What are the pitfalls and perils of intracranial pressure?

Crudely speaking, the nervous system is made up of two parts. The peripheral nervous system, composed of nerves and muscles, is rather robust and roams free, exposed to the elements. On the other hand, the central nervous system, consisting of the brain and spinal cord, is delicate and fragile. It is therefore protectively cocooned in a rigid skull and a hardy vertebral skeleton. But even this tough fortress isn’t secure enough for these dainty neurones; they are, after all, the command and control system for the whole body. Therefore, to further insulate them from the physical and physiological perturbations that continuously threaten them, nature has further sequestered them within a very exquisitely regulated irrigation system, the cerebrospinal fluid (CSF).

Internet Archive book Images on Flickr. https://www.flickr.com/photos/internetarchivebookimages/14769907251/

The CSF is actually a fine filtrate of the blood that flows in the arteries. The sieve is the very forbidding blood-brain barrier (BBB) which turns away all the blood cells, and carefully sets a target on how much protein and glucose to let in. The pressure within the CSF is also very finely tuned, not too high…and not too low; that is how the neurones like it.

 

By Dr. Johannes Sobotta – Atlas and Text-book of Human Anatomy Volume III Vascular System, Lymphatic system, Nervous system and Sense Organs, Public Domain, https://commons.wikimedia.org/w/index.php?curid=29135482

 

Alas, as with all systems, the CSF is vulnerable to external miscreants; infections such as meningitis,  encephalitis, and brain abscesses which cause brain swelling or cerebral edema. The CSF is also largely defenceless to internal insurgents, fifth columnists, such as a brain tumours, haematomas (bleeds), and cerebral vein thrombosis (venous clots). The smooth flow of the CSF may also be obstructed, resulting in hydrocephalus or enlargement of the brain’s ventricular system. In all these circumstances, the intracranial pressure is often elevated, a situation aptly dubbed intracranial hypertension. Very often, intracranial hypertension may occur without any obvious cause, and this condition is referred to as idiopathic intracranial hypertension (IIH). Because IIH threatens vision, neurologists have abandoned its old and misleading name, benign intracranial hypertension (BIH).

By BruceBlaus. When using this image in external sources it can be cited as:Blausen.com staff (2014). “Medical gallery of Blausen Medical 2014“. WikiJournal of Medicine 1 (2). DOI:10.15347/wjm/2014.010. ISSN 2002-4436. – Own work, CC BY 3.0, Link

Intracranial hypertension is no walk in the park as it portends disaster, whatever its cause. As it is a  potentially fatal state, the early warning signs are drilled into all doctors in medical school…when their brains are still malleable. These red flag features are severe headache, impaired consciousness, progressive visual loss, dilated or blown pupils, papilledema (swelling of the optic nerve head), and neck stiffness. The standard operating procedure for intracranial hypertension is to deflate the pressure as quickly as possible, by hook or by crook. This may be medical, with infusions such as mannitol, or surgical, with procedures such as decompressive craniectomy (removal of part of the skull). The terminal stage of intracranial hypertension, the most ominous neurological emergency, is cerebral herniation: this is the catastrophic compression of the brainstem into the narrow and tight spinal canal: a physical state that is incompatible with life.

By Ambika S., Arjundas D., Noronha V. – https://openi.nlm.nih.gov/detailedresult.php?img=2859586_AIAN-13-37-g001&query=papilledema&it=xg&req=4&npos=2, CC BY-SA 2.0, https://commons.wikimedia.org/w/index.php?curid=47658492

As with all waves, intracranial pressure also has its lows, and it is a no-brainer that neurologists call this intracranial hypotension. This is not as hazardous as intracranial hypertension, but it is worthy of respect in view of its devastating morbidity. The usual cause, and again no prizes for guessing this, is a leak. The puncture in this case is often iatrogenic, in other words, the whodunnit is the doctor. This may be deliberate, such as when the doctor attempts to remove some CSF to test, via a procedure called a  lumbar puncture (LP). It may also be accidental, such as when your friendly anaesthetist performs an epidural to relieve pain. In both situations, the dura protecting the CSF is perforated, causing spinal fluid leakage. This manifests as postural or orthostatic headache; by definition, this is a headache that sets in within 15 minutes of standing up, and resolves within 15 minutes of lying down flat. The treatment in such cases is strict bed rest, drinking loads of fluids, including caffeinated drinks, and waiting for the dura to heal itself…usually within one week. If this does not happen, then an intravenous caffeine infusion may be required. An epidural blood patch may also be carried out, again by your friendly anaesthetist, who squirts a little of the victims blood around the site of the leak, to, well, ‘patch it up’. In extremis, surgery may be needed to seal the leak, but this is way beyond my pay grade.

By Paul Anthony Stewart – Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=75808444

Intracranial hypotension may however develop without any apparent cause, and this is called spontaneous intracranial hypotension (SIH). The causes of SIH include unpredictable dural tears, ruptured meningeal diveticuli (outpouchings of the dura), and direct CSF-venous fistulae (don’t ask!) There are a variety of risk factors for SIH such as connective tissue diseases and bariatric surgery. It is very helpful that SIH leaves characteristic tell-tale clues on brain MRI scans, and these include subdural hygroma (plain fluid collections under the dura); subdural haematoma (blood under the dura); meningeal enhancement with contrast dye; engorgement of the pons and pituitary; and the interesting dinosaur tail sign on fat suppression T2 MRI (FST2WI). The gold standard test to localise the site of leakage in SIH is radionuclide cisternography. In the absence of this rather sophisticated test, a CT myelogram may be considered. Treatment is similar to that of other forms of intracranial hypotension, but other measures that may be required to seal the leak, including the use of fibrin sealeant.

By Hellerhoff – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=18946727

If you have reached the end of this blog post, then you deserve a prize. Four prizes actually: recent interesting reports in the field of SIH to explore:

  1. The use of transorbital ultrasound in making a diagnosis.
  2. Treatment of complicated SIH with intrathecal saline infusion.
  3. SIH complicated by superficial siderosis.
  4. Severe SIH complicated by sagging brain causing causing postural loss of consciousness.
By © Nevit Dilmen, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=45660723

 

 

7 ominous signs that suggest you need to see a neurologist

Neurologists spend most of their time diagnosing benign conditions which are curable or treatable, or at least people learn to live with. Every now and then we see people with startling symptoms such as coma, convulsions, neck stiffness, or paralysis. These are obviously concerning to patients and their families who have a foreboding of diseases such as meningitis, epilepsy, and stroke. Serious as these disorders are, they at least announce themselves and show their hands. Many other neurological symptoms unfortunately give no hint of the serious diseases that follow in their trail. That is when things get a bit tricky.

Ominous. Ankakay on Flikr. https://www.flickr.com/photos/ankakay/4101391453
Ominous. Ankakay on Flikr. https://www.flickr.com/photos/ankakay/4101391453

What are these seemingly benign symptoms which jolt neurologists out of their blissful complacency? What are these red flag symptoms that pretend they are grey? Here are my 7 deceptively ominous neurological signs everyone should know about.

7. A numb chin

By Henry Vandyke Carter - Henry Gray (1918) Anatomy of the Human Body (See "Book" section below)Bartleby.com: Gray's Anatomy, Plate 784, Public Domain, https://commons.wikimedia.org/w/index.php?curid=531758
By Henry Vandyke CarterHenry Gray (1918) Anatomy of the Human Body (See “Book” section below)Bartleby.com: Gray’s Anatomy, Plate 784, Public Domain, https://commons.wikimedia.org/w/index.php?curid=531758

This must be the most deceptive sinister symptom in neurology. Not many people will rush to their doctors to complain about a numb chin, but it is a symptom that makes neurologists very nervous. This is because the chin gets its sensory supply from the mandibular branch of the fifth cranial nerve, also called the trigeminal nerve because it has three branches. And neurologists know that, for some bizarre reason, cancers from other parts of the body occasionally send deposits to this nerve. The numb chin syndrome is therefore not to be treated lightly.

6. Muscle twitching

OK, don’t panic yet. We have all experienced this; a flickering of an overused and tired muscle; a twitching of the odd finger; the quivering of the calf muscles in older people. Neurologists call these fasciculations, and they are only a concern if they are persistent, progressive, and widespread. And also usually only if the affected muscles are weak. In such cases neurologists worry that fasciculations are the harbingers of sinister diseases, particularly motor neurone disease (MND), better known in America as amyotrophic lateral sclerosis (ALS) or Lou Gehrig disease. Many people with muscle twitching will however have nothing seriously wrong with them, and many will be shooed out of the consulting room with the label of benign fasciculations syndrome (we love our syndromes, especially when they are benign). There are many other causes of fasciculations, but MND is clearly the most sinister of them all.

5. Transient visual loss

Scott Maxwell on freestockphotos. http://www.freestockphotos.biz/stockphoto/9747
Scott Maxwell on freestockphotos. http://www.freestockphotos.biz/stockphoto/9747

Neurologists often ask people with headache if their vision blurs or disappears for brief periods of time. These visual obscurations are not as dramatic as the visual loss that accompanies minor strokes or transient ischaemic attacks (TIAs). Visual obscurations affect both eyes and last only a few seconds. They are the result of sudden but brief increases in an already elevated pressure in the head. This may occur with relatively benign conditions such as idiopathic intracranial hypertension (IIH), but it may also portend a serious disorder such as a brain tumour.

4. Sudden loss of bowel or bladder control

bubble-1013915_1920

Loss of control down there would surely concern many people, but often not with the urgency it deserves. There are many non-neurological causes of bowel or bladder incontinence, but a sudden onset suggests that it is arising from the nervous system. The worrying diagnoses here are spinal cord compression and spinal cord inflammation (transverse myelitis). These disorders are often associated with other symptoms such as leg stiffness and weakness, but I really wouldn’t wait until these set in before I ask to see a neurologist.

3. Saddle anaesthesia

bicycle-saddle-791704_1920

Whilst we are on the topic of things down there, a related sinister symptom is loss of sensation around the genitals and buttocks, something your doctor will prudently call saddle anaesthesia. This arises when the nerves coming off the lower end of the spinal cord, collectively called the cauda equina, are compressed. The unpalatable condition, cauda equina syndrome (CES), worries neurologists because the compression may be due to a tumour in the spinal canal.

PS: The bicycle saddle is an apt analogy, but if you prefer horse riding, below is an alternative image to soothe your hurt feelings.

 

By BLW - Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=1956552
By BLW – Own work, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=1956552

2. A painful droopy eyelid

A droopy eyelid is a deceptively benign symptom which worries neurologists. This symptom, which neurologist prefer to call ptosis, is particularly concerning if it is accompanied by double vision. One worrying disorder which causes ptosis is myasthenia gravis (MG), and this presents with ptosis on both sides. More sinister is ptosis which is present only on one side, particularly if it is painful. This may be caused by brain aneurysms, especially those arising from a weakness of the posterior communicating artery (PCOM) artery. As the aneurysm grows, it presses on the third cranial or oculomotor nerve, one of three nerves that controls the eyeballs and keeps the eyelids open. An aneurysm is literally a time-bomb in the brain as they wield the threat of bursting and causing a catastrophic bleeding around the brain. This makes ptosis an ominous, but also a helpful, neurological symptom.

