diseases of nerves
DESCRIPTION
Diseases of NervesTRANSCRIPT
MIGRAINE:
•Also called migraine syndrome.
•It is dominantly inherited disorder characterized by varying degree of recurrent vascular headache, photophobia, sleep disruption and depression.
•Is a paroxysmal, unilateral and rarely bilateral, disabling headache.
Etiology:
•Mechanism is not completely understood.
•It is thought to be vasoconstriction or vasospasm of portions
of cerebral arteries leading to cerebral ischemia, followed
by vasodialation with subsequent pain and cerebral edema.
Clinical features:
•Prevalent in 3rd through 5th decade of life but 1st symptoms often begin at puberty .
•Affects women more than men.
•Frequency of attacks is variable, may occur at frequent intervals over a period of years or only few occasions during lifetime.
•Initially pain is mild, with time severity increases.
•Patient experience an aura before headache. •Aura appear as lethargy, dejection, hallucinations or scotomas.
•Other less common prodromal phenomena includes confusion, unilateral paresthesia , facial weakness.
•Headache phase consists of severe pain in temporal, frontal and retro-orbital areas.
•Other sites occasionally involved are parietal, postauricular, occipital, suboccipital.
•Pain is deep, aching, throbbing type.
•Patient appears ill, face is pale, sallow, sweaty
•Patient is irritable, fatigued, memory and concentration are
impaired, anorexia, vomiting, visual disturbances may
occur.
•Prolonged and painful contraction of head and neck
muscles is found in some patients.
Treatment:
•Includes variety of drugs- acetylsalicyclicacid , codeine , ergotamine, methysergide, norepinephrine.
•Prognosis is good and condition may undergo complete and permanent remission.
MCQ:
1. Following are the inflammatory disturbances of TMJ except:a. Traumatic arthiritisb. Osteoarthritisc. Ankylosisd. Rheumatoid arthritis
2. Which one of the following is associated with extraraticular disturbances of TMJ:a. Costen syndromeb. Trotter’s syndromec. MPDSd. Both a & c
3. Resorption of articular eminence and sclerotic underlying bone seen in:a.Osteoarthritisb.Rheumatoid arthritisc.Costen syndromd.Ankylosis
4. Rose waller test is done to investigate:a.Ankylosisb.Rheumatoid arthritisc.Hypermobilityd.Both a & C
5. Bird face appearance is the clinical feature of :a.Unilateral ankylosisb.Hypermobilityc.Bilateral ankylosisd.Both a & c
6. The following contribute to mouth opening:a. Medial pterygoid muscleb. Lateral pterygoid musclec. Masseter muscled. Temporalis muscle
7. The following radiographs/imaging methods can be used to measure joint space width:a. Panoramic radiographb. Transpharyngeal radiographc. Transcranial oblique lateral radiographd. Transorbital (Zimmer) radiograph
8. Condylar hyperplasia:a. Is a developmental disorderb. Is an inflammatory disorderc. Causes ankylosisd. None of the above
9. Erosion of the condyle may occur in:a. Pain/dysfunctionb. Internal derangementc. Psoriasisd. b & c both
10. Deviation to the left side on opening could be caused by:
a. Right TMJ disc anterior displacement without reduction
b. Left TMJ disc anterior displacement without reduction
c. Right TMJ effusion
d. a & c both
11. Syndrome asociated disease of maxillary sinus:a. Crouzon syndromes b.Trecher syndromes c. Binder syndrome d.All of the above
12. Inflammatory disease of maxillary sinusa. Mucocele b.Maxillary sinusitis c. Emphyema d.B & C
13. Early Synostosis of the suture produces hypolplasia
of maxilla and therefore the maxillary sinus , together with
the high arched palate found in :
a. Crouzon syndromes
b. Trecher syndromes
c. Binder syndrome
d. All of the above
14. When inflammation develops in the sinus either due to infection or allergy, it is termed as:
a. Mucocele b.Maxillary sinusitis c. Emphyema d.Mucocitis
15. Rare tumour of respiratory epithelium that
occurs in the nasal cavity and paranasal sinuses:
a. Antral papilloma
b. Oesteoma
c. Ameloblastoma
d. None of the above
III. DISTURBANCES OF NINTH CRANIAL NERVE:
GLOSSOPHARYNGEAL NEURALGIA:
•Pain similar to that of trigeminal neuralgia but not as common as that
Clinical features:
•Etiology is unknown.
