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.
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.
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.
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.
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 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.
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.
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.
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.
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 encephalomyelitis, lymphocytic 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.
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.
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.
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.
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.
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.
Transverse myelitis (TM)
Optic neuritis (ON)
Internuclear ophthalmoplegia (INO)
Excessive daytime sleepiness (EDS)
Paroxysmal kinesigenic dyskinesia (PKD)
Hand muscle atrophy
Balo’s concentric sclerosis
Why not explore the whole of MS with comprehensive checklists? Below are some MS related Neurochecklists to start you off:
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.
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.
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 causesof viral encephalitis and its management.
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 thefull neurological manifestations of HEV.
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 encephalopathy, Guillain Barre syndrome (GBS), acute demyelinating encephalomyelopathy (ADEM), and stroke. Not one to be treated lightly at all. Check out everythingabout Influenza H1N1 and the different ways influenzaaffects the nervous system.
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 20things we now know for certain about the Zika virusonour sister blog, The Neurology Lounge.
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.
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 thefull VZV on neurochecklists.
Check out the other deadly viral neurological infections on neurochecklists: