Demylinating Disorders Of CNS

Question 1

How Acquired demyelinating disorders of the central nervous system will present …..?

Answer 1

They present with neurologic dysfunction caused by immune-mediated attacks on the white matter insulating the brain, optic nerves, and spinal cord

Question 2

The white matter insulation is formed which cells…?

Answer 2

By myelin contained within oligodendrocytes wrapping around nerve axons.

Question 3

What are the antibodies implicated in demyelination ..?

Answer 3

IgG antibodies recognized as playing an important role in demyelination,

aquaporin 4-antibody (AQP4-Ab) and

myelin oligodendrocyte glycoprotein antibody (MOG-Ab).

Question 4

Pediatric demyelinating syndromes are characterized clinically by…?

Answer 4

1) localization of neurologic deficits (i.e., a single site, such as the spinal cord [transverse myelitis, TM], optic nerves [optic neuritis, ON], or brainstem versus a polyregional demyelination);
(2) the presence or absence of encephalopathy;
(3) the disease course (i.e., monophasic versus repeated attacks involving either the same region or new CNS regions); and
(4) the presence or absence of specific antibodies.

Question 5

What is ADEM ….?

Answer 5

ADEM is an inflammatory, demyelinating event of early childhood presenting with an acute onset of polyfocal neurologic deficits, accompanied by encephalopathy and changes compatible with demyelination on brain MRI

Question 6

What is multiphasic disseminated encephalomyelitis (MDEM) ..,?

Answer 6

ADEM is usually monophasic, but recurrence can occur; if the recurrence is 3 mo or longer after the first episode, the condition is termed multiphasic disseminated encephalomyelitis (MDEM)

Question 7

MS may be diagnosed by characteristics of demyelination..?

Answer 7

An episode of ADEM can also be followed by non-ADEM demyelination in a new location. In this scenario, if the MOG-Ab is negative, MS may be diagnosed

Question 8

What is ADEM-ON ….?

Answer 8

If ADEM is followed by a relapse in a specific location, such as the optic nerve (ON), then ADEM-ON is diagnosed

Question 9

What is Neuromyelitis optica spectrum disorders (NMOSDs) ….?

Answer 9

If the ON(optic nerve) and spinal cord are involved, then NMOSD

Question 10

Hallmark of ADEM …?

Answer 10

Encephalopathy is the hallmark of ADEM, ranging from changes in behavior and persistent irritability to coma

Question 11

Imaging study of choice for ADEM ….?

Answer 11

MRI

Question 12

What is Clinically isolated syndrome (CIS) ..?

Answer 12

A first monofocal or multifocal CNS demyelinating event Encephalopathy is absent, unless caused by fever

Question 13

Multiple sclerosis (MS) definition..?

Answer 13

MS can be diagnosed in those for whom there is no better explanation if dissemination in time (DIT) and dissemination in space (DIS) can be demonstrated.

DIS : is met when there are neurologic lesions affecting separate sites (periventricular, juxtacortical, infratentorial, or spinal regions) within the CNS

DIT : is met when MRI demonstrates the simultaneous presence of a gadolinium-enhancing lesion and nonenhancing lesion OCBs are positive in the CSF or follow-up MRI after at least 30 days shows accumulation of a new T2 lesion

Question 14

What is Weston-Hurst disease ……?

Answer 14

Severe involvement may progress to an acute hemorrhagic leukoencephalopathy (Weston-Hurst disease) with large lesions, edema, mass affect, and a polymorphonucleated cell pleocytosis

Question 15

Which is the Most common of the acquired demyelinating syndromes …..?

Answer 15

Optic neuritis (ON)

Question 16

What is the typical presentation of Optic neuritis (ON) ……?

Answer 16

T he typical presentation is unilateral or bilateral visual loss over hours to days, abnormal color vision, visual field loss, and sometimes a relative afferent pupillary defect