•No gender predilection is there.
•Occurs in middle-aged or older persons.
•Certain actions precipitate these trigger zones as swallowing,
talking, yawning or coughing.
•Manifests as sharp, shooting pain in ear, pharynx, nasopharynx, tonsil or posterior portion of tongue, almost unilateral, rapidly subsiding type of pain.
•Patient has trigger zones in posterior oropharynx or tonsillar fossa.
Diagnosis:
•Much the same as for trigeminal neuralgia, is a clinical
diagnosis based on history and examination.
•Computed tomography and MRI are prescribed to detect
related intra or extracranial disease.
Treatment:
• Resection of extracranial portion of nerve or intracranial section.
• Many patients require use of membrane stabilizing medications or tricyclic or heterocyclic medications.
• Periods of remission with recurrence are common
ATYPICAL FACIAL PAIN
Common form of facial pain
Described as continuous dull ache with intermittent excruciating
throbbing episodes
Localized to non – muscular site as alveolar bone or over
maxillary antrum
Pain may be bilateral with wide extra facial distribution
Not provoked by jaw movements and rarely relieved by analgesics
Strong resemblance to facial migrainous neuralgia with sensation
of nasal stuffiness or obstruction and pain waking patient in early
hours of morning
Bouts of pain last for hours or days
Patient have history of intermittent pain over period of many years
COMMON FEATURE 1. Pain provoked by trauma or dental treatment
2. In older edentulous case, patient cannot wear dentures
despite bone smoothing procedures and prostheses
with and without soft linings
3. Marked hyperaemia of oral mucosa or slight oedema
of face
Term introduced by Frazier and Russell (1924)
Patient displays certain recognizable characteristics as
- obsessive but dependent personality
- inadequate support from parents or spouse,
- extreme protectiveness or guilt in relation to child who have
some congenital problem as epilepsy or cleft palate
Pain exacerbated at times when these close
relationships disturbed by illness or death
FEATURES SUGGESTIVE OF PSYCHOGENIC (ATYPICAL) FACIAL PAIN:
Women of middle age or older mainly affected
Absence of organic signs
Pain often poorly localised
Description of pain may be bizarre
Delusional symptoms occasionally associated
Lack of response to analgesics
Unchanging pain persisting for many years
Lack of any triggering factors
Common site for pain is maxillary region or in
relation to upper teeth
Patient's sleeping or eating may be unaffected
Although teeth have been extracted and
diseased teeth may be present, none of these
can be related to the pain
Treatment of diseased teeth does not relieve
the symptoms
TREATEMENT-
Symptoms cause suffering to patient and should
be relieved
Important to avoid unnecessary surgery
Sometimes good response to antidepressive
treatment
Atypical odontalgia (idiopathic periodontalgia, Phantom tooth pain):
Usually due to infective or traumatic inflammation of pulp or
periodontal membrane
Pain severe and throbbing in character
Teeth hypersensitive to any stimulus
Often widespread and bilateral, occasionally localized
Some cases precipitated by dental procedure as fitting of
bridge or extraction
Important differential diagnosis is "split tooth"
Here vertical crack in vital tooth detected with fibre-optic light
Bruxism in patients with long standing atypical odontalgia give
rise to split tooth
Which despite treatment or extraction does not relieve pain
One is confronted by patient with history of pain accelerated by
unnecessary pulp extirpations and extractions
Remaining teeth even when sound and vital are tender to
percussion
History reveal significant emotional problems as
period of depression, especially where drug
therapy has ceased
Occasionally,hypotensive drug therapy
precipitate condition
These patients best considered to have variant of
AFP and treated in same manner
Condition considered deafferentation neuralgia arising when extraction or pulp extirpation produces either
o amputation neuroma or o central degenerative change in trigeminal nucleus (Marbach,
1986) Many cases arise without history of extraction or pulp
extirpation Atypical odontalgias not abolished with dental local analgesic
block as in deafferentation neuralgia Attempts to curette or excise microscopic neuromas have little
effect beyond 1-2 weeks
Repeated root canal therapy and local
surgery could produce causalgia
Management is that of AFP, using tricyclic
antidepressants and phenothiazines
Some patients experience relief lasting 4-
6 days following stellate ganglion block
IV. MISCELLANEOUS DISTERBANCES OF NERVES:
OROFACIAL DYSKINESIA
•Condition is thought to be result of either an extrapyramidal disorder or complication of phenothiazine therapy
•Edentulous patients with both upper and lower dentures in gross malocclusion, exhibited involuntary movements typical of orofacial dyskinesia
•Symptoms disappear when dentures with proper physiologic craniomandibular relationships were given
Clinical features:
•Occurs in persons over 60 years.