By Cumulus z niderlandzkiej Wikipedii, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=3167579
By Cumulus z niderlandzkiej Wikipedii, CC BY-SA 3.0, https://commons.wikimedia.org/w/index.php?curid=3167579

There are many other causes of ptosis including Horner’s syndrome, so don’t panic yet but get that eyelid checked out if it refuses to straighten out.

 

1. Thunderclap headache

By © Marie-Lan Nguyen / Wikimedia Commons, CC BY 2.5, https://commons.wikimedia.org/w/index.php?curid=24189896
By © Marie-Lan Nguyen / Wikimedia Commons, CC BY 2.5, https://commons.wikimedia.org/w/index.php?curid=24189896

thunderclap headache is a symptom that means exactly what it says on the label! Neurologists will ask if the onset felt as if one was hit by a cricket bat. Even though most people have never been so assaulted, almost everyone with thunderclap headache readily agree this is what it feels like. It is such a distressing symptom that it doesn’t strike the afflicted person (pun intended) that their doctors are more concerned about investigating them, then they are in curing their headache. They patient is rushed to the CT scanner, and then subjected to a lumbar puncture. The doctors then heave a huge sigh of relief when the spinal fluid shows no blood or blood products, reassured that the patient has not suffered a subarachnoid haemorrhage (SAH) from a ruptured a brain aneurysm. The patient, who now has just another headache, is left to get to grips with their now, suddenly, very uninteresting symptom. There are many other causes of a thunderclap headache, but a ruptured aneurysm is the most sinister. If you develop a thunderclap headache, don’t wait to see a neurologist…just get to the nearest hospital!

PS: Don’t feel aggrieved if you are across the Pacific; it is also a thunderclap headache if it felt like being hit by a baseball bat!

Baseball bat in sun. Peter Chen on Flikr https://www.flickr.com/photos/34858596@N02/3239696542
Baseball bat in sun. Peter Chen on Flikr https://www.flickr.com/photos/34858596@N02/3239696542

 

Want to check out more ominous signs? Check out Smart handles and red flags in neurological diagnosis by the neurologist Chris Hawkes in Hospital Medicine.

 

What are the most iconic neurological disorders?

Neurology is a broad specialty covering a staggering variety of diseases. Some neurological disorders are vanishingly rare, but many are household names, or at least vaguely familiar to most people. These are the diseases which define neurology. Here, in alphabetical order, is my list of the top 60 iconic neurological diseases, with links to previous blog posts where available.

 

1. Alzheimer’s disease

By uncredited - Images from the History of Medicine (NLM) [1], Public Domain, https://commons.wikimedia.org/w/index.php?curid=11648572
By uncredited – Images from the History of Medicine (NLM) [1], Public Domain, https://commons.wikimedia.org/w/index.php?curid=11648572

2. Behcet’s disease

By Republic2011 - Own work, CC BY 3.0, https://commons.wikimedia.org/w/index.php?curid=17715921
By Republic2011Own work, CC BY 3.0, https://commons.wikimedia.org/w/index.php?curid=17715921

3. Bell’s palsy

By http://wellcomeimages.org/indexplus/obf_images/69/f2/8d6c4130f4264b4b906960cf1f7e.jpgGallery: http://wellcomeimages.org/indexplus/image/M0011440.html, CC BY 4.0, https://commons.wikimedia.org/w/index.php?curid=36350600
By http://wellcomeimages.org/indexplus/obf_images/69/f2/8d6c4130f4264b4b906960cf1f7e.jpgGallery: http://wellcomeimages.org/indexplus/image/M0011440.html, CC BY 4.0, https://commons.wikimedia.org/w/index.php?curid=36350600

4. Brachial neuritis

5. Brain tumours

6. Carpal tunnel syndrome

7. Cerebral palsy (CP)

8. Cervical dystonia

9. Charcot Marie Tooth disease (CMT)

By http://wellcomeimages.org/indexplus/obf_images/66/09/4dfa424fe11bb8dc56b2058f04ba.jpgGallery: http://wellcomeimages.org/indexplus/image/V0026141.html, CC BY 4.0, https://commons.wikimedia.org/w/index.php?curid=36578490
By http://wellcomeimages.org/indexplus/obf_images/66/09/4dfa424fe11bb8dc56b2058f04ba.jpgGallery: http://wellcomeimages.org/indexplus/image/V0026141.html, CC BY 4.0, https://commons.wikimedia.org/w/index.php?curid=36578490

10. Chronic inflammatory demyelinating polyneuropathy (CIDP)

11. Cluster headache

12. Creutzfeldt-Jakob disease (CJD)

By Unknown - http://www.sammlungen.hu-berlin.de/dokumente/11727/, Public Domain, https://commons.wikimedia.org/w/index.php?curid=4008658
By Unknownhttp://www.sammlungen.hu-berlin.de/dokumente/11727/, Public Domain, https://commons.wikimedia.org/w/index.php?curid=4008658

13. Duchenne muscular dystrophy (DMD)

By G._Duchenne.jpg: unknown/anonymousderivative work: PawełMM (talk) - G._Duchenne.jpg, Public Domain, https://commons.wikimedia.org/w/index.php?curid=9701531
By G._Duchenne.jpg: unknown/anonymousderivative work: PawełMM (talk) – G._Duchenne.jpg, Public Domain, https://commons.wikimedia.org/w/index.php?curid=9701531

14. Encephalitis

15. Epilepsy

16. Essential tremor

17. Friedreich’s ataxia

By Unknown - http://www.uic.edu/depts/mcne/founders/page0035.html, Public Domain, https://commons.wikimedia.org/w/index.php?curid=3960759
By Unknownhttp://www.uic.edu/depts/mcne/founders/page0035.html, Public Domain, https://commons.wikimedia.org/w/index.php?curid=3960759

