember who they are and recognize the people they know well.  But that doesn’t make their memory loss less any less disturbing.

Fortunately, episodes are usually short-lived, recover spontaneously, and are unlikely to recur.

The precise cause of TGA is unknown.  Atherosclerotic risk factors (eg. hypertension, diabetes, hypercholesterolemia) are not associated with TGA.
However there may be a link between TGA and history of migraines.

The primary site of neurologic functional disturbance is the medial temporal lobe and hippocampus.
The_hippocampus
The pathogenesis of this transient disruption is unknown. Current theories include arterial ischemia, venous congestion, and migraine, but no theory explains all of the clinical features.

The diagnosis is made by the following signs and symptoms:

  • Sudden onset of memory loss, verified by a witness
  • Retention of personal identity despite memory loss
  • Normal cognition, such as the ability to recognize and name familiar objects and follow simple directions
  • Absence of signs indicating damage to a particular area of the brain, such as limb paralysis, involuntary movement or impaired word recognition
  • Duration of no more than 24 hours
  • Gradual return of memory
  • No evidence of seizures during the period of amnesia
  • No history of active epilepsy or recent head injury

Some common triggers identified are:

  • Sudden immersion in cold or hot water
  • Strenuous physical activity
  • Sexual intercourse
  • Medical procedures, such as angiography or endoscopy
  • Mild head trauma
  • Acute emotional distress, as might be provoked by bad news, conflict or overwork

There are no confirmatory diagnostic tests. The initial evaluation and management of patients with TGA focuses on excluding other diagnoses and should include the following:

  • If the patient is symptomatic on presentation, the patient should be observed in the hospital until the amnesia resolves.
  • Diagnostic testing includes oxygenation status, serum electrolytes, glucose, and a toxicology screen.

The need for further testing varies depending on the circumstances, such as how typical the event is for TGA, the presence of vascular risk factors, and whether the ictus was observed. Patients with recurrent or brief episodes, or activity suggesting motor automatism should be evaluated with EEG for possible epilepsy. A neuroimaging study may be performed in all patients, preferably a brain MRI with DWI, to exclude acute ischemia, head trauma, and other causes.

Treatment is not required for TGA. The condition usually does not recur, and the patient does not need to be restricted from driving unless events are recurrent.

There is no increased risk of mortality, epilepsy, or stroke following TGA as compared with age-matched controls.

2012-13 Neurology Student Research Presented at Drexel University College of Medicine

Monmouth neurology students presenting their posters during medical student research day at Drexel University College of Medicine in Philadelphia on March 20, 2013:

medical-student-research-day-2

1: Addressing blood glucose control in diabetic peripheral neuropathy:  A missed opportunity for neurologists?

Darsi Pitchon and Seun Ku Kim

IMG_20130320_143958 IMG_20130320_152931

xxx

xxx

pichon1

Key points:

Most neurology visits for diabetic neuropathy did not include counseling about blood glucose levels, unless they were with a neuromuscular fellowship trained sub specialist.   Because neuropathy can be the presenting and/or predominant problem in diabetes, some of these patients may be primarily followed by their neurologist, so this is a missed treatment opportunity.

xx

xx

2.  Acute myelopathy with normal CSF and imaging:

Denis Chang

IMG_20130320_144226chang2

Chang

xx

Key Points:

Spinal cord infarction from fibrocartilaginous embolism can present with acute quadriparesis in young patients with normal CSF and MRI scans.  If this diagnosis is suspected, a follow-up MRI 2-3 days can be quite helpful.  This is not an inflammatory process, and will not improve with steroids or other immunosupressive medications, which can hurt more than they help.

xx

xx

3. The benefits of an on-line neurology clinical course for 4th year clerkship students

Ilya Grinberg

IMG_20130320_144208

ginsberg2

Key points:

The on-line video course used by the Monmouth neurology clerkship led to improved test scores on an on-line clinical test compared to students who did their clerkship at other sites.  The on-line clinical test is a low cost but effective method of evaluating students’ clinical skills in neurology over multiple clerkship sites.

Parsonage-Turner Syndrome

shoulder pain
Case Report and discussion prepared by Sidra Ghafoor, Drexel University College of Medicine Class of 2013.

Case: 65 yr old male presents with painful weakness, wating and tingling in the right arm.

When did it all begin? Three months ago patient experienced sudden onset severe pain down his neck accompanied by a tingling sensation in his right forearm and thumb. There was no apparent cause for his symptoms (i.e. no trauma). After 3-4 weeks, his pain miraculously disappeared! Unfortunately, once the pain subsided, he began experiencing weakness in the right shoulder/upper arm and right hand.

Physical Exam (*all findings pertain to patient’s right side*)

  • Muscle Bulk/Tone: Atrophy of supraspinatous, infraspinatus, deltoid, and biceps          
  • Muscle Strength
    • Weakness of supraspinatus, infraspinatus, biceps, brachioradialis, and pronator teres
    • Normal strength of Rhomboids, trceps, wrist and hand muscles    
  • Sensation: Numbness over lateral arm, forearm, and anatomic snuffbox             
  • Reflexes: Absent biceps and brachioradialis

Parsonage-Turner Syndrome (Neuralgic Amyotrophy, Brachial Neuritis)

    • Incidence: relatively rare (1-2 cases per 100,000)
    • Sex/Age
    • Cause: unknown etiology – but linked to recent illness/events:
    • Clinical Correlation
      • Affects Lower Motor Neurons of brachial plexus and/or individual nerves or nerve branches (this explains the patchy clinical findings on exam).  The suprascapular nerve long thoracic nerve, phrenic nerve, and branches of the median nerve are nearly always affected:
Brachial neuritis, wasting of the shoulder girdle muscles

Brachial neuritis, wasting of the shoulder girdle muscles innervated by the suprascapular nerve.

Brachial neuritis, winged scapula

Brachial neuritis, winged scapula from involvement of the long thoracic nerve

Brachial neuritis, elevated hemidiaphragm from involvement of the phrenic nevre

Brachial neuritis, elevated hemidiaphragm from involvement of the phrenic nerve

  • Clinical Sequence of Events
    • (1) Acute onset severe unilateral pain (throbbing, aching) in shoulder/arm
    • (2) Pain resolves within 1-3 weeks
    • (3) weakness/wasting of involved muscles and variable sensory impairment
  • Prognosis
    • Full recovery in majority of cases by 6 months to 1 year
    • Rarely recurrent (exception: familial neuralgic amyotrophy is typically recurrent)
  • Diagnosis
    • Clinical Clues: acute onset, pain followed by weakness & impaired sensation, no preceding trauma
      • aggravated by movement but not by coughing, sneezing, laughing (this helps to differentiate it from Cervical Radiculopathy in which pain is aggravated by coughing/sneezing/laughing)
    • Physical Exam Clues: patchy involvement of nerves
      • affected arm is internally rotated and adducted at shoulder.  flexed at elbow.
    • EMG: localizes lesion to brachial plexus and differentiates it from Peripheral Focal Neuropathy and Cervical Radiculopathy
      • 1 wk after onset: Loss of sensory and motor amplitudes with relatively normal conduction velocity
      • 2-3 wks after onset: shows denervation (fibrillations, positive sharp waves, and/or motor unit potential changes) in affected muscles
  • Management

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.
Untitled-1 copy

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.

Untitled-2 copy

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.