•Characterized by severe, involuntary ,dystonic movements of facial, oral and cervical musculature.
•Movements as lip-smacking ,lip-licking,protrusion of lips ,protrusion of tongue and mandible are seen.
•Condition can occur alone or inassociation with torticollis or generalized dystonia
Treatment:
•Surgical operations as in case of parkinson’s disease causes improvement in symptoms.
•Correction of denture occlusion may be an effective procedure.
MIGRAINE:
•Also called migraine syndrome.
•It is dominantly inherited disorder characterized by varying degree of recurrent vascular headache, photophobia, sleep disruption and depression.
•Is a paroxysmal, unilateral and rarely bilateral, disabling headache.
Etiology:
•Mechanism is not completely understood.
•It is thought to be vasoconstriction or vasospasm of portions
of cerebral arteries leading to cerebral ischemia, followed
by vasodialation with subsequent pain and cerebral edema.
Clinical features:
•Prevalent in 3rd through 5th decade of life but 1st symptoms often begin at puberty .
•Affects women more than men.
•Frequency of attacks is variable, may occur at frequent intervals over a period of years or only few occasions during lifetime.
•Initially pain is mild, with time severity increases.
•Patient experience an aura before headache. Aura appear as lethargy, dejection, scintillations, hallucinations or scotomas.
•Other less common prodromal phenomena includes vertigo, aphasia, confusion, unilateral paresthesia , facial weakness.
•Headache phase consists of severe pain in temporal, frontal and retro-orbital areas.
•Other sites occasionally involved are parietal, postauricular, occipital, suboccipital.
•Pain is deep, aching, throbbing type.
•Patient appears ill, face is pale, sallow, sweaty
•Patient is irritable, fatigued, memory and concentration are
impaired, anorexia, vomiting, visual disturbances may
occur.
•Prolonged and painful contraction of head and neck
muscles is found in some patients.
Treatment:
•Includes variety of drugs-acetylsalicyclicacid , codeine , ergotamine, methysergide, norepinephrine.
•Prognosis is good and condition may undergo complete and permanent remission.
TEMPORAL/GIANT CELL ARTERITIS/CRANIAL ARTERITIS:
•It is the cause of headache called as atypical migraine.
•It is basically focal granulomatous inflammation of arteries especially cranial vessels.
•Temporal arteries are more prone to develop these lesion.
•When lesion found throughout the skeletal muscles,condition termed as polymyalgia arteritica.
Etiology:
•It is a disease of cellular immunity.
•Vasculitic damage is mediated by activated CD4+T helper cells responding to an antigen presented by macrophages.
•Inflammatory response affects internal elastic lamina.
•Multinucleated giant cells are histologic hallmark of this condition.
Clinical features:•Occurs in older persons between 55 to 80 years.
•Onset may be slow and insidious or may develop suddenly with headache , burning , throbbing pain.
•Pain is preceded by malaise , chills , fever , weight loss , anorexia , nausea ,vomiting and with increased erythrocyte sedimentation rate.
•Pain may be localized first in teeth ,TMJ ,scalp ,occiput.
•Patient complains of tiredness ,fatigue ,pain on repetitive chewing.
•Affects women more than men.
•Sometimes followed by aching and stiffness of muscles of shoulders and hips,termed as polymyalgia rheumatic.
•Localized inflammation or cellulitis over swollen ,nodular ,tortuous artery is seen.
•Other symptoms may be eye pain,photophobia,diplopia and even blindness.
•ESR is raised,mild leukocytosis may be found.
•Unilateral , arising during mastication or wearing hats.
Histologic features:
•Intima and tunica media proliferation with resultant luminal
stenosis , disruption of internal elastic lamina by mononuclear cell
infiltrate , invasion and necrosis of media progressing to
panarteritic involvement by mononuclear cells , giant cell
formation with granulomata within mononuclear cell infiltrate ,
intravascular thrombosis.
