The Olfactory Nerve

First cranial nerve

Unique - Constituting the only examples
of neuronal regeneration in humans
- The olfactory cells = Receptor
cells = Bipolar neurons
- Olfactory impulses reach the
cerebral cortex without relay through
the thalamus
The primary olfactory cortex=medial and
cortical nuclei of the amygdaloid
complex and the prepiriform area

Connected with the neighboring
entorhinal cortex and medial dorsal
nucleus of the talamus

To be perceived as an odor, an inhaled

sustance must be volatile
 Disturbances of olfaction

1. Quantitative abnormalities: anosmia,

hyposmia, hyperosmia
2. Qualitative abnormalities: distortions
or illusions of smell dysosmia or
parosmia
3. Olfactory halucinations
4. Olfactory agnosia
 Anosmia
If unilateral usually not recognized by the patient
Categories - nasal: odorants do not reach the
olfactory receptors - hipertrophy and hiperemia of
the nasal mucosa (havy smoking, chronic rhinitis
and sinusitis)
- neuroepithelial: distruction of
receptors or their axon filaments influenza,
herpes simplex, hepatitis virus infections; local
radiation therapy; esthesioneuroblastoma; Kallman
syndrome; toxic agents (benzen); head injury
- central: olfactory pathway lesions,
head injury, tumors, aneurysms
 The Foster Kennedy syndrome
A meningioma of the olfactory groove may implicate
the olfactory bulb and tract and may extend
posteriorly to involve the optic nerve
Clinical - ipsilateral: anosmia and optic atrophy
- opposite site: papilledema

Anosmia or hyposmia in
Parkinson disease and Multiple
sclerosis for reasons that are
quite unclear
 Hyperosmia
Migraine attacks, aura in epilepsy, neurotic
individuals

Dysosmia or Parosmia
Pervertion of the sense of smell - in local
nasopharingeal conditions such as empyema of
the nasal sinuses ex: cacosmia and cacogeuzia in
ozena
- in middle-aged
and elderly persons with depression
 Olfactory halucinations
Are always of central origin
Episodic in temporal lobe seizures or as
aura in epilepsy
In combinations with delusions in
psychiatric illnesses (endogenous in
depression and exogenous in
schizophrenia) or in dementia
In alcohol withdrawal syndrome
 Olfactory agnosia
The primary perceptual aspects of
olfaction are intact, but the capacity to
distinguish between odors and the
recognition is impared or lost
Is most likely due to lesions in the medial
dorsal nucleus of the thalamus
Characterize patients the alcoholic form of
Korsakoff psychosis
The Optic Nerve
 The second cranial nerve
The photoreceptors are the rod cells and the cones
cells
The bipolar cells = the first neuron
The ganglionic cells = the second neuron

the axons traverse the optic discthe optic nerve; the
nasal fibers cross in the optic chiasmoptic tract
lateral geniculate body(the third neuron) visual
radiations visual striate cortex
Pupillary fibers optic nerve optic tract
terminate in the pretectum and both Edinger-
Westphal nuclei which subserve puppilary
constriction
 Abnormalities of vision
1. Reduced or loss of vision
2. Visual field defects
3. Positive sensory visual experiences
4. Abnormalities of colour vision
5. Visual agnosia
6. Visual halucinations

The diagnosis is based on:

- the historical data (age of the patient at the time
of onset, mode of onset, evolution)
- the topography of the lesion
Optic nerve examination involves:

Test of visual acuity

Examination of the visual field

FO exam
A. Transient monocular blindness
-Amaurosis fugax (TIA)
-Migraine

B. Irreversible monocular blindness

acute onset
-Ocular pathology
-Vascular occlusion
-Ischemic optic neuropathy
-Leber neuropathy
C. Progressive (evolves over hours-
several days) impairment of visual
acuity
-Typical/atipical optical neuropathies:
granulomatous, parainfectious, imune

D. Progressive (evolves over days-

months) impairment of visual acuity
-Compressive and infiltrative neuropathies
-Chronic inflamatory diseases
-Heredo-familial neuropathies
 Abnormalities of the optic nerve
Can be inspected only in the optic nerve head
may reflect:
-the presence of raised intracranial pressure -
papilledema
-optic neuritis papillitis
-infarction of the optic nerve head disc edema
-congenital defects of the optic nerve
colobomas
-hypoplasia and atrophy of the optic nerve
-glaucoma
 Papilledema
Has a great neurologycal significance increased
intracranial pressure
Steps of evolution:
1. blurring and slight elevation of the disc especially
of the superior and inferior margins hypermetropia
2. disappearence pulsations of the retinal veins
3. mushrooming of the entire disc and surrounding
retina with edema and obscuration of vessels at the
disc margins and peripapillary hemorrhages
4. when advanced, papilledema is almost always
bilateral
5. as it becomes chronic, elevation of the disc margin
becomes less prominent and pallor of the optic nerve
head more evidentoptic atropy
 Acute papilledema does not affect visual acuity except
during waves of greatly increased pressure
Differential diagnosis:
-papillitis (severly reduced vision)
-infarction of the nerve head (severe loss of vision;
extension of the swelling beyond the nerve head)
Papilledema without raised intracranial pressure may
occure in children with cyanotic congenital heart
diseases and polycythemya
 Main causes of unilateral and
bilateral optic neuropathy
I. Demyelinative (optic neuritis)
-Multiple sclerosis, Devic
-Postinfectious and viral neuroretinitis
II. Ischemic
-Arterioslerotic
-Granulomatous (giant cell) arteritis
-Syphilitic arteritis
III. Parainfectious
-Cavernous sinus thrombosis
-Paranasal sinus infection
IV. Toxins and drugs
-Methanol, ethambutol, chloroquine, streptomicin, ergot
V. Deficiency states
-B 12
-Thiamine (tobacco-alcohol amblyopia)
VI. Heredofamilial and developmental
-Leber optic atrophy
VII. Compressive and infiltrative
-Meningioma of sphenoid wing or olfactory roove
-Metastasis to optic nerve or chiasm
-Glioma of optic nerve (neurofibromatosis type I)
-Optic atrophy following long standing papilledema
-Thyroid ophtalmopathy
-Sarcoid
-Wegener granulomatosis
-Lymphoma and leukemia
 Papillitis and the Syndrome of Retrobulbar
Neuropathy (Optic Neuritics)
Clinical acute impairment in vision in one or both eyes (the eyes may
be affected either simultaneously of succesively)
- scotoma
- impairment of color vision
- pain on movement and tenderness on presure of the globe
- the pupil on the affected side- mute response to direct light
FO exam swelling of the optic disc; the disc margins are elevated,
blurred and rarely surrounder by hemorrhages
PEV modified
CSF may be normal or may contain 10-100 Lf, protein, -globulin,
oligoclonal bands
Recovery in 2-6 weeks; vision returns to normal in more than 2/3 of
cases
Demyelinative disease is the most common cause of unilateral
retrobulbar neuritischeck-up for MS
 Ischemic Optic Neuropathy
In persons over 50 years of age is the most common cause
of persistent monocular loss of vision
Clinical - abrupt onset
- painless
- visual field defect is often altitudinal and involves
the area of central fixation, accounting for the severe loss of
acuity
- 1/3 cases bilateral affection (HTA, DZ)
FO exam swelling of the optic disc and beyond the disc
margin small, flamed-shaped hemorrhages
Pathogenesis ischemia in the posterior ciliary artery
circulation; in cranial or giant-cell arteritis; may complicate
intraocular surgery of severe blood loss or other type of
ischemia and hypotension
 Toxic and nutrition optic neuropathies
Clinical - impairment of vision in the two eyes evolves over
several days or a week or two
- centrocecal scotomas
Ex: tobbaco-alcohol amblyopia or B 12 deficiency

