Ent Board Review - Kj Lee Chapter 9 Syndromes

Front 1

Grisel Syndrome

Back 1

- 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

Front 2

Hanhart syndrome

Back 2

-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

Front 3

Hollander syndrome

Back 3

- 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

Front 4

Hunt Syndrome

Back 4

- 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

Front 5

Hunter syndrome

Back 5

- 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

Front 6

Jackson Syndrome

Back 6

- 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

Front 7

Jacod Syndrome

Back 7

- total ophthalmoplegia
- optic tract lesion with unilateral amaurosis
- trigeminal neuralgia
- caused by middle cranial fossa tumor involving the 2nd through 6th cranial nerves

Front 8

Job Syndrome

Back 8

- 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

Front 9

Jugular Foramen (Vernet)

Back 9

- 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

Front 10

Kimura Disease

Back 10

- 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

Front 11

Maffuci

Back 11

- 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

Front 12

Mal de Debarquement

Back 12

-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

Front 13

Shy-Drager

Back 13

- 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

Front 14

Tolosa-Hunt

Back 14

-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

Front 15

Villaret Syndrome

Back 15

- Jugular foramen syndrome (Vernet) plus Horner syndrome
- more extensive involvement in the region of the jugular foramen, retroparotid area, and the lateral pharyngeal space

Front 16

Wallenberg Syndrome (lateral medullary syndrome)

Back 16

- 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

Front 17

Wildervanck (Cervico-Oculo-Acoustic) Syndrome

Back 17

- mixed hearing loss
- Klippel-Feil anomaly (fused cervical vertebrae)
- bilateral abducens palsy with retracted bulb
- 75:1 ratio female:male

Front 18

Griesinger Sign

Back 18

- Edema of the tip of the mastoid in thrombosis of the sigmoid sinus

Front 19

Guttman test

Back 19

- 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

Front 20

Lhermitte Sign

Back 20

- 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

Front 21

Mikulicz Cells and Russel Bodies

Back 21

- (M)ikulicz cells are (M)acrophages in rhinoscleroma
- (R)ussell bodies are eosinophilic round structures associated with plasma cells found in (r)hinoscleroma

Front 22

Does vestibular supressant improve vestibular neuronitis

Back 22

yes along with physical therapy and vestibular exercises

Front 23

What are the 4 classes of medication to treat vertigo?

Back 23

1. Anticholinergic
2. Monoaminergic
3. Antihistiminic
4. Antidopaminergic

Front 24

What's the MOA of anticholinergics and monoaminergics on vestibular nerve

Back 24

They both diminish the excitability of the neurons in the vestibular nucleus

Front 25

What's the MOA of antihistamines on vestibular nerve

Back 25

- 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