Treatment:
•Corticosteroid therapy has good response.
COMPLEX REGIONAL PAIN SYNDOME:
•Also called as causalgia.
•Term applied to severe pain which arises after injury or sectioning of peripheral sensory nerve.
Etiology:•Some believe development of condition requires triad of conditions: an injury , abnormal sympathetic response , predisposing personality.
Clinical features:•Develops in any age.
•Usually follows extraction of multirooted tooth when it has been traumatic.
•Pain arises within few days to several weeks after extraction.
•Emotional disturbance ,ingestion of alcohol,menstrual periods can also elicit the pain
•It has a typical burning quality and is evoked by contact of
heat or cold.
Differential diagnosis:
•Local pain due to traumatic injury to soft tissue or bone during extraction.
•Subacute thyroiditis-produces referred pain in posterior portion of mandible,occurs in over 35% of patients with thyroiditis.
Treatment:
•In some instances, resection of nerves in retrogasserian region has afforded relief
HORNER’S SYNDROME:
•Also called sympathetic ophthalmoplegia.
•It is condition characterized by miosis or contraction of pupil of eye due to paresis of dilator of pupil.
•Ptosis or dropping of eyelid due to paresis of smooth muscle elevator of upper lid.
•Anhidrosis and vasodilatation over the face due to interruption of secretomotor and vasomotor control.
•It indicates the presence of primary disease.
•Exact features depend upon degree of damage of sympathetic pathways to the head and site of this damage.
•Lesions as tumors or infection in brain stem, cervical or high thoracic cord produces this syndrome.
•Trauma,infection or pressure by tumor or aneurysm to preganglionic fibres in anterior spinal roots to sympathetic chain in low cervical and high thoracic area produces the syndrome.
•Involvement of carotid sympathetic plexus by lesions
of gasserian ganglion or aneurysm of internal carotid
artery produces typical facial sweating defect , facial
pain and sensory loss.
MOTOR SYSTEM DISEASE:
Also called as amyotrophies.
It is group of closely related conditions,occurring in 3
forms:progressive muscular atrophy,amyotrophic
lateral sclerosis and progressive bulbar palsy
•Etiology is unknown.
•Called as motor system disease since all forms manifest
corticospinal and anterior horn degeneration with either
bulbar or limb muscle involvement.
Clinical features:
PROGRESSIVE MUSCULAR ATROPHY:
characterized by progressive weakness of limbs with muscular
atrophy,reflex loss and sensory disturbances.
•Shows hereditary pattern.
•Affects male more frequently than female.
•Tend to occur in childhood.
•Initial symptoms are difficulty in walking with leg pain and
paresthesia.
•Ultimately atrophy of foot,leg and hand muscles occurs with
appearance of typical foot-drop,steppage gait and storklegs.
AMYOTROPHIC LATERAL SCLEROSIS:
•Occurs between 40 and 50 years.
•Affects males more.
•Precipitating factors include fatigue , alcohol intoxication ,
trauma and infections as syphilis , influenza , typhus and
epidemic encephalitis
•Initial symptoms consist of weakness , spasticity of limbs ,
difficulty in swallowing , talking with indistinct speech and
hoarseness.
•Atrophy and fasciculation of tongue with impairment or loss
of palatal movements
PROGRESSIVE BULBAR PALSY:
•Characterized by difficulties in swallowing and phonation ,
hoarseness , facial weakness and weakness of mastication
occurs in 5th and 6th decades of life.
•Initial symptoms are gradual in onset and consist of difficulty in
articulation with impairment and finally loss of swallowing,
chewing is difficult as facial muscles become weakened.
Patient show atrophy of face , masseter and temporal muscles and
tongue with fasciculations of face and tongue.
Impairment of palate and vocal cords is also found.
PSEUDOBULBAR PALSY:
It results from loss or disturbance of cortical innervations of bulbar
nuclei.
Usually seen in patients with multiple cerebral thrombi as a
result of cerebral arteriosclerosis.
Patient suffers from cerebrovascular accident with paralysis
of one arm and leg but no swallowing difficulty.
Stroke may result in paralysis of opposite limbs,impairment
of swallowing and talking,loss of emotional control.
Hypertonia and failure of voluntary muscle control is also seen.
Treatment:
No specific treatment.
