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DISEASES OF NERVES
Q
Trigeminal neuralgia
(Synonyms: Fothergills disease; Tic douloureux)
Neuralgia is defined as the pain along the pathway of the nerve. The term
trigeminal neuralgia means pain along the distribution of the trigeminal
nerve.
It is described as a sudden, sharp, lancinating, electric shock like, recurrent
pain in the distribution of one or more branches of the trigeminal nerve.
Trigeminal neuralgia (TN) is the most frequently occurring nerve pain
disorder. It has one of the highest suicidal rates and is often described as the
most terrible pain known to humans.
TN was first described by John Fothergill in 1773.
TN is also called tic douloureux because of the characteristic muscle spasm
that typically accompanies the pain attack.
Types
The International Headache Society (IHS) has identified two types of TN:
1. Classical TN
2. Symptomatic TN
Pathogenesis
Classical TN
It is also known as idiopathic TN, typically caused by a blood vessel (usually
the superior cerebellar artery) compressing the trigeminal nerve as it exits the
brainstem. The constant compression leads to the loss of myelin sheath, the
covering which surrounds the nerve fibres. The loss of the protective nerve
covering is called demyelination. Without proper insulation, the nerve cells
become hyperexcitable and begin to fire in an erratic and disorganized
manner resulting in significant pain. This is believed to be the cause of 90%
cases of TN.
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Symptomatic TN
It includes all cases of TN secondary to an underlying, demonstrable medical
condition not related to vascular compression. The causes could be:
Multiple sclerosis: An autoimmune disease caused by demyelination of nerve
fibres in brain and spinal cord. Approximately, 35% of patients with TN
suffer from multiple sclerosis. The patients are usually younger and have
bilateral presentation.
Other diseases which cause damage to myelin sheath like leukodystrophy,
chronic inflammatory demyelinating disease, Guillan-Barre syndrome, etc.
A tumour compressing the trigeminal nerve.
Abnormalities at the base of the skull.
Arteriovenous malformations.
Approximately, 510% cases of TN are caused by any of these medical
conditions.
Clinical features
Age. 5070 years.
Gender. Female predilection.
Location. The maxillary and mandibular branches are more commonly
affected. The pain may be felt in the ear, eye, lips, nose, scalp, forehead,
cheeks, teeth, or jaw and side of the face.
Signs and symptoms
Solitary involvement of ophthalmic branch is seen in less than 5% of
cases.
Pain is usually unilateral, affects right side more than left side.
Triggers: Many patients describe a specific event (trigger) that may
precipitate an attack. The triggers include chewing, talking, swallowing,
brushing teeth, face washing, shaving, cool breeze across the face, light
touch or vibration.
Trigger zones: The pain attack is initiated by touching specific areas on the
face. These are known as trigger zones and are present on ala of the nose,
cheeks, vermilion of the lips, around the eyes, skin on chin, forehead,
midface, alveolar bone, etc.
Diagnosis
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Differential diagnosis
Otitis media
Sinusitis
Post-herpetic neuralgia
Vagal and superior laryngeal neuralgia
Prognosis and predictive factors
Early diagnosis and appropriate treatment helps in controlling the progression
of the disease as well as lesser side effects due to less dosage of medication.
Medical
Anticonvulsants
First-line drugcarbamazepine
Second-line drugsoxycarbazepine, gabapentin, lamotrigine, phenytoin
Surgical
Microvascular decompression
Radiofrequency thermocoagulation
Retrogasserian glycerol injections
TN is not a life-threatening illness. Some patients experience a remission of
pain lasting many years, while for others, the symptoms intensify. Initially,
most patients respond to medication, but over time, medication become less
effective. When the pain becomes intractable or interferes significantly with
daily activity, surgical options can be explored.
Q
Glossopharyngeal neuralgia
Glossopharyngeal neuralgia (GPN) is characterized by electric shock like pain
in the area of distribution of the ninth cranial nerve, the glossopharyngeal
nerve.
It is in every way similar to trigeminal neuralgia except for the distribution
of the pain and the customary site of the triggering stimulus.
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Pathogenesis
The vast majority of patients with GPN are thought to have an artery
compressing the nerve as it exits from the medulla and travels through the
subarachnoid space to the jugular foramen.
Clinical features
Excruciating pain in the region of the ear, throat, tonsillar fossa, pharynx or
base of the tongue.
It can radiate to the ear or the angle of the jaw or into the upper lateral neck.
The pain occurs in episodes and may be severe. It is usually on one side, and
feels jabbing. The episodes can occur many times each day, and awaken the
person from sleep.
The trigger zone is often in the same area, and patients frequently report that
swallowing, yawning, chewing, coughing, laughing, speaking or clearing the
throat could be the precipitating stimulus.
Often the pain appears to be spontaneous. Chewing or touching the face does
not precipitate an attack.
Diagnosis
The nature of the pain, its description by the patient and the chronology of
the attacks are identical to those of tic douloureux of the trigeminal nerve.
The diagnosis can be confirmed by the cessation of pain when this nerve is
blocked at the jugular foramen or when topical anaesthesia of the pharynx
stops the pain.
Prognosis and predictive factors
The pharmacologic management is the same as that for tic douloureux of the
trigeminal nerve.
When medical management fails, suboccipital craniectomy with exploration
of the glossopharyngeal nerve is indicated.
Q
Bells palsy
(Synonym: Idiopathic facial paralysis)
Bells palsy is named after Sir Charles Bell, a nineteenth century Scottish
anatomist who was the first to describe the condition in detail.
Bells palsy is defined as an idiopathic unilateral facial nerve paralysis, usually
self-limiting. The hallmark of this condition is a rapid onset of partial or
complete paralysis that often occurs overnight. In rare cases (1%), it can
occur bilaterally resulting in total facial paralysis.
Pathogenesis
Several conditions cause paralysis of the facial nerve, e.g. Ramsay Hunt
syndrome, brain tumour, stroke, acute and chronic otitis media, Lyme disease,
neurosarcoidosis, temporal bone fractures, Moebius syndrome, tumours
compressing the facial nerve like parotid gland neoplasms, haemangiomas, etc.
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Recent studies suggest that Bells palsy could be a polyneuritis with possible
viral, inflammatory, autoimmune, and ischaemic aetiologies. Increasing
evidence implicates herpes simplex virus type 1 (HSV-1) and herpes zoster
virus reactivation from cranial nerve ganglia.
The various risk factors suggested for reactivating the virus are:
Exposure to cold
Sudden changes in atmospheric pressure
Emotional stress
Trauma
Metabolic disorders
The exact pathophysiology is still unknown.
It is thought that as a result of inflammation and oedema of the facial nerve,
pressure is produced on the nerve where it exits the skull within its bony
canal, blocking the transmission of neural signals or damaging the nerve and
the nerve conductive properties are impaired by the inflammatory, ischaemic
and demyelinating processes.
A recent study proposed a hypothesis that the HSV-1 infection is associated
with demyelination of nerves. Demyelination may not be directly caused by
the virus, but an unknown immune system causes virus reactivation. It stated
that HSV-1 replication itself is not responsible for the damage to the facial
nerves because treatment with acyclovir does not prevent the progression of
nerve dysfunction.
Clinical features
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Diagnosis
Bells palsy is a diagnosis of exclusion, by elimination of other reasonable
possibilities.
The diagnosis is made based on the history, clinical presentation and a
thorough physical examination to examine upper and lower facial weakness.
There is no specific laboratory test to confirm diagnosis of the disorder.
Electromyography (EMG) is used to confirm the presence of nerve damage
and determine the severity and the extent of nerve involvement.
Prognosis and predictive factors
Some cases are mild and do not require treatment as the symptoms usually
subside on their own within 2 weeks.
Medical treatment includes corticosteroids like prednisone with potent antiinflammatory function. Analgesics such as aspirin, acetaminophen or
ibuprofen may relieve pain.
Eye protectionlubricating eyedrops, such as artificial tears or eye ointments
or gels and eye patches are effective.
Physiotherapy to stimulate the facial nerve and help maintain muscle tone
may be beneficial to some individuals.
Other therapies that may be useful for some individuals include relaxation
techniques, acupuncture, electrical stimulation, biofeedback training and
vitamin therapy (including vitamin B12, B6 and zinc), which may help restore
nerve function.
The prognosis for individuals with Bells palsy is generally very good. The
extent of nerve damage determines the extent of recovery. Improvement is
gradual and recovery times vary.
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Clinical features
Age. Older age group.
Sex. Females predilection.
Signs and symptoms
Moderate to severe burning in the mouth is the main symptom of BMS and
can persist for months or years. For many people, the burning sensation
begins in late morning, builds to a peak by evening, and often subsides at
night.
Pain may be consistent or intermittent.
Glossodynia (painful tongue) and glossopyrosis (burning tongue).
Tingling or numbness on the tip of the tongue or in the mouth.
Bitter or metallic changes in taste.
Dry or sore mouth.
Prognosis and predictive factors
Depending on the cause and symptoms, possible treatments include:
Adjusting or replacing irritating dentures.
Treating existing disorders such as diabetes, Sjgrens syndrome or a thyroid
problem to improve burning mouth symptoms.
Supplements for nutritional deficiencies.
Prescribing medications to:
Relieve dry mouth.
Treat oral candidiasis.
Help control pain from nerve damage.
Relieve anxiety and depression.
When no underlying cause can be found, treatment is aimed at the symptoms
to try to reduce the pain associated with BMS.
Q
Auriculotemporal syndrome
(Synonyms: Freys syndrome; FreyBaillarger syndrome; gustatory sweating
syndrome)
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Pathogenesis
The injury to the auriculotemporal nerve may be from any of the following
causes:
Penetrating wound of the parotid region
Infection of the parotid region
Parotidectomy
Birth traumatrauma of the auriculotemporal nerve after forceps delivery
Mandibular and zygomatic fractures
Temporomandibular joint (TMJ) surgery
Internal maxillary artery pseudoaneurysm
Diabetes mellitus
Herpes zoster
Platinum-induced neuropathy
CNS diseases (syringomyelia, cerebrovascular accident, encephalitis)
The auriculotemporal branch of the trigeminal nerve carries sympathetic
fibres to the subcutaneous sweat glands and parasympathetic fibres to the
parotid gland.
FS is believed to be the result of misdirected autonomic nerve regeneration
following injury to the parotid region. After injury, the sectioned postganglionic secretomotor parasympathetic fibres which normally innervate the
parotid gland, regenerate and get misdirected and get connected to sympathetic receptors which innervate sweat glands and subcutaneous blood capillaries. Hence, stimuli that normally cause salivation (smell, sight and thought
of food) simultaneously cause pathologic vasodilatation, sweating and flushing in the preauricular area on the side of the nerve injury (Fig. 19.1).
Sweat glands
Sympathetic
Sensory
Parasympathetic
Normal
Parotid
Sweat glands
Sympathetic
Parasympathetic
Mixed
Perotid
bed
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Clinical features
Severity of symptoms varies from person to person. Few of them may have
slight localized increase of temperature and erythema of preauricular skin
with gustatory stimuli, whereas some suffer from profuse sweating and facial
flushing.
Although FS does not cause significant physiological harm, profuse gustatory
flushing and sweating can cause social embarrassment and psychological
distress in some patients.
Diagnosis
In most cases, FS patients do not complain of their symptoms and are often
treated effectively with topical antiperspirant gels applied to the affected area.
However, when the symptoms become annoying, various prophylactic and
therapeutic surgical strategies have been proposed to minimize the incidence
or severity of FS following parotidectomy.
Orolingual paraesthesia
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Clinical features
Damage to the lingual nerve results in pain within the tongue and inner
buccal mucosa.
The patients suffer from burning or dull or achy pain or numbness could be
the only symptom.
In general, the inferior alveolar nerve injuries (mucosa and lip numbness) are
tolerated well than the lingual nerve injuries (affect tongue and inner gingival
mucosa).
Prognosis and predictive factors
Most of the injuries resolve on their own.
Short course of corticosteroids, if the injury is recent.
Palliative treatment for nerve paintopical lidocaine, capsaicin candies, systemic analgesics, acupuncture, nerve block and neurectomy in severe cases.
Q
The term atypical facial pain (AFP) is used to describe the facial pain for which
no cause can be found and which does not respond to the usual analgesics.
Pathogenesis
The exact aetiology is still unknown and many possible causes are proposed:
Some studies postulate a low-grade infectious and inflammatory process
occurring over a long period can result in nerve damage and could be the
triggering factor for AFP pain.
Some believe that vascular compression of the trigeminal nerve in the same
area that is postulated to lead to trigeminal neuralgia is a cause of AFP.
Dental or some sort of physical trauma is also linked to AFP.
Clinical features
Age. Older age group.
Sex. Female predilection.
Signs and symptoms
The pain can be intermittent or continuous of varying intensity and can
last for years.
It may affect a small area of the face, but it can also spread across the
whole of the face and mouth.
The pain is described as nagging, throbbing, burning, pinching, pulling,
aching and is unilateral, often in the region of the trigeminal nerve and can
extend up to the upper neck or back of the scalp.
Clinical presentation
Symptoms
Site
Pain in the region of the ear may be referred from the skin, teeth,
tonsils, pharynx, larynx or neck.
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DISEASES OF MUSCLE
Q
Muscular dystrophy
Muscular dystrophy (MD) refers to a group of more than 30 genetic diseases
that cause progressive weakness and degeneration of skeletal muscles used
during voluntary movement. The word dystrophy is derived from the Greek
word dys, which means difficult or faulty and troph means nourish.
These disorders vary in age of onset, severity and pattern of affected muscles.
All forms of MD grow worse as muscles progressively degenerate and
weaken. Many patients eventually lose the ability to walk.
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Oral manifestations
In cases of mild MD, inability to close the eyes, facial muscle weakness,
weak lips, inability to whistle or smile.
In cases of severe MD, the muscles of mastication are affected along with
all other groups of muscles. The teeth are not properly aligned in the arch
due to lack of muscle tension.
