Categories
Neuroinflammation

What factors predict poor outcomes of anti MOG antibody disorders?

Myelin oligodendrocyte glycoprotein antibody-associated demyelination: comparison between onset phenotypes

Zhou Y, Jia X, Yang H, et al.

Eur J Neurol 2019; 26:175-183.

Abstract

BACKGROUND:

The aim of this study was to analyse the clinical and prognostic features of myelin oligodendrocyte glycoprotein (MOG) antibody-associated demyelination with different onset phenotypes.

METHODS:

A total of 52 MOG-IgG-seropositive patients were divided into four groups: (i) optic neuritis (ON) at onset (MOG-ON+ , n = 23), (ii) transverse myelitis (TM) at onset (MOG-TM+ , n = 12), (iii) pure brain symptoms at onset (MOG-ON -TM , n = 14) and (iv) both ON and TM at onset (n = 3). This final group was not included in further analyses. Data were collected through medical records and regular follow-up.

RESULTS:

Median age at presentation was 24 (range, 3-63) years in the whole cohort (50% female). MOG-ON -TM patients had the youngest age of onset across the three groups. Patients with MOG-TM+ tended to relapse more frequently and had a longer interval to first relapse than was observed in MOG-ON+ and MOG-ON -TM patients. High MOG-IgG titres were associated with increased cerebrospinal fluid leukocytes. The likelihood of harbouring transient, low MOG-IgG titres was higher in the MOG-TM+ group than in the other groups. After a median disease duration of 20 months, most but not all cases had a favourable outcome, with 8% developing severe visual deficit, 2% becoming wheelchair-dependent and 6% developing cognitive impairment. The onset phenotype appeared to be an important predictor of disability type. Having high MOG-IgG titres(odds ratio, 0.168, P = 0.027) or female gender (odds ratio, 0.270, P = 0.067) was associated with a lower likelihood of complete recovery.

CONCLUSIONS:

Onset phenotype may influence long-term presentation, MOG-IgG status as well as outcome. Further large and prospective studies are needed to better clarify the clinical implications of the first demyelinating event.

This article is cited in the neurochecklist:

Anti MOG antibody disorders: clinical features

Abstract link

Optic nerve side view. Francisco Bengoa on Flikr. https://www.flickr.com/photos/frecuenciamedicafb/7404373800

 

Categories
Neurological infections

The 7 most ruthless bacterial infections of the nervous system

This is a follow up to our previous post, the 7 most devastating viral neurological infections. The list of bacteria that invade the nervous system is endless, but some stand out because of the fear they evoke, and the peril they pose. Here then are the 7 most horrifying bacterial infections that threaten the nervous system.

1. Bacterial meningitis

Klebsiella pneumonia bacterium. NIAID on Flikr. https://www.flickr.com/photos/niaid/13383468143

Many bacteria invade the covering of the brain, the meninges, without invading the brain substance. The commonest are Neisseria meningitidis, causing meningococcal meningitis, and Streptococcus pneumoniae, causing pneumococcal meningitis. Other relatively frequent meningeal intruders include Listeria monocytogenes and Haemophilus influenzae. Bacteria may get into the brain following infections elsewhere, such as sinusitis or otitis media (inner ear infection). There are many complications of bacterial meningitis such as cerebral venous thrombosis (CVT) and brain abscess.

2. Tuberculosis

Mycobacterium tuberculosis bacteria, the cause of TB. NIAID on Flikr. https://www.flickr.com/photos/niaid/5149398656

Tuberculosis (TB) is probably as old as history. It is caused by mycobacterium tuberculosis, a slow groing but pernicious organism. TB spares no part of the nervous system, and manifests often as tuberculous meningitis (TBM) or Pott’s disease of the spine. Nervous system TB may also present as an encephalopathy, tuberculoma, brain abscess, vasculopathy, arachnoiditis, radiculomyelitis, and calvarial TB.

3. Neurosyphilis

ff treponema pallidum. isis335 on Flikr. https://www.flickr.com/photos/92708411@N07/8579266595

Treponema pallidum, the bacterium behind the dreaded syphilis, is another ancient bug. It has a variety of ways it terrorises the nervous system, and the longer it inhabits the neurones, the worse the outcome. Typical manifestations of neurosyphilis are tabes dorsalis, general paresis of the insane (GPI), taboparesis, stroke, meningovascular syphilis, optic neuritis (ON), and several movement disorders.

4. Lyme neuroborreliosis

Lyme Disease Bacteria, Borrelia Burgdoferri. NIAID on Flikr. https://www.flickr.com/photos/niaid/5661846104

Lyme disease has acquired an infamy which is probably beyond its real notoriety. It is best known for its tick-borne transmission, and for its classical dermatological feature, erythema chronicum migrans. It affects the nervous system in diverse ways such as encephalomyelitislymphocytic meningitis, cranial neuropathies, spinal radiculitis, stroke, diaphragmatic paralysis, and peripheral neuropathy. Post-Lyme syndrome is a very contentious topic; you may read more on this in a post from our sister blog, The Neurology Lounge, titled ‘Why is chronic Lyme disease so frustrating to neurology.

