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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


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









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


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 disc→the optic nerve; the

nasal fibers cross in the optic chiasm→optic 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


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


  • 5. as it becomes chronic, elevation of the disc margin

becomes less prominent and pallor of the optic nerve

head more evident→optic 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


-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, ↑ γ-globulin, oligoclonal bands

Recovery in 2-6 weeks; vision returns to normal in more than 2/3 of


Demyelinative disease is the most common cause of unilateral retrobulbar neuritis→check-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


  • - 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 chiasm→bitemporal 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 tract→incongruous homonymous hemianopia + RFM absent

  • c. Lesions in the visual radiations→homonymous quadran anopia

  • d. Lesions in the visual cortex→congruous homonimous hemianopia

  • e. Lesions of both occipital lobes→cortical ambliopia →below or above the calcarine sulcus→homonimous 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


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, Sjögren syndrome

-demyelinative diseases: Multiple sclerosis, especially in young patients


-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


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 nerve→supplies 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


→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=Bell‘s Palsy -the most common disease of the facial nerve -occurs in all ages -the onset is acute, ½ attain maximum paralysis in 48


-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


-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 Bell‘s 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,


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 ,


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


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