Cranial Neuropathies
Cranial nerve examination |
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|
Nerve |
innervation |
Symptoms
in the lesion |
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olfactory |
Smell |
Anosmia, hyposmia |
|
Optical |
Seeing |
visual field defects; loss of direct,
indirect pupillary reflex |
|
oculomotor |
M. rectus sup., inf., med., inf.
Oblique and constrictor pupil |
Eye cannot look up inward, limitation
of looking down, loss of indirect light reflex, diplopia |
|
trochlear |
superior oblique muscle |
Limitation of looking down, head
tilts to the opposite side of the lesion |
|
trigeminus |
All the senses of the face, chewing
muscles |
Hypothesis anesthesia, loss of
corneal reflex, shifts to the lesion side when the jaw is opened |
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abdusens |
M. rectus lateralis |
Can't look outward, diplopia becomes
internal strabismus |
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fascialis |
mimic muscles; 2/3 foretaste of the
tongue; sublingual submandibular, lacrimal glands |
Facial paralysis, loss of 2/3 foretaste
of the tongue, cessation of tear secretion |
|
Vestibulo cochlear |
Hearing, balance |
Hearing loss, tinnitus, vertigo,
nystagmus, balance disorder |
|
Glossopharyngeus |
Muscles of the pharynx 1/3 posterior
taste and touch of the tongue and tactile sensation of the pharynx-tonsils |
Loss of gag reflex, 1/3 posterior
taste of tongue, loss of tactile sensation, loss of tactile sensation of
pharynx and tonsils |
|
vagus |
Muscles of the larynx, viscera and
auditory canal sense, parasympathetic innervation of organs up to the middle
of the transverse colon |
Nasal speech, nasal regurgitation,
vocal cord paralysis, palatal arch asymmetry |
|
accessory |
M. trapezius and Accessorius
sternocleidomostaideus |
Can't turn head to the healthy side,
can't raise the shoulder on the lesion side |
|
hypoglossus |
tongue muscles |
In peripheral lesions: When the
tongue is brought out, it shifts to the side of the lesion. Centrally shifts
to the opposite side of the lesion |
Approach to the Hypoglossus Paralyzed Patient
- Presence of central paralysis (supranuclear lesion):
Tongue turns to opposite side of lesion
Atrophy, no fasciculation
- Peripheral (intra and infranuclear) paralysis:
Tongue deviates to the lesion side
There is atrophy and fasciculation
In a patient with suspected hypoglossal palsy
The first thing to look for is atrophy + presence of fasciculation, peripheral lesion
Central lesion if there is no atrophy, fasciculation
It is examined under three groups:
1) Axonal polyndropathy. If it causes axon damage
2) Neuropathy. If it affects the neuron body
3) Myelinopathy I · If the myelin sheath is affecting
Polyneuropathy is a clinical picture that occurs when peripheral nerves are commonly infected together due to the same cause.
In the Patient with Polyneuropathy
It progresses with the damage of peripheral nerve axons starting from distal and progressing to proximal. Sensory symptoms consist of paresthesia and pain starting in the distal lower extremities. In neurological examination, sensory defect in the form of gloves and socks is detected in the distal extremities.
Diagnosis is made by EMG.
In mononeuropathy multiplex, the same disease process affects the peripheral nerves as multiple foci, usually located at separate times.
The most typical examples are PAN and Churg-Strauss allergic granulomatosis. It is seen in diseases affecting the Vaso Nervorum.
Causes of mononeuropathy multiplex
Polyarteritis nodosa
Wegener's granulomatosis
Diabetes
Cryoglobulinemia
Sarcoidosis
Lyme disease
HIV
Leprosy
Systemic lupus erythematosus
Rheumatoid arthritis
TB meningitis does not cause mononeuropathy multiplex
Etiology
I. Acquired
a. metabolic disorders
Diabetes mellitus
Neuropathies due to kidney disease
Vitamin deficiencies
b. due to immune disorder
Guillain-Barré syndrome (GBS)
Chronic inflammatory demyelinating polyneuropathy (CIDP) and its variants
Vasculitis
c. due to infection
Herpes zoster
Associated with Leprosy, Lyme, HIV and sarcoidosis
D. Associated with cancer and lymphoproliferative diseases
Associated with lymphoma, myeloma and cancer
Primary amyloidosis
to. drugs or toxins
Chemotherapy dependent
Heavy metals and industrial toxins
II. Hereditary
Hereditary motor-sensory neuropathy (Charcot-Marie-Tooth disease)
Hereditary pressure sensitivity neuropathy
Familial brachial plexopathy
Familial amyloidosis
Porphyria
Other rare peripheral neuropathies (Fabry disease, metachromatic leukodystrophy, adrenoleukodystrophy, Refsum's disease)
Motor neuron disease
Spinal muscular atrophy
Familial amyotrophic lateral sclerosis
a. Charcot-Marie-Tooth Disease
It is the most common hereditary neuropathy.
Pes cavus and hammer toe deformities are seen in the feet.