Heredofamilial neuropathies
Hereditary Optic Atrophy of Leber = mitochondrial disease
Age of onset 20-30 years
Clinical - acute onset amblyopia
- after weeks and months the second eye is
affected
- central vision is affected before peripheral vision
- painless
- the vision loss is irreversible
 Lesions of the Chiasm, Optic tract and
Geniculocalcarine Pathway
Generates hemianopia (hemianopsia) = blindness in half of
the visual field
a. Lesions in the chiasmbitemporal hemianopia: extrasellar
extension of a tumor of the pituitary gland,
craniopharyngioma, sacular aneurysm of the circle of Willis
binasal hemianopia: arachnoiditis
b. Lesions in the optic tractincongruous homonymous
hemianopia + RFM absent
c. Lesions in the visual radiationshomonymous quadran
anopia
d. Lesions in the visual cortexcongruous homonimous
hemianopia
e. Lesions of both occipital lobescortical ambliopia
below or above the calcarine
sulcushomonimous altitudinal hemianopia
 Visual Agnosia
Disturbance of central origin; pacients cannot understand
the meaning of what they see

Positive sensory visual experiences

Phosphenes (flashes of light and colored spots in the
absence of luminous stimuli)
-occur in migraine, epilepsy
Visual halllucinations
-simple and unformed in epilepsy
-complex or formed (people, animals) in the withdrawal
state following chronic intoxication with alcohol, in Alzheimer
disease, diseases of occipito-parietal or occipito-temporal
lesions
The Trigeminal Nerve
 The fifth cranial nerve; is a mixt, sensory and motor nerve
It conducts sensory impulses from the greater part of the face
and head; from the mucous membranes of the nose, mouth and
paranasal sinuses; from the cornea and conjunctiva; it also
inervates the dura of the anterior and middle cranial fossae
The motor portion of the fifth nerve supplies the masseter and
pterygoid muscles
To exam the fifth nerve you must check-up:
-the sensibility in the oftalmic, maxilar and mandibular teritory
-movements of the mandibula against opposition (the motor
fibers are seldom affected; a motor deficit is observed in
pseudobulbar palsy)
-brainstem reflexes: corneal reflex, jaw jerk
Because of their wide anatomic distribution, complete
interruption of both the motor and sensory fibers is rarely
observed
 Trigeminal neuralgia
A. Idiopatic mean age of onset is 52-58 years
=Tic Douloureux=paroxysms of intense, stabbing pain
- in the distribution of the mandibular and maxilary divisions
- the pain seldom lasts more than a few seconds and recur
frequently
- is so intense that the patient involuntarly winces
- initiated by stimulation of certain areas of the face, lips or
gums as in shaving or brushing the teeth or by chewing,
talking or yawning=trigger zones
- the clinical exam is normal
- the mechanism of paroxismal pain is in the nature of
allodynia
 Strict criteria for TN as defined by the International
Headache Society (IHS) are as follows[1] :

A Paroxysmal attacks of pain lasting from a fraction of

a second to 2 minutes, affecting 1 or more divisions of
the trigeminal nerve and fulfilling criteria B and C
B Pain has at least 1 of the following characteristics:
(1) intense, sharp, superficial or stabbing; or (2)
precipitated from trigger areas or by trigger factors
C Attacks stereotyped in the individual patient
D No clinically evident neurologic deficit
E Not attributed to another disorder
- differential diagnosis: a) other forms of facial and cefalic
neuralgia and pain arising from diseases of the jaw, teeth
or sinuses
b) symptomatic forms
- is important to have a MRI-may reveal a possible cause-in
the elderly vascular compression of the trigeminal
ganglion
- prognosis most patients can be treated effectively
medicaly
- treatment:
1. medications used: anticonvulsant drugs-
carbamazepine 600-1200 mg/day, gabapentin, lamotrigine
phenytoin, clonazepam, sodium valproate,
2. surgical options: radiofrequency thermocoagulation,
microvascular decompression, stereotactic radiosurgery
 Treatment of TN comprises the
following:

Pharmacologic therapy
Percutaneous procedures (eg,
percutaneous retrogasserian glycerol
rhizotomy)
Surgery (eg, microvascular
decompression)
Radiation therapy (ie, gamma knife
surgery
 Features of pharmacologic therapy are as follows:

Single-drug therapy may provide immediate and satisfying relief

Carbamazepine is the best studied drug for TN and the only one
with US Food and Drug Administration (FDA) approval for this
indication
Because TN may remit spontaneously after 6-12 months, patients
may elect to discontinue their medication in the first year following
the diagnosis; most must restart medication in the future
Over the years, patients may require a second or third drug to
control breakthrough episodes and finally may need surgical
intervention
Lamotrigine and baclofen are second-line therapies
Controlled data for adding a second drug when the first fails exist
only for the addition of lamotrigine to carbamazepine
Gabapentin has demonstrated effectiveness in TN, especially in
patients with multiple sclerosis
B. Symptomatic - mean age of onset is 30-35 years, caused
by trauma, vascular, neoplastic and demyelinative diseases
the branches of the trigeminal nerve can be affected in: -
inflamatory and infectious diseases: HSV infection, HZV
infection, middle ear infections, osteomyelitis of the apex of
the petrous bone, LES, Sjgren syndrome
-demyelinative diseases: Multiple sclerosis, especially in
young patients
-trauma
-vascular diseases: aneurysmal dilatation of the basilar
artery or an arteriovenous malformation
-neoplasia: the trieminal root can be compressed or
invaded by intracranial meningiomas, acoustic neuromas,
trigeminal neuromas; in elderly-infiltrative glioma of the
brainstem; can be the first sign of metastatic diseases
especially from carcinoma of the brest and prostate and
multiple mieloma
 In rare cases trigeminal neuralgia is
preceded or accompanied by
hemifacial spasm, a combination that
Cushing called tic convulsif
The Facial Nerve
The facial nerve (cranial nerve VII) carries motor, secretory,
and afferent fibers from the anterior two thirds of the
tongue.
It originates in the facial nucleus, which is located at the
caudal pontine area.
Corticobulbar fibers from the precentral gyrus (frontal
lobe) project to the facial nucleus, with most crossing to
the contralateral side. As a result, crossed and
uncrossed fibers are found in the nucleus.
Moreover, the facial nucleus can be divided into two
parts: (1) the upper part, which receives corticobulbar
projections bilaterally and later courses to the upper
parts of the face, including the forehead, and
(2) the lower part, the predominantly crossed
projections of which supply innervation to lower facial
muscles (stylohyoid; posterior belly of digastric,
buccinator, and platysma).
 The nervus intermedius