Histopathology
There is gradual disappearance of muscle fibres as the disease progresses.
Prognosis and predictive factors
The prognosis varies according to the type of MD and the speed of progression.
Some types are mild and progress very slowly, allowing normal life expectancy,
while others are more severe and result in functional disability and loss of
ambulation. Life expectancy may depend on the degree of muscle weakness and
any respiratory and cardiac complications.
Q
Myotonias
Myotonias are a group of neuromuscular disorders characterized by slow
relaxation of muscles after voluntary contraction.
Repeated effort is needed to relax the muscles, and the condition improves
after the muscles have warmed up.
However, prolonged, rigorous exercise may also trigger the condition.
Individuals with the disorder may have trouble releasing their grip on objects
or may have difficulty rising from a sitting position and a stiff, awkward gait.
Myotonia can affect all muscle groups; however, the pattern of affected
muscles can vary depending on the specific disorder involved.
Patients suffering from disorders involving myotonia can have a life-threatening reaction to certain anaesthetics; one of these conditions occurs when the
patient is under anaesthesia and is termed malignant hyperthermia.
Myotonia is not always a disease-related phenomenon. Humans often display
myotonia, when placed in situations of extreme stress or fear; a resultant
increase in fight-or-flight hormones such as epinephrine and cortisol may
cause increased muscle tension throughout the body.
Pathogenesis
It may be acquired or inherited, and is caused by an abnormality in the
muscle membranespecifically, the ion channels that control the contraction
of muscle fibres.
Myosin is defective in myotonia.
Clinical features
The main types of myotonias are dystrophic, acquired and congenital.
Dystrophic myotonia can be encountered by the dentist and is characterized
by ptosis of eyelids, atrophy of masseter and sternocleidomastoid muscles.
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Lower part of the face becomes narrow due to muscular weakness and
atrophy and is referred to as myopathic face and swan neck.
Myositis/myositis ossificans/dermatomyositis
The term myositis ossificans refers to the formation of bone within a muscle.
It is a disorder of unknown cause in which connective tissue and muscle are
replaced by bone.
It is of two types:
In the more common local type (myositis ossificans circumscripta), only
one area is affected and ossification occurs in a muscle following injury.
This condition is also known as traumatic myositis ossificans. It needs
immediate treatment and has a good prognosis.
The rare progressive type (myositis ossificans progressiva) is characterized
by ossifications in muscles and connective tissue fascia without any injury,
group after group of muscles become ossified in a predictable manner, until
the individual is completely rigid. This is known as Stiffman syndrome and
is inherited in an autosomal dominant pattern. Breathing and swallowing
become difficult, and fatal respiratory infections may occur. The survival
rate is usually low and the use of medications to prevent calcification may
slow the progression of the disorder.
Oral manifestations
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Histopathology
Q
Myasthenia gravis
Myasthenia gravis (MG) is a chronic neuromuscular disease characterized by
fluctuating muscle weakness and fatigability.
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Dental considerations
The facial muscles are commonly involved, giving the patient an immobile
and expressionless appearance. Patients with MG cannot wear complete
dentures because of the weak muscles; they have a hard time chewing their
food, keeping their mouths closed; when they eat, have a weakened tongue
and a weakened palate.
This disease commonly affects middle-aged females and the patients often
exhibit difficulty in mastication, deglutition, dropping of jaw, slurred speech
and loss of taste sensation.
Dental management of patients diagnosed with MG presents a challenge to
the dental profession. Multiple, short early morning appointments should be
scheduled and oral anticholinesterase agents should be administered 1 hour
before dental treatment to achieve maximal effect during the dental
procedures.
Treatment
Muscular hypertrophy
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KEY POINTS
Trigeminal neuralgia means pain along the distribution of trigeminal nerve. It is a sudden, sharp,
lancinating, electric shock like, recurrent pain in the distribution of one or more branches of the
trigeminal nerve.
Bells palsy: Dropping of angle of mouth on affected side, inability to raise eyebrow on affected side,
inability to close eye on affected side, excessive tearing or dry eye, Bells phenomenon and
expressionless face.
Freys syndrome is the phenomenon secondary to gustatory stimulus, manifested by flushing and
sweating in the preauricular region consequent to injury of auriculotemporal nerve.
Atypical facial pain is a facial pain which does not have any significant cause and does not respond to
the usual analgesics.
Muscular dystrophy refers to a group of genetic diseases that cause progressive weakness and
degeneration of skeletal muscles used during voluntary movement.
Myotonias are a group of neuromuscular disorders characterized by slow relaxation of muscles after
voluntary contraction.
Myositis refers to inflammation of muscle tissues and could be caused by physical and chemical injuries
to the muscles as well as infections.
Myasthenia gravis is a chronic neuromuscular disease characterized by fluctuating muscle weakness
and fatigueability.
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