18. Frontotemporal dementia (FTD)

19. Guillain-Barre syndrome (GBS)

By Anonymous - Ouvrage : L'informateur des aliénistes et des neurologistes, Paris : Delarue, 1923, Public Domain, https://commons.wikimedia.org/w/index.php?curid=28242077
By Anonymous – Ouvrage : L’informateur des aliénistes et des neurologistes, Paris : Delarue, 1923, Public Domain, https://commons.wikimedia.org/w/index.php?curid=28242077

20. Hashimoto encephalopathy

21. Hemifacial spasm

22. Horner’s syndrome

By Unknown - http://ihm.nlm.nih.gov/images/B15207, Public Domain, https://commons.wikimedia.org/w/index.php?curid=19265414
By Unknownhttp://ihm.nlm.nih.gov/images/B15207, Public Domain, https://commons.wikimedia.org/w/index.php?curid=19265414

23. Huntington’s disease (HD)

https://en.wikipedia.org/wiki/George_Huntington#/media/File:George_Huntington.jpg
https://en.wikipedia.org/wiki/George_Huntington#/media/File:George_Huntington.jpg

24. Idiopathic intracranial hypertension (IIH)

25. Inclusion body myositis (IBM)

26. Kennedy disease

27. Korsakoff’s psychosis

28. Lambert-Eaton myasthenic syndrome (LEMS)

29. Leber’s optic neuropathy (LHON)

30. McArdles disease

31. Meningitis

32. Migraine

33. Miller-Fisher syndrome (MFS)

By J3D3 - Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=34315507
By J3D3Own work, CC BY-SA 4.0, https://commons.wikimedia.org/w/index.php?curid=34315507

34. Motor neurone disease (MND)

35. Multiple sclerosis (MS)

36. Multiple system atrophy (MSA)

37. Myasthenia gravis (MG)

38. Myotonic dystrophy

39. Narcolepsy

40. Neurofibromatosis (NF)

41. Neuromyelitis optica (NMO)

42. Neurosarcoidosis

43. Neurosyphilis

44. Parkinson’s disease (PD)

45. Peripheral neuropathy (PN)

46. Peroneal neuropathy

47. Progressive supranuclear palsy (PSP)

48. Rabies

49. Restless legs syndrome (RLS)

50. Spinal muscular atrophy (SMA)

51. Stiff person syndrome (SPS)

52. Stroke

53. Subarachnoid haemorrhage (SAH)

54. Tension-type headache (TTH)

55. Tetanus

56. Transient global amnesia (TGA)

57. Trigeminal neuralgia

58. Tuberous sclerosis

59. Wernicke’s encephalopathy

By J.F. Lehmann, Muenchen - IHM, Public Domain, https://commons.wikimedia.org/w/index.php?curid=9679254
By J.F. Lehmann, Muenchen – IHM, Public Domain, https://commons.wikimedia.org/w/index.php?curid=9679254

60. Wilson’s disease

By Carl Vandyk (1851–1931) - [No authors listed] (July 1937). "S. A. Kinnier Wilson". Br J Ophthalmol 21 (7): 396–97. PMC: 1142821., Public Domain, https://commons.wikimedia.org/w/index.php?curid=11384670
By Carl Vandyk (1851–1931) – [No authors listed] (July 1937). “S. A. Kinnier Wilson“. Br J Ophthalmol 21 (7): 396–97. PMC: 1142821., Public Domain, https://commons.wikimedia.org/w/index.php?curid=11384670

===============================

The Neurology Lounge has a way to go to address all these diseases, but they are all fully covered in neurochecklists. In a future post, I will look at the rare end of the neurological spectrum and list the 75 strangest and most exotic neurological disorders.

What are the most controversial questions in neurology?

Uncertainty and doubt abound in Neurology. There are many evidence-free areas where experts rub each other the wrong way. These controversies are big and occur in all neurology subspecialties. Controversy-busters have tried for about a decade to iron out these wrinkles on neurology’s face, but the unanswered questions remain. This is why there is a 10th World Congress of Controversies in Neurology (CONy) holding in Lisbon this year.

I want to assure you I have no conflict of interest to declare in this blog. My interest is to explore  which questions have plagued this conference over the last 10 years to pick out the most controversial topics in neurology. To do this I reviewed all previous conference programs and focused on the items that were slated for debate. I looked for practical topics that have remained unresolved, or are just emerging. Here are my top controversial neurological questions:

Raccoon argument II. Tambako The Jaguar on Flikr. https://www.flickr.com/photos/tambako/7460999402
Raccoon argument II. Tambako The Jaguar on Flikr. https://www.flickr.com/photos/tambako/7460999402

 

1st CONy 2007 (Berlin, Germany)

  • Clinically isolated syndromes (CIS): To treat or not to treat
  • Is stem cell therapy an imminent treatment in advanced multiple sclerosis (MS)?
  • Vascular cognitive impairment is a misleading concept?
  • Is mild cognitive impairment a misleading concept?

 

2nd CONy 2008 (Athens, Greece)

  • Can physical trauma precipitate multiple sclerosis?
  • Should patients with Parkinson’s disease (PD) be treated in the pre-motor phase?
  • What is the first line therapy for chronic inflammatory demyelinating polyneuropathy (CIDP)?
  • Is intravenous immunoglobulin (IVIg) effective in chronic myasthenia gravis (MG)?
  • Tau or ß-amyloid immunotherapy in Alzheimer’s disease (AD)?
  • Chronic fatigue syndrome is an organic disease and should be treated by neurologists?