Distal weakness and atrophy in the lower extremities dominate the clinic.
b. Dejerine-Sottas disease
In childhood characterized by histopathologically demyelination
It is a sensory-motor neuropathy that causes motor developmental delay.
They have very low nerve conduction velocities.
There are palpable hypertrophic nerves
c. Familial pressure sensitivity neuropathy (tomacular neuropathy)
It is autosomal dominant. It is characterized by transient painless motor-sensory deficits. Focal nerve thickening is seen on biopsy. This look is called tomakula - sausage.
While a homogeneous slowdown in nerve conduction is observed in hereditary polyneuropathies, nerve conduction velocities in acquired polyneuropathies (such as GBS, CIDP) vary significantly between different nerves in the same extremity segment, different segments of the same nerve, and nerves of the same name of the two opposite extremities.
III. due to immune disorder
Guillain-Barré syndrome (GBS)
• History of upper respiratory tract or gastrointestinal tract infection (C. jejuni-fetus)
• There may be a history of surgery or vaccination.
• Sensory symptoms predominate.
• In the following periods, symmetrical paralysis starts distally and progresses upwards.
In severe cases, respiratory and bulbar muscles are involved.
Typical features:
1. acute
2. Inflammatory
3. Demyelinating
4. Segmental
5 Peripheral nerve involvement
• Mechanical ventilation may be required in 1/3 cases.
• If autonomic dysfunction occurs, tachycardia, urinary retention and blood pressure fluctuations occur.
• The mortality of the disease is 10%.
• Increased protein in CSF in most cases; however, it may be normal up to 2-3 weeks after the onset of the disease.
• CSF gammaglobulin fraction increases.
• In the following period, sensory complaints are more in the background and paresthesia at the extremities, back, waist and leg pains.
• Despite the increase in protein in CSF, no cells are seen (albuminocytological dissociation).
• Cranial nerve involvement may be seen.
• The most common cranial double involvement is facial paralysis, which can be bilateral, accompanying 50% of the cases.
• Deep tendon reflexes are typically absent.
• The main treatment is supportive treatment.
• The benefit of steroids has not been demonstrated.
• Plasmapheresis or intravenous human IgG (IVIG) accelerates recovery.
Miller-Fisher syndrome:
It is the form of Guillain-Barre syndrome accompanied by ophthalmoplegia, areflexia and ataxia.
Diagnosis: Anomaly in nerve conduction velocities, EMG findings, albuminocytological dissociation, and motor nerve block are helpful.
Nerve biopsy has no place in the diagnosis.
Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)
• As in GBS, it is a disease that develops with an autoimmune mechanism and progresses with multifocal demyelination in peripheral nerves.
• Generally, there is muscle weakness involving the distal and proximal extremities quite symmetrically.
• Unlike GBS, muscle weakness progresses slowly, gradually, or with remissions and exacerbations over months.
• Despite the increase in protein in CSF, no cells are seen.
• Shows that corticosteroids (unlike GBS), IVIG and plasmapheresis are effective in the treatment of CIDP
IV. Linked to Metabolic Disorders
diabetic neuropathy
• It is a mixed type polyneuropathy that includes motor, sensory and autonomic dysfunction.
• Pain and paresthesias are common in 40% of those with long-term diabetes.
a) Diabetic polyneuropathy: Sensory symptoms are evident. Distal weakness may also occur. Paresthesias begin in the feet. Achilles reflex is absent. It is the most common form of diabetic neuropathy.
b) Autonomic neuropathy: Impotence, nocturnal diarrhea, postural hypotension and excessive sweating occur.
c) Diabetic mononeuropathy
d) Diabetic amyotrophy: It is characterized by proximal paresis, nocturnal pain and fasciculations in the thigh, perineum, hip and back. Sensory loss is minimal.
In the course of diabetes, the most common cranial nerve: 3rd nerve is involved.
The pupillary light reflex is not affected in diabetic third nerve involvement, while it is affected in aneurysm.
The most common site of involvement of diabetic peripheral polyneuropathy is the femoral nerve.
Thoracic Outlet Syndrome
0 The lower trunk of the brachial plexus may be under pressure for reasons such as arteria and vena subclavia, cervical rib in the neck, fibrous bands, cervical muscle hypertrophy.
0 In 30% of cases, sensory loss, muscle weakness, atrophy and signs of vascular compression are observed.
0 Diagnosis is made by the disappearance of the radial pulse when the arm is placed in hyperabduction.
0 This is called the Adson test. It can be seen in the following situations. (Servico-thoracic syndromes)
1. Hyperabduction syndrome
2. Scalenus anterior syndrome
3. Costoclavicular syndrome
4. Subclavian-axillary vein thrombosis
Carpal Tunnel Syndrome
0 Etiological factors include increased connective tissue, rheumatoid arthritis, acromegaly, hypothyroidism, ligament infiltration, amyloid, fluid retention, and excessive weight loss.
0 In the clinic, there is pain and paresthesias that increase at night in the median nerve distribution.
0 Sensory loss and thenar atrophy are seen.
0 Local hydrocortisone, diuretic and decompression surgeries are used in the treatment.
0 Tinel, Phalen, and reverse phalen test are used for diagnosis.