5. Neurobrucellosis

By This media comes from the Centers for Disease Control and Prevention‘s Public Health Image Library (PHIL), with identification number #1902.Note: Not all PHIL images are public domain; be sure to check copyright status and credit authors and content providers.English | Slovenščina | +/−, Public Domain, Link

Neurobrucellosis is a rarely discussed bacterial infection but it is a significant contributor to neurological morbidity and mortality around the world. It is caused by various brucella species usually grouped under the name Brucella militensis. It has a long reach in the nervous system, causing  a variety of insults such as encephalitis, meningoencephalitis, cranial neuropathies, intracerebral haemorrhage (ICH), subarachnoid haemorrhage (SAH), transverse myelitis, radiculitis, and peripheral neuropathy.

6. Leprosy

Public Domain, Link

This most distasteful of infectious diseases unfortunately has a strong affinity for the nervous system. Unlike its distant cousin, TB, leprosy favours the peripheral over the central nervous system. Its hallmark is thickening of the nerves or nerve hypertrophy. Caused by Mycobacterium leprae, leprosy has a legion of neurological manifestations such as mononeuritis, mononeuritis multiplex, cranial and peripheral neuropathy, myelitis, and leprous ganglionitis.

7. Botulism

Clostridium botulinum. Phil Moyer on Flikr. https://www.flickr.com/photos/hukuzatuna/2537594892

Botulism is the end result of damage by the toxin of Clostridium botulinum. This toxin produces a deadly paralysis by blocking neural transmission across the neuromuscular junction (NMJ). Botulinum toxin respects no borders, able to gain access to the nervous system through the gut, the skin, or the lungs. It paralyses everything, causing acute limb, ocular, and bulbar weakness. Left unchecked, botulism results in autonomic dysfunction and respiratory failure.

 

PS: For Tetanus, check out The Neurology Lounge blog post on The 13 most dreadful neurological disorders

_________________________________________________________________________

Explore more neurological infections in neurochecklists!

Bacteria. AJ Cann on Flikr. https://www.flickr.com/photos/ajc1/8975675759
Categories
Multiple sclerosis

The 50 faces of multiple sclerosis: the diverse presentations of a common disease

Multiple sclerosis (MS) dominates neurological practice in many parts of the world. This is no doubt because it is a common disorder which favours the young.The typical form, relapsing remitting MS (RRMS), is often easy to recognise and diagnose. The are however other types and variants such as primary progressive MS (PPMS), that often pose a challenge to neurologists to pin down.

MRI scan. NIH Image Gallery on Flikr. https://www.flickr.com/photos/nihgov/30805879596

There is no single clinical symptom or sign that is pathognomonic of MS. Some presentations are red flags for MS, such as optic neuritis (ON) and transverse myelitis (TM). Many other MS symptoms and syndromes are however non-specific. The ways MS presents are diverse, and below is a list of the 50 different faces of MS.

MRI scan. NIH Image Gallery on Flikr. https://www.flickr.com/photos/nihgov/30842540675

Ataxia

Tremor

Trigeminal neuralgia

Vertigo

Dizziness

Diplopia 

Incontinence

Constipation

Dysphagia

Dysarthria

*** 

Transverse myelitis (TM)

Optic neuritis (ON)

Memory impairment

Executive dysfunction

Depression

Psychosis

Spastic paraparesis

Lhermitte’s phenomenon 

Impaired libido

Erectile dysfunction

***

Uhthoff’s phenomenon

Pulfrich phenomenon

Nystagmus

Internuclear ophthalmoplegia (INO) 

Fatigue

Seizures

Pain

Sleep disorders

Heat sensitivity

Tonic spasms

***

Pathological laughter

Pathological crying

Sensory impairment

Paroxysmal attacks

Radicular symptoms

Restless legs

Excessive daytime sleepiness (EDS)

Olfactory dysfunction

Taste dysfunction

***

Hyperacusis

Horner’s syndrome

Respiratory impairment

Paroxysmal kinesigenic dyskinesia (PKD)

Takotsubo cardiomyopathy 

Encephalopathy

Hand muscle atrophy

Tumefactive MS

Balo’s concentric sclerosis

Movement disorders

MRI scan. NIH Image Gallery on Flikr. https://www.flickr.com/photos/nihgov/30805878966
Why not explore the whole of MS with comprehensive checklists? Below are some MS related Neurochecklists to start you off:

Multiple sclerosis (MS): risk factors

Multiple sclerosis (MS): typical clinical features

Multiple sclerosis (MS): unusual features

Multiple sclerosis (MS): differential diagnosis

Primary progressive multiple sclerosis (PPMS)

Clinically isolated syndromes (CIS)

Radiologically isolated syndrome (RIS)

Multiple sclerosis (MS): atypical variants

Tumefactive multiple sclerosis (TMS)

Progressive solitary sclerosis

By Monthly_multiple_sclerosis_anim.gif: Waglionederivative work: Garrondo – This file was derived from  Monthly multiple sclerosis anim.gif: , Public Domain, Link

Categories
Neurological infections

The 7 most devastating viral neurological infections

One may be forgiven for thinking that neurology is all about neuroinflammatory and neurodegenerative diseases. This is because these disorders seem to get a lot of attention. But nothing could be further from the truth-globally, infections impose a heavier burden on neurological practice than say Multiple Sclerosis (MS) or Parkinson’s disease (PD). And medical advances have done very little to deter all sorts of creatures from invading the nervous system.