 

3rd CONy 2009 (Prague, Czech Republic)

  • Should cerebrospinal fluid (CSF) be tested in every clinically isolated syndrome?
  • Can we prevent multiple sclerosis (MS) by early vitamin D supplementation and EBV vaccination?
  • Does Parkinson’s disease (PD) have a prion-like pathogenesis?
  • Patients with medication overuse headache should be treated only after analgesic withdrawal?

 

 

4th CONy 2010 (Barcelona, Spain)

  • Camptocormia in parkinson’s disease (PD): Is this dystonia or myopathy?
  • Does chronic venous insufficiency play a role in the pathogenesis of multiple sclerosis (MS)?
  • IVIg or immunosuppression for long-term treatment of CIDP?

 

5th CONy 2011 (Beijing, China)

  • Is sporadic Parkinson’s disease etiology predominantly environmental or genetic?
  • Is multiple sclerosis (MS) an inflammatory or a primarily neurodegenerative disease?
  • Are the new multiple sclerosis oral medications superior to conventional therapies?
  • Is bilateral transverse venous sinus stenosis a critical finding in idiopathic intracranial hypertension (IIH)?

 

6th CONy 2012 (Vienna, Austria)

  • Will there ever be a valid biomarker for Alzheimer’s disease (AD)?
  • Is amyloid imaging clinically useful in Alzheimer’s disease (AD)?
  • Do functional syndromes have a neurological substrate?
  • Should blood pressure be lowered immediately after stroke?
  • Migraine is primarily a vascular disorder?

 

 

7th CONy 2013 (Istanbul, Turkey)

  • Is intravenous thrombolysis the definitive treatment for acute large artery stroke?
  • Atrial fibrillation related stroke should be treated only with the new anticoagulants?
  • Is the best treatment for chronic migraine botulinum toxin?
  • IS CGRP the key molecule in migraine?
  • Is chronic cluster headache best treated with sphenopalatine ganglion (SPG) stimulation?
  • When should deep brain stimulation (DBS) be initiated for Parkinson’s disease?
  • Do interferons prevent secondary progressive multiple sclerosis (SPMS)?
  • Is deep brain stimulation (DBS) better than botulinum toxin in primary dystonia?
  • Are present outcome measures relevant for assessing efficacy of disease modifying therapies in multiple sclerosis (MS)?
  • Should radiologically isolated syndromes (RIS) be treated?
  • Does genetic testing have a role in epilepsy management?
  • Should cortical strokes be treated prophylactically against seizures?
  • Should enzyme-inducing antiepileptic drugs (AEDs) be avoided?
  • EEG is usually necessary when diagnosing epilepsy

 

8th CONy 2014 (Berlin, Germany)

  • Is late-onset depression prodromal neurodegeneration?
  • Does Parkinson’s disease begin in the peripheral nervous system?
  • What is the best treatment in advanced Parkinson’s disease?
  • Are most cryptogenic epilepsies immune mediated?
  • Should epilepsy be diagnosed after the first unprovoked seizure?
  • Do anti-epileptic drugs (AEDs) contribute to suicide risk?
  • Should the ketogenic diet be prescribed in adults with epilepsy?
  • Do patients with idiopathic generalized epilepsies require lifelong treatment?
  • Cryptogenic stroke: Immediate anticoagulation or long-term ECG recording?
Southern Chivalry: Argument Vs Clubs. elycefeliz on Flikr. https://www.flickr.com/photos/elycefeliz/6271932825
Southern Chivalry: Argument Vs Clubs. elycefeliz on Flikr. https://www.flickr.com/photos/elycefeliz/6271932825

 

9th CONy 2015 (Budapest, Hungary)

  • Is discontinuation of disease-modifying therapies safe in  long-term stable multiple sclerosis?
  • Is behavioral therapy necessary for the treatment of migraine?
  • Which is the first-line therapy in cases of IIH with bilateral papilledema?
  • Should patients with unruptured arterio-venous malformations (AVM) be referred for intervention?
  • Should survivors of hemorrhagic strokes be restarted on oral anticoagulants?
  • Will stem cell therapy become important in stroke rehabilitation?
  • Do statins cause cognitive impairment?

 

10th CONy 2016 (Lisbon, Portugal)

  • Which should be the first-line therapy for CIDP? Steroids vs. IVIg
  • Should disease-modifying treatment be changed if only imaging findings worsen in multiple sclerosis?
  • Should disease-modifying therapies be stopped when secondary progressive MS develops?
  • Should non-convulsive status epilepsy be treated aggressively?
  • Does traumatic chronic encephalopathy (CTE) exist?
  • Does corticobasal degeneration (CBD) exist as a clinico-pathological entity?
  • Is ß-amyloid still a relevant target in AD therapy?
  • Will electrical stimulation replace medications for the treatment of cluster headache?
  • Carotid dissection: Should anticoagulants be used?
  • Is the ABCD2 grading useful for clinical management of TIA patients?
  • Do COMT inhibitors have a future in treatment of Parkinson’s disease?

 

Debate Energetico. Jumanji Solar on Flikr. https://www.flickr.com/photos/jumanjisolar/5371921203
Debate Energetico. Jumanji Solar on Flikr. https://www.flickr.com/photos/jumanjisolar/5371921203

 

Going through this list, I feel reassured that the experts differ in their answers to these questions? The acknowledgement of uncertainty allows us novices to avoid searching for non-existent black and white answers. It is however also unsettling that I thought some of these questions had been settled long ago. It goes to show that apparently established assumptions are not unshakable?

Do you have the definitive answers to resolve these controversies? Are there important controversies that are missing here? Please leave a comment

 

10 bizarre things neurologists do to their patients

This is a follow-up to my previous blog post, So what is remarkable about neurology anyway? That post reviewed the challenging tasks neurologists face everyday. How do they go about it? How do they evaluate their patients with suspected neurological disorders?

Neurology by MV Maverick on Flikr. https://www.flickr.com/photos/themvmaverick/11396461045
Neurology by MV Maverick on Flikr. https://www.flickr.com/photos/themvmaverick/11396461045

 

For the uninitiated, the process of the neurological assessment must seem like an outlandish ritual. Unlike cardiologists who approach patients with the familiar stethoscope, neurologists come armed to the hilt with an arsenal of threatening equipment. Patients are often bewildered, and occasionally irritated, with the neurological exam. Admitted, they sometimes, they sometimes emerge from the assessment feeling battered and bruised-all for a good cause of course!

So what are these bizarre deeds that marks the neurological consultation?

1. Neurologists welcome you with an overly firm handshake 

By liftarn (http://openclipart.org/media/files/liftarn/2604) [Public domain], via Wikimedia Commons
By liftarn (http://openclipart.org/media/files/liftarn/2604) [Public domain], via Wikimedia Commons

The handshake is a valuable neurological tool. It tells the neurologist right from the beginning if there is any weakness or if there is a form of muscle stiffness called myotonia. Therefore avoid the neurologist’s handshake if you suffer with arthritis or other painful hand conditions.

2. Neurologists make you do the catwalk 

The way you walk, the gait, may show the neurologist a variety of clues or signs. There are a variety of abnormal gaits that often point to a diagnosis even before the consultation actually begins. Examples include the shuffling gait in Parkinson’s disease, the hemiparetic gait in Stroke, and the waddling gait in diseases that give rise to hip girdle weakness. More embarrassing for some patients is that the neurologist may actually ask them to do a catwalk, all for the sake of making a diagnosis you must understand!

Other bizarre associated tests are walking an imaginary tightrope, standing on one leg, standing on tip toes and then on the heels, and marching in one spot with eyes shut

3. Neurologists stare intently at you 

"Thisisbossi Symmetry" by Andrew Bossi - Own work. Licensed under CC BY-SA 3.0 via Wikimedia Commons - https://commons.wikimedia.org/wiki/File:Thisisbossi_Symmetry.JPG#/media/File:Thisisbossi_Symmetry.JPG
“Thisisbossi Symmetry” by Andrew Bossi – Own work. Licensed under CC BY-SA 3.0 via Wikimedia Commons – https://commons.wikimedia.org/wiki/File:Thisisbossi_Symmetry.JPG#/media/File:Thisisbossi_Symmetry.JPG

 

The face often give the neurologist the clue to many diagnoses. Conditions such as Bell’s palsy and Stroke are evident from the face as are Parkinson’s disease, myotonic dystrophy and facio-scapulo-humeral muscular dystrophy (FSHD). There’s no need to blush therefore when the intent gaze seems to go on endlessly.

4. Neurologists come up very close- to peer into your soul

If the eyes are the windows to the soul, then neurologists are second only to ophthalmologists in recognising this nebulous entity. The back of the eye, or retina, holds a variety of valuable clues for many neurological diseases. The neurologist typically looks for signs of increased pressure in the head and this may occur with brain tumours, meningitis, encephalitis, This may also occur without any obvious cause in a condition called idiopathic intracranial hypertension (IIH). Other eye signs such as cataracts and pigmented retina seen with disorders for example mitochondrial diseases. 

To peer into the soul, the neurologist may come very uncomfortably close, (hoping the aftershave isn’t too strong and that the morning deodorant has lasted till then). Don’t hold your breath however, as this gazing into the soul may take longer than you anticipate.

5. Neurologists ask you to roll your eyes-in all sorts of directions

Muscles of the eye, circa 1900 by Double-M on Flikr. https://www.flickr.com/photos/double-m2/5551619158
Muscles of the eye, circa 1900 by Double-M on Flikr. https://www.flickr.com/photos/double-m2/5551619158

 

Abnormal eye movements are key pointers to many neurological disorders. There are six muscles that move each eyeball, and these are under the control of three pairs of cranial nerves-the oculomotor, the trochlear, and the abducens nerves. These nerves in turn are coordinated by complex nerve cell bodies or nuclei in the brain stem.The eyelids and pupils are also muscles under control of nerves.

These cranial nuclei coordinate a symphony of unparalleled and unimaginable complexity. This allows us to focus on moving objects without any hinderance. Things may go wrong with this symphony, and this typically results in double vision (diplopia) and droopy eyelids (ptosis). Diseases that cause these symptoms include brain aneurysms, myasthenia gravis (MG), and brainstem stroke. Some diseases may cause the eyeballs to move in uncontrollable and chaotic ways called nystagmus, oscillopsia, and opsoclonus (neurologists love these names!) 

Don’t be shocked therefore when your neurologist asks you to look up, look down, look to the right and left; to follow this or the other hand; to look at this fist then at these fingers…. It’s all a helpful game-honest!

6. Neurologists ask you to pretend to brush your teeth 

 

"Marcel Marceau - 1974" by press photo - ebay. Licensed under Public Domain via Commons.
Marcel Marceau – 1974” by press photo – ebay. Licensed under Public Domain via Commons.

 

Your neurologist may request you to brush your teeth or hair with an imaginary brush, or ask you to do victory sign or the thumbs-up sign (never thumbs-down mind you). Almost verging on the comedic, this is a serious test because these simple tasks are impaired in many diseases. The difficulty in performing tasks one has previously been proficient at is called dyspraxia, or apraxia if the ability is completely lost. Without any weakness or numbness, people with dyspraxia are unable to use common tools and equipment, reporting that they have no idea how to manipulate them. This could be seen in some forms of stroke and some dementias. Do decline however if she asks you to mimic the great mime Marcel Marceau.

