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25 Cards in this Set

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Grisel Syndrome
- subluxation of the atlantoaxial joint
- associated with overdistention of the atlantoaxial joint ligaments by effusion, rupture of the transverse ligament, excessive passive rotation during general anesthesia
- features: spontaneous torticollis in a child, a flexed and rotated head with limited range of motion, flat face, Sudeck sign (displacement of the spine of the axis to the same side as the head is turned
- treatment: skeletal skull traction under fuoroscopic control to realign the odontiod process within the transverse ligament sling, followed by 6-12 wks of immobilization
Hanhart syndrome
-Bird like profile of face with micrognathia
-peromelia (severely deformed limbs)
-small growth
-normal intelligence
-branchial arch deformities resulting in CHL
-tongue deformities
-carefully consider ear surgery due to abnormal course of facial nerve
Hollander syndrome
- appearance of goiter during 3rd decade of life related to partial defect in the coupling mechanism in thyroxine biosynthesis
- deafness due to cochlear abnormalities related to this
Hunt Syndrome
- cerebellar tumor, an intention tremor that begins in one extremity gradually increasing in intensity and then involve other parts of body
- facial paralysis, otalgia, and aural herpes due to disease of both motor and sensory fibers of CN7
- a form of juvenile paralysis agitans associated with primary atrophy of the pallidal system
Hunter syndrome
- hereditary and sex-linked, incurable syndrome involves multiple organ systems through mucopolysaccharide infiltration
- death by 2nd decade, often by an infiltrative cardiomyopathy and valvular dx leading to heart failure
- prominent supraorbital ridges, large flattened nose with flared nares, low-set ears, progressive corneal opacities, generous jowls, patulous lips and prognathism, short neck, hirsutism, short stature, extensive arthritis, TMJ arthritis, pseudopapilledema, and low pressure hydrocephalus
Jackson Syndrome
- CN 10, 11, 12 are affected by nuclear or radicular lesion
- ipsilateral flaccid paralysis of the palate, pharynx, and larynx with weakness and atrophy of the SCM and trapezius muscle and muscles of the tongues
Jacod Syndrome
- total ophthalmoplegia
- optic tract lesion with unilateral amaurosis
- trigeminal neuralgia
- caused by middle cranial fossa tumor involving the 2nd through 6th cranial nerves
Job Syndrome
- one of the group of hyperimmunoglobulin E syndromes that are associated with defective chemotaxis
- fair skin, red hair, recurrent staphylococcal skin abscesses with concurrent other bacterial infections and skin lesions, as well as chronic purulent pulmonary infections and infected eczematoid skin lesions
- name obtained from Biblical passage referring to Job being smitten with boils
Jugular Foramen (Vernet)
- CN 9, 10, 11 are paralyzed
- CN 12 is spared because it's in hypoglossal canal
- Horner syndrome is not present
- Commonly caused by lymphadenopathy of the nodes of Krause in the foramen
Kimura Disease
- chronic inflammatory condition occurring in subcutaneous tissues, salivary glands, and lymph nodes
- histo shows dense fibrosis, lymphoid infiltration, vascular proliferation, and eosinophils
- lab shows eosinophilia, and elevated IgE
- treat with steroids, cryotherapy, radiation and surgery
Maffuci
- multiple cutaneous hemangiomas with dyschondroplasia and often enchondroma
-dyschondroplasia may cause sharp bowing or an uneven growth of the extremities as well as frequent fractures
-head and neck involvement (10%) include CN dysfunction and nasopharyngeal and laryngeal hemangiomas
-can have dysphagia and airway problems
Mal de Debarquement
-an imbalance or rocking sensation that occurs after prolonged exposure to motion
- may experience up to months after disembarking
-imbalance is generally not associated with any nausea nor is it alleviated by typical motion sickness drugs
-symptoms most pronounced when the patient is sitting still, minimized by walking or driving
Shy-Drager
- present at late middle age
- a form of neurogenic orthostatic hypotension that results in failure of the autonomic nervous system and signs of multiple system atrophy affecting corticospinal and cerebellar pathways and basal ganglia
- symptoms include postural hypotension, impotence, sphincter dysfunction, and anhidrosis with later progression to panautonomic failure
- followed by atypical parkinsonism, cerebellar dysfunction with debilitation or both then death
- Should consider Shy-Drager when patient displays orthostatic hypotension, laryngeal stridor, restriction in range of vocal cord abduction, dysphonia, intermittent diplophonia, and slow speech rate
Tolosa-Hunt
-cranial polyneuropathy presenting as recurrent unilateral painful ophthalmoplegia
-CN 2, 3, 4, V1, and 6 may be involved
-tendency for spontaneous resolution and for recurrence
- orbital venogram may show occlusion of the superior ophthalmic vein and at least partial obliteration of the cavernous sinus
- treat with steroids
Villaret Syndrome
- Jugular foramen syndrome (Vernet) plus Horner syndrome
- more extensive involvement in the region of the jugular foramen, retroparotid area, and the lateral pharyngeal space
Wallenberg Syndrome (lateral medullary syndrome)
- the posterior-inferior cerebellar artery thrombosis or lateral medullary syndrome
- results in ischemia of the brain stem/lateral medullary region
- vertigo, nystagmus, n/v, Horner's syndrome, dysphagia, dysphonia, hypotonia, asthenia, ataxia, falling to the side of the lesion, and loss of pain and temperature sense on the ipsilateral face and contralateral side below the neck
Wildervanck (Cervico-Oculo-Acoustic) Syndrome
- mixed hearing loss
- Klippel-Feil anomaly (fused cervical vertebrae)
- bilateral abducens palsy with retracted bulb
- 75:1 ratio female:male
Griesinger Sign
- Edema of the tip of the mastoid in thrombosis of the sigmoid sinus
Guttman test
- In the normal subject, frontal pressure on the thyroid cartilage lowers the tone of the voice; whereas lateral pressure produces a higher tone
- opposite is true for paralysis of the CT muscle
Lhermitte Sign
- rare complication of XRT to the head and neck region cause damage to the cervical spinal cord
- sxs consist of lightning-like electrical sensation spreading to both arms, down the dorsal spine, and to both legs upon neck flexion
- (L)hermitte = (L)ightning-like electrical sensation
Mikulicz Cells and Russel Bodies
- (M)ikulicz cells are (M)acrophages in rhinoscleroma
- (R)ussell bodies are eosinophilic round structures associated with plasma cells found in (r)hinoscleroma
Does vestibular supressant improve vestibular neuronitis
yes along with physical therapy and vestibular exercises
What are the 4 classes of medication to treat vertigo?
1. Anticholinergic
2. Monoaminergic
3. Antihistiminic
4. Antidopaminergic
What's the MOA of anticholinergics and monoaminergics on vestibular nerve
They both diminish the excitability of the neurons in the vestibular nucleus
What's the MOA of antihistamines on vestibular nerve
- They have significant antimuscarinic cholinergic activity in addition to antagonism of histamine H1 receptors.
- Histamine excites neurons in the vestibular nuclei
- there is also histaminergic innervation in the brainstem autonomic regions and the parabranchial nucleus, the antimotion sickness efficacy of these drugs may reflect actions at multiple levels in the vestibuloautonomic pathways