Bacteria. Cesar Herada on Flikr. https://www.flickr.com/photos/worldworldworld/4095866396/

The major types of organisms that infect the nervous system are viruses and bacteria, but fungi and parasites also take their toll. In this blog we will focus on the 7 most devastating viral neurological infections.

CC BY-SA 3.0, Link

1.  Viral encephalitis

Encephalitis is infection of the brain substance, as opposed to meningitis which is infection of the covering of the brain. Viral encephalitis, for some reason, tends to favour the temporal lobes of the brain causing seizures and memory problems, amongst other symptoms. The main villain responsible for viral encephalitis is herpes simplex type 1 (HSV1), but almost every other virus can carry out the job with deadly precision. The list is long and includes geographically specific viruses as West Nile and Japanese B. Check out the full list of causes of viral encephalitis and its management.

Herpes Simplex Virus Type 1: Procapsid and Mature Capsid. NIH Image gallery on Flikr. https://www.flickr.com/photos/nihgov/28295539863

2. HIV associated neurological infections

No part of the nervous system is immune to the ravages of the dreaded HIV. The list includes HIV  associated neurocognitive disorders (HAND)myelopathiesneuropathies, drug-induced syndromes, and tumours. The worst aspect of HIV, of course, is that it opens the flood gates for opportunistic infections to invade the nervous system.

By BruceBlausOwn work, CC BY-SA 4.0, Link

3. Hepatitis E virus (HEV)

Hepatitis E virus is just emerging as a scourge of neurology. It is particularly villainous because of its protean manifestations, from Guillain Barre syndrome (GBS) to neuralgic amyotrophy (brachial neuritis), from transverse myelitis to idiopathic intracranial hypertension (IIH). Check out the full neurological manifestations of HEV.

By Transferred from en.wikipedia to Commons.This media comes from the Centers for Disease Control and Prevention‘s Public Health Image Library (PHIL), with identification number #5605.Note: Not all PHIL images are public domain; be sure to check copyright status and credit authors and content providers.English | Slovenščina | +/−, Public Domain, Link

4. Influenza H1N1

Influenza is bad, and H1N1 is a particularly nasty variant. This subtype of Influenza A is epidemic in pigs and birds, and unleashes havoc when it crosses over to humans. Its nervous system manifestations include encephalopathyGuillain Barre syndrome (GBS), acute demyelinating encephalomyelopathy (ADEM), and stroke. Not one to be treated lightly at all. Check out everything about Influenza H1N1 and the different ways influenza affects the nervous system.

H1N1 influenza viral particles. NIAID on Flikr. https://www.flickr.com/photos/niaid/8414750984

5. Zika virus infection (ZIKV)

This new kid on the infection block is fast establishing itself as a menace. Apart from causing myelitis, meningoencephalitis, encephalitis, encephalomyelitis, Guillain-Barre syndrome (GBS), and myasthenia gravis (MG), it is responsible for a variety of congenital defects, particularly microcephaly. Zika virus pathology and management are extensively covered in neurochecklists. Or check out 20 things we now know for certain about the Zika virus on our sister blog, The Neurology Lounge.

By Manuel Almagro RivasOwn work, CC BY-SA 4.0, Link

6. Ebola virus disease (EVD)

This ancient virus gained recent notoriety when it ravaged a large section of West Africa, sending chilling waves across the world. It is an RNA filovirus whose main reservoir is bats. It causes, among other things, an encephalitis and meningoencephalitis. It appears to be on vacation in the meantme, but it will surely rear its ugly head sometime soon. Check out the comprehensive clinical features and management of Ebola virus disease on neurochecklists.

By Scientific Animations – http://www.scientificanimations.com/wiki-images/, CC BY-SA 4.0, Link

7. Varicella zoster virus (VZV)

The varicella virus must take the prize for the most diverse ways a virus affects the nervous system. Neurochecklists has listed >20 neurological manifestations of VZV, ranging from herpes zoster to post herpetic neuralgia (PHN), from meningitis to encephalitis. VZV also causes all forms of cranial and peripheral neropathy, and may result in stroke, aneurysms, and giant cell arteritis (GCA). Not to mention the curiously named progressive outer retinal necrosis (just don’t mention its acronym!). Check out the full VZV on neurochecklists.

 

Check out the other deadly viral neurological infections on neurochecklists:

Dengue virus infection (DENV)

West Nile virus (WNV) infection

Japanese encephalitis virus (JEV)

Rabies encephalitis