7. Neurologists ask you to wiggle your tongue and poke it out 

New Zealand Maori culture 009 by Steve Evans on Flikr. https://www.flickr.com/photos/babasteve/5418324230
New Zealand Maori culture 009 by Steve Evans on Flikr. https://www.flickr.com/photos/babasteve/5418324230

 

The tongue is a very important muscle and holds countless clues for the neurologist. It is innervated by the last of the 12 cranial nerves, the hypoglossal nerve. which may be paralysed by a very localised stroke and this is often in the context of a condition called cervical artery dissection. This is a tear in one of the big arteries in the neck which take blood to the brain. The tear may arise from trivial neck movements and manipulations such as look up for a long time or staying too long on the hairdressers couch. A clot then forms at the site of the tear, and this then migrates to block a smaller blood vessel supplying the brainstem where the hypoglossal nerve sets off from…phew! Anyway, when this kind of stroke occurs, the tongue deviates to the the weaker side when it is poked out.

The more general weakness of the tongue is seen in conditions such as motor neurone disease (MND), in which the tongue also quivers at rest-something neurologists call fasciculations. The cheeky neurologist (pun intended) will ask you to push against her finger through your cheek to test its full strength.

Another problem that may affect the tongue is myotonia, a condition in which he tongue and other muscles are stiff and relax very slowly after they are activated. To test this, your neurologist may actually tap on your tongue, and then watches in fascination as it stiffens and then relaxes very slowly. Strong but slow moving tongues may be seen in Parkinson’s disease (PD). So, when next your neurologist says ‘open up’, he really means business.

8. Neurologists flex their muscles against yours

 

"FreestyleWrestling2" by Staff Sergeant Jason M. Carter, USMC - Defenseimagery.mil, VIRIN 040307-M-RS496-226. Licensed under Public Domain via Commons - https://commons.wikimedia.org/wiki/File:FreestyleWrestling2.jpg#/media/File:FreestyleWrestling2.jpg
“FreestyleWrestling2” by Staff Sergeant Jason M. Carter, USMC – Defenseimagery.mil, VIRIN 040307-M-RS496-226. Licensed under Public Domain via Commons – https://commons.wikimedia.org/wiki/File:FreestyleWrestling2.jpg#/media/File:FreestyleWrestling2.jpg

 

OK, she will not literally wrestle you to the ground but it may appear so at times. Pushing against your head, pressing down against your elbows, leaning hard against your leg-she will do everything to show she is stronger than you. Only if she fails will she score your power as grade 5/5-the best you can get. If you do not score full marks however you place the neurologist in a bit of a quagmire; a score between 0-5 is not always easy to allocate, and the obsessive neurologist may get in a bind and may give you marks such as 3+ or 4-. Just for fun let her win, and see her consternation!

9. Neurologists hit you with a hammer-in all sorts of places

Lego man and reflex hammer by Dr. Mark Kubert on Flikr. https://www.flickr.com/photos/clearpathchiropractic/7590265518
Lego man and reflex hammer by Dr. Mark Kubert on Flikr. https://www.flickr.com/photos/clearpathchiropractic/7590265518

 

The reflex hammer is perhaps the most well-recognised tool of the neurologist. These hammers come in all shapes and sizes, and some are really quite scary. People expect to have their knees tapped and look forward to what they have seen many times on TV-the leg kicking out. Most patients find this amusing. They are however often surprised  when the neurologist proceeds to use the hammer on their jaw, elbow, wrist and ankles. The then often bristle at having the soles of their feet stroked by the end of the hammer’s handle, a sharp uncomfortable end it is. All the hammer does is to stretch the tendons of muscles, and this elicits a reflex that causes the muscle to contract or tighten up. This response may be exaggerated (hypereflexia) if there is any problem in the central nervous system. Conversely the reflex response may be diminished (hyporeflexia) with problems of the peripheral nervous system.  Stroking the foot is called the Babinski response and gives a similar form of information to the neurologist. But beware the neurologist who then proceeds to stroke the side of your foot or squeeze your shins, all in an effort to get the same information-it is really an unnecessary and uncomfortable duplication of tests.

10. Neurologists prick and prod you with a sharp pin

Now this must take the cake, and quite rightly often comes at the end of the neurological examination. As threatening as this tests appears, this is probably the neurologist at his most acute. Using a sterile pin, the neurologist asks you to respond ‘yes’ if the sensation you perceive is sharp, and ‘no’ if it is dull. He then carefully proceeds to map out areas of reduced sensation or feeling, frowning as he struggles to keep track of your responses in his mind. He tries to establish if you have a glove and stocking pattern of sensory loss seen in peripheral neuropathy (nerve end damage). It may also be a dermatomal pattern seen with radiculopathy (trapped nerve in the spine). Unfortunately for the neurologist however many patients do not understand the rules of the game and give all sorts of unimaginable responses; not surprising when one is under the threat of a sharp pointy object!

"User-FastFission-brain". Licensed under CC BY-SA 3.0 via Wikimedia Commons - https://commons.wikimedia.org/wiki/File:User-FastFission-brain.gif#/media/File:User-FastFission-brain.gif
“User-FastFission-brain”. Licensed under CC BY-SA 3.0 via Wikimedia Commons – https://commons.wikimedia.org/wiki/File:User-FastFission-brain.gif#/media/File:User-FastFission-brain.gif

 

These are but a few of the bizarre doings of neurologists.  Seeing a neurologist soon? Be prepared-you have been warned!

PS. Images used in this blog post are for illustration purposes only and do not necessary depict the actual equipment used by neurologists. The examination steps described are however a good reflection of actual neurological practice.

10 remarkable breakthroughs that will change neurology

This is the age of rapidly advancing technology. Blink, and the scene changes unrecognisably. It would be unbelievable if we weren’t actually living it. What technological advances will impact Neurology in the near future? Here are my top 10 neurology-impacting technologies.

