160	8 Multiple Sclerosis and other Myelinopathies
		Table 8.2 Differential diagnosis of multiple sclerosis
		Clinical manifestation	Possible alternative diagnoses	Useful criteria for differential diagnosis
		Lhermitte sign	status post traumatic brain injury, status post radiother-	accompanying history and physical findings
			apy; pathological process in the thoracic spinal cord, the
			dorsal cervical spinal cord, or the junction of the spinal
			cord and medulla; vitamin B12 deficiency

Intermittent visual loss amaurosis fugax in carotid stenosis; amblyopic attacks in papilledema

age of the patient, signs of intracranial hypertension, duration of episodes, cervical bruits

Optic disc pallor	optic nerve compression by a mass	slowly progressive visual impairment; mass on
		CT

Paresis of extraocular diabetic mononeuritis; compression of one or more muscles cranial nerves by a mass, e. g., at the skull base or in the

region of the cavernous sinus

diabetes, pain; tumor or aneurysm on CT or MRI

Nystagmus

Progressive spastic paraparesis

Intermittent paraparesis

Symptoms and signs arising from multiple foci in the CNS, all at once or with relapses and recurrences

disease affecting the cerebellum

spinal cord compression

arteriovenous fistula

spastic spinal paralysis

parasagittal mass

vascular spinal cord lesion (e. g., arteriovenous malformation)

benign spinal cord tumor (e. g., lipomatosis after prolonged steroid treatment)

multiple vascular lesions in the CNS

other cerebellar signs

slow progression, sensory level

relapsing course

no relapses or remissions; purely motor deficit

possibly, headache

very rapid or sudden worsening

look for a sensory level; is it constant or variable?

simultaneous involvement of other organs (e. g., coronary artery disease, occlusive peripheral vascular disease)

vascular risk factors

Other Demyelinating Diseases of Unknown Pathogenesis

In addition to the genetic, immune-mediated, and metabolic myelinopathies, there are a number of other demyelinating diseases whose causes remain unknown. These diseases are briefly listed and characterized in Table 8.3.

Table 8.3 Rarer demyelinating diseases

Disease	Clinical manifestations	Special features
Concentric sclerosis (Baló sclerosis,	onset at any age, slow progression, associated	concentric bands of demyelination
periaxial encephalitis)	with dementia

Acute disseminated	acute multiple sclerosis; simultaneous appear-
encephalomyelitis (ADEM)	ance of symptomatic foci in the brain and spi-
	nal cord; acute onset, with fever; peripheral
	nervous system also involved
Neuromyelitis optica	simultaneous appearance of symptomatic foci
(Devic disease)	in the optic nerve and spinal cord; mainly af-
	fects young women

MRI shows foci that arose simultaneously; histopathological examination shows axonal injury as well; dramatic course; responds to cortisone and immunosuppressive drugs

elevated CSF protein; no oligoclonal bands in CSF; inflammatory axonal damage

Subacute myelo-optic	ascending paresthesiae and weakness of the
neuropathy (SMON)	lower limbs, appearing days or weeks after a
	gastrointestinal illness; the optic nerve is also
	involved in one-third of patients

most common in Japan; many patients have a prior history of oxyquinoline use

Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.