conveys (1) afferent taste fibers from the chorda tympani nerve,
which come from the anterior two thirds of the tongue; (2) taste
fibers from the soft palate via the palatine and greater petrosal
nerves;
(3) preganglionic parasympathetic innervation to the
submandibular, sublingual, and lacrimal glands.
The fibers for taste originate in the nucleus of the tractus solitarius
(NTS),
and the fibers to the lacrimal, nasal, palatal mucus, and
submandibular glands originate in the superior salivatory nucleus.
Fibers to the lacrimal gland are carried with the greater superficial
petrosal nerve until it exits the skull, at which point the fibers branch
off as the Vidian nerve.
(4) The nervus intermedius also has a small cutaneous
sensory component from afferent fibers originating from the skin of
the auricle and postauricular area
 Is the seventh cranial nerve; is a mixt, mainly motor
nervesupplies all the muscles corcerned with facial
expression on one side
sensory component is small (the anterior wall of the
external auditory canal)
convase taste sensation of the 2/3 anterior of the
tongue
secretomotor fibers innervate the lacrimal gland,
sublingual and submaxilary glands
The exam of the facial nerve:
-exam of facial movements
-exam of the sensibility
-exam of the taste and the lacrimal gland, sublingual and
submaxilary glands
-brainstem reflexes (corneal reflex)
 Facial Palsy
A. Supranuclear type
- it manifests only in the lower part of the face, since the upper
facial muscles receive upper motor neuron innervation from
the motor cortex of the both hemispheres
B. Peripheral type
- the skin folds are effased
- the forehead is unfurrowed
- the palpebral fissure is widened
- the eyelids will not close when attempted both eyes roll
upward
- the tears spill over the cheek
- the salyva may dribble from the corner of the mouth
- Ethiology:
Idiopatic=Bells Palsy
-the most common disease of the facial nerve
-occurs in all ages
-the onset is acute, attain maximum paralysis in 48
hours
-pain behind the ear may precede the paralysis by a day
or 2 +/- impairment of taste and hyperacusis or distortion of sound
in the ipsilateral ear (paralysis of the stapedius muscle)
-MRI: gadolinium enhanced of the facial nerve
-CSF: Lf, mononuclear cells; important for diffential
diagnosis of GB syndrome and Lyme disease
-prognostic: 80% recover in a few weeks, recovery of
taste preccedes recovery of motor function; early recovery of the
motor function in the first 5-7 days is the favorable prognostic sign
- ! EMG
-treatment: prednisone 40-60 mg/day during the first
week to 10 days + vitamins + massage of the weakened muscles
+ protection of the eye during sleep +/- surgical lid closure +/-
acyclovir
 Secundary:
-inflamatory and infectious diseases: Lyme disease, HIV
infection, TBC, HZV infection (Ramsey Hunt syndrome), otitis
media
-neoplasia: tumors that invade the temporal bone
tumors of the ponto-cerebelar angle: acustic
neuromas, neurofibromas
-trauma: fracture of the temporal bone, middle ear surgery
-aneurysmal dilatation of the vertebral or basilar artery
*intranevraxial lesions may be - vascular
- demyelinative
- neoplastic

Millard-Goubler Syndrome
Foville Syndrome
! Bilateral Bells palsy is most often manifestation of the GB
syndrome, Lyme disease, HIV,sarcoidosis
 Hemifacial spasm
First described by Gowers in 1884,
represents a segmental myoclonus of muscles
innervated by the facial nerve.
almost always unilaterally, although bilateral
involvement may occur rarely in severe cases.
Hemifacial spasm generally begins with brief
clonic movements of the orbicularis oculi and
spreads over years to other facial muscles
(corrugator, frontalis, orbicularis oris, platysma,
zygomaticus
 The causes - include
vascular compression,
facial nerve compression by a mass,
brainstem lesions such as stroke or
multiple sclerosis plaques,
and secondary causes such as trauma or
Bell palsy ,
dystonia
 Compressive lesions (eg, tumor, arteriovenous malformation, Paget
disease) and noncompressive lesions (eg, stroke, multiple sclerosis
plaque, basilar meningitis) may present as hemifacial spasm.

Most instances of hemifacial spasm

previously thought to be idiopathic were
probably caused by aberrant blood
vessels (eg, distal branches of the anterior
inferior cerebellar artery or vertebral
artery) compressing the facial nerve within
the cerebellopontine angle
 treatment

In most patients with hemifacial spasm, the treatment of choice is

injection of botulinum toxin under electromyographic (EMG)
guidance. Chemodenervation safely and effectively treats most
patients, especially those with sustained contractions. Relief of
spasms occurs 3-5 days after injection and lasts approximately 6
months.
Medications used in the treatment of hemifacial spasm include
carbamazepine and benzodiazepines for noncompressive lesions.
Carbamazepine, benzodiazepines, and baclofen also may be used
in patients who refuse botulinum toxin injections.
Compressive lesions need to be treated surgically. Microvascular
decompression surgery may be effective for those patients who do
not respond to botulinum toxin