1. Nanotechnology to deliver clot-busting drugs

CSIRO [CC BY 3.0 (http://creativecommons.org/licenses/by/3.0)], via Wikimedia Commons
CSIRO [CC BY 3.0 (http://creativecommons.org/licenses/by/3.0)%5D, via Wikimedia Commons

Clot-busting or thrombolysis is life saving treatment following stroke. This however requires getting to hospital within 4.5 hours of the event, and is given by intravenous injections. How much better if it would be if thrombolysis could be delivered by mouth, and at the point of contact with paramedics. Indeed this is the idea behind clot-busting nanocapsules. Nanoparticles may also have future applications in prevention of MS relapses.

2. Disease-monitoring wearables

What if people with epilepsy could predict their next seizure? Or if someone with multiple sclerosis (MS) could predict an impending relapse? Well,  wearable technology promises to do just that. This goes beyond the fitbit which measures basic biological processes; these technologies will monitor realtime data such as a watch that measures skin moisture for seizure-prediction, or an iPad strapped to the back to monitor walking speed of patients with MS. I predict this technology will rapidly spread to many other chronic neurological diseases.

3. Nanoscale-resolution brain imaging

From the humble X-ray to the CT scan, brain imaging has progressed in leaps and bounds to a proliferation of MRI modalities with ever-increasing resolution or power. But nanoscale resolution imaging promises to make things more SciFi than healthcare. With the ability to look at ‘every nook and cranny‘ of the brain, this technology will visualise brain connections with incredible detail. Imagine how this will enhance diagnostic accuracy (and diagnostic conundrums in equal measure). This work is still in mice butI’m sure human application will follow shortly.

4. High-resolution eye selfies

Mobile phones are ubiquitous and the camera function seems to be more valuable than the talk mode. What with the number of selfies proliferating like a rah over social media. This may however be of advantage to healthcare. As the camera resolution increases exponentially, eye-selfies may come to the aid of neurologists and  ophthalmologists who treat patients with a condition called idiopathic intracranial hypertension (IIH). In this condition the pressure of the fluid around the brain is elevated. This shows as a blurring of the margins of an area called the disc and this is seen in the back of the eye using an ophthalmoscope. With advanced mobile phone cameras patients with IIH could make an eye-selfie diagnosis or assist in monitoring their eyes themselves.

5. Wireless brain EEG monitoring

Thinker Thing. https://www.flickr.com/photos/thinkerthing/8075309856
Attribution: Thinker Thing. https://www.flickr.com/photos/thinkerthing/8075309856

 

The electroencephalogram (EEG) is an invaluable tool for making the diagnosis of epilepsy. The process requires a time-consuming application of several electrodes to specific points on the scalp. The electrodes are then connected by wires or leads to a machine which records the brains electrical activity. This cumbersome process is time consuming especially for patients that need to keep the wires on for days. To the rescue is the wireless brain helmetThis will not only make the recording easier, it will send the recording wirelessly to the physiologist who will interpret the test. More interestingly, it will allow receive signals sent by the physiologist which will be targeted to treat epilepsy or other conditions like depression. The NeuroPace’s RNS system is one such device leading the way.

6.Wireless drug delivery

This is another wireless technology which facilitates the direct delivery of drugs into the brainThe device, not thicker than a human hair, is implanted into the brain and wirelessly controlled to deliver the required dose of drug, at specified times. The likely beneficiary diseases are epilepsy and depression (again). It is still in the stage of trials in mice but coming to your neighbourhood hospital very soon. If you want the complicated details then see the journal Cell for the research paper titled Wireless Optofluidic Systems for Programmable In-vivo Pharmacology and Optogenetics. What a mouthful!

7. Suicide-prediction technology

By RyanJWilmot (Own work) [CC BY-SA 4.0 (http://creativecommons.org/licenses/by-sa/4.0)], via Wikimedia Commons
By RyanJWilmot (Own work) [CC BY-SA 4.0 (http://creativecommons.org/licenses/by-sa/4.0)%5D, via Wikimedia Commons

A blood test to warn of the risk of impending suicide? Wouldn’t that be great? It is not a far-fetched dream if reports that a blood test for RNA biomarkers of suicidal thoughts fulfils its potential. This will have psychiatrists whooping for joy-or out of a job!

 8. Optogenetics

Optogenetics is the use of light to control cells. This has the potential to alter nervous system function with exciting prospects for disease treatment. Again epilepsy appears to be a prime beneficiary if this takes off. Imagine programming a brain cell or neurone to glow red when calcium flows into it. This glow then dampens the activity of neighbouring cells thereby inhibiting any rouge electrical impulse that may result in an epileptic seizure. The process requires the injection of a genetically engineered virus which infects the brain cells. This ability to modify brain cell behaviour also has implications for the treatment of Parkinson’s disease (PD) and depression among other things. More SciFi you say.

9. Gene therapy for muscular dystrophy

Genetic therapy is an old dog that is still barking. This is just as well because it remains the only hope for many genetic conditions.  Genetic therapy has had its ups and downs and a very recent high is the positive outcome in leukaemia. Neurology is however not too far behind if this report that muscular dystrophy gene therapy has been successful in dogs is translated to humans. The research is rather complex but the academically minded may be interested in details of the trial.

10. Molecular spies for early cancer detection

A molecular spy is an antibody probe that is directed at the brain to detect and destroy ‘rogue’ cells. The leading researcher for this is Sam Gembhir who is based at the Canary Center at Stanford for Cancer Early Detection. Best to hear it from the horse’s mouth- speaking here at a TED talk.

https://www.youtube.com/embed/yG1J5e6-uT4” target=”_blank”>

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