Disease is fatal,although temporary remissions occur.
PERIODIC PARALYSES:
Called as paramyotonia.
Characterized by episodes of flccid muscle weakness occurring at irregular intervals.
Most conditions are hereditary.
Divided into primary and secondary disorders.
Features include:
-Are hereditary.
-Alteration in serum potassium levels.
-Myotonia co-exists.
-Results due to defective ion channels.
Clinical features:
Manifested by cramping , stiffness , weakness of muscles of
face , neck , fingers and hands upon exposure to cold.
Eyelids are closed and face has mask-like appearance.
Tongue show cramping after drinking cold liquids and speech
becomes slurred.
Muscular cramping disappears within an hour,weakness may
persists for several days.
Histologic features:
No significant changes in muscle fibres.
Treatment:
No specific treatment but prognosis is excellent
MULTIPLE SCLEROSIS:
•Also known as disseminated sclerosis.
•It is an idiopathic inflammatory demyelinating disease of CNS.
Etiology:
•It is an autoimmune process.
•Some believe HHV-6(human herpes virus) while others implicate
Chlamydia pneumonia as causative agent.
•Environmental factors may also be responsible.
Clinical features:
•Frequently seen between 20 to 40 years.
•Female affected more than males in ratio of 2:1.
•Familial incidence is often observed.
•Disease is characterized by:
Ocular disturbances as retrobulbar neuritis,nystagmus and
diplopia.
Fatigability,weakness and stiffness of extremities with ataxia or
gait difficulty involving one or both legs.
Superficial or deep paresthesia.
Personality and mood deviation toward friendliness and
cheerfulness.
Atonomic effector derangements as bladder and rectal retention
or incontinence.
• Charcot’s triad is well-known diagnostic feature
consisting of intention tremor,nystagmus and dysarthria or
scanning speech.
• Facial and jaw weakness ,staccato speech occurs in some
patients.
Treatment:
•No treatment.
•Patient dies due to supervening infection.
MENIERE’S DISEASE:
•Also known as endolymphatic hydrops.
•Is an inner ear(labyrinthine) disorder in which there is
increase in volume and pressure of endolymph of inner ear.
•Patient presents with waxing and waning hearing loss and
tinnitus associated with vertigo.
Etiology
•Current theory states that it is response of inner ear to injury.
•Autopsy have shown an increase in volume of endolymph with
distention of entire endolymphatic system.
•This leads to permanent damage to both vestibular and cochlear
apparatuses.
Clinical features:
•Characterized by deafness,tinnitus and vertigo.
•Begins in middle age.
•Low-pitched tinnitus is described as roar or hum or hissing
sound.
•Deafness described as inner ear deafness of conductive type
which fluctuates in degree.
•Vertigo is late sign,accompanied by nausea,vomiting.
•Vertigo has sudden explosive onset and persists for several
minutes to hours.
•There is dilatation of of endolymphatic spaces of labyrinth
with absence of inflammatory reaction.
•Tinnitus and deafness is unilateral in 90% cases.
•Increased endolymphatic fluid pressure to diminish and
distort response to stimulate.
•Ion of end-organs of lagyrinth and cochlea.
•Basic cause of disorder is either an autonomic vasomotor
dysfunction or intrinsic allergy.
Treatment:
•Some patients react favorably to vasodilators as histamine
or niacin.
•Surgical intervention can be considered to relieve the
vertigo,consisting of section of 8th nerve or destructive
labyrinthotomy.
MARCUS GUNN JAW-WINKING SYNDROME:
•Also called as trigemino-oculomotor synkinesis.
•Consists of congenital unilateral ptosis with rapid elevation of
ptotic eyelid occurring on movement of mandible to
contralateral side.
•Recognized in infants by mother on breastfeeding,when one of
the eyelids shoot up.
•Males affected more.
•Left upper eyelid affected more than right.
•Connection seen between motor branches of trigeminal nerve
innervating external pterygoid muscle and fibres of superior
division of oculomotor nerve that innervate the levator superior
muscle of upper eyelid.
•Some cases are hereditary and others follow later In life due to
injury or disease.
•Condition MARIN AMAT SYNDROME OR MARCUS
PHENOMENON ,consist of eye closure when patients opens his
mouth forcefully in chewing,tears may flow,usually seen after
peripheral facial paralysis.