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EPONYM CLINICAL SYMPTOMS ETIOLOGYa
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Anterior part of the base of the skull
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Olfactory disturbances (uni- or bilateral anosmia), possibly psychiatric disturbances, seizures
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580 Rochon-Duvigneau; syndrome of the pterygopalatine fossa (Behr) and the base of the orbit (DeJean) commencing with a lesion of the maxillary and pterygoid rami and evolving into the superior orbital ssure syndrome Jacod-Rollet (often combined with the syndrome of the superior orbital ssure); infraclinoid syndrome of Dandy Foix-Jefferson; syndrome of the sphenopetrosal ssure (Bonnet and Bonnet) corresponding in part to the cavernous sinus syndrome of Raeder Gradenigo-Lannois Lesions of the third, fourth, sixth, and rst divisions of the fth nerves with ophthalmoplegia, pain, and sensory disturbances in the area of V1; often exophthalmos, some vegetative disturbances Visual disturbances, central scotoma, papilledema, optic nerve atrophy; occasional exophthalmos, chemosis Ophthalmoplegia due to lesions of the third, fourth, sixth, and often fth nerves; exophthalmos; vegetative disturbances Jefferson distinguished three syndromes: (1) the anterior-superior, corresponding to the superior orbital ssure syndrome; (2) the middle, causing ophthalmoplegia and lesions of V1 and V2; (3) the caudal, in addition affecting the whole trigeminal nerve Lesions of the fth and sixth nerves with neuralgia, sensory, and motor disturbances, diplopia
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Superior orbital ssure
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Cavernous sinus
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Tumors that invade the anterior part of the base of the skull from the frontal sinus, nasal cavity, or the ethmoid bone, osteomas Meningiomas of the olfactory groove Tumors: meningiomas, osteomas, dermoid cysts, giant-cell tumors, tumors of the orbit, nasopharyngeal tumors; more rarely optic nerve gliomas; eosinophilic granulomas, angiomas, local or neighboring infections, trauma Optic nerve glioma, infraclinoid aneurysm of the internal carotid artery, trauma, orbital tumors, Paget disease Tumors of the sellar and parasellar area, infraclinoid aneurysms of the internal carotid artery, nasopharyngeal tumors, stulas of the sinus cavernosus and the carotid artery (traumatic), tumors of the middle cranial fossa, eg, chondromas, meningiomas, and neurinomas
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Apex of the petrous temporal bone
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In ammatory processes (otitis), tumors such as cholesteatomas, chondromas, meningiomas, neurinomas of the gasserian ganglion and trigeminal root, primary and secondary sarcomas at the base of the skull
The main clinical syndromes caused by tumors at the base of the skull
EPONYM CLINICAL SYMPTOMS ETIOLOGYa
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Sphenoid and petrosal bones (petrosphenoidal syndrome) Jacod
Tumors of the sphenoid and petrosal bones and middle cranial fossa, nasopharyngeal tumors, metastases
Jugular foramen Vernet
Anterior occipital condyles Collet-Sicard (Vernet-Sargnon)
Retroparotid space (retropharyngeal syndrome) Villaret Garcin (Guillain-Alajouanine-Garcin); also described by Hartmann in 1904
Tumors of the glomus jugulare; neurinomas of eighth, ninth, tenth, and eleventh nerves; chondromas, cholesteatomas, meningiomas, nasopharyngeal and ear tumors; infections, angiomas, rarely trauma Tumors of the base of the skull, ear, parotid; leukemic in ltrates; aneurysms, angiomas, and in ammations Tumors of the retroparotid space (carcinomas, sarcomas), trauma, in ammations Nasopharyngeal tumors, primary tumors at the base of the skull, leukemic in ltrates of basal meninges, trauma, metastases Acoustic neuromas (raised protein in CSF), meningiomas, cholesteatomas, metastases, cerebellar tumors, neurinomas of the caudal group of nerves and the trigeminal nerve, vascular processes such as angiomas, basilar aneurysms
Half of the base of the skull
Cerebellopontine angle
Ophthalmoplegia due to loss of function of the third, fourth, and sixth nerves; amaurosis; trigeminal neuralgia possibly with sensory signs Lesions of ninth, tenth, and eleventh nerves with disturbance of deglutition; curtain phenomenon; sensory disturbances of the tongue, soft palate, pharynx, and larynx; hoarseness; weakness of the sternocleidomastoid and trapezius Loss of twelfth nerve function (loss of normal tongue mobility) in addition to the symptoms of the jugular foramen Lesions of the lower group of nerves (ColletSicard) and Bernard-Horner syndrome with ptosis and miosis Loss of function of all twelve cranial nerves of one side; in many cases, isolated cranial nerves spared; rarely signs of raised intracranial pressure or pyramidal tract symptoms Loss of function of eighth nerve (hearing loss, vertigo, nystagmus); cerebellar disturbances; lesions of the fth, seventh, and possibly ninth and tenth cranial nerves Signs of raised intracranial pressure, brainstem symptoms
SOURCE:
Metastatic deposits may produce any of these syndromes Adapted by permission from Bingas
PART 4
MAJOR CATEGORIES OF NEUROLOGIC DISEASE
destructive lesions with remarkable delity, but in some cases, even when the tumor is seen with various studies, it may be dif cult to obtain a satisfactory biopsy Tumors of the Foramen Magnum Tumors in the region of the foramen magnum are of particular importance because of the need to differentiate them from diseases such as multiple sclerosis, Chiari malformation, syringomyelia, and bony abnormalities of the craniocervical junction Failure to recognize these tumors is a serious matter, since the majority are benign and extramedullary, ie, potentially resectable and curable If unrecognized, they terminate fatally by causing medullary and high spinal cord compression Although these tumors are not numerous (about 1 percent of all intracranial and intraspinal tumors), sizable series have been collected by several investigators (see F B Meyer et al for a complete bibliography) In all series, meningiomas, schwannomas, neuro bromas, and dermoid cysts are the most common types; others, all rare, are teratomas, dermoids, granulomas, cavernous hemangiomas, hemangioblastomas, hemangiopericytomas, lipomas, and epidural carcinomas Pain in the suboccipital or posterior cervical region, mostly on the side of the tumor, is usually the rst and by far the most prominent complaint In some instances the pain may extend into the shoulder and even the arm The latter distribution is more frequent with tumors arising in the spinal canal and extending intracranially than the reverse For uncertain reasons, the pain may radiate down the back, even to the lower spine Both spine and root pain can be recognized, the latter due to involvement of either the C2 or C3 root or both Weakness of one shoulder and arm progressing to the ipsilateral leg and then to the opposite leg and arm ( around the clock paralysis, page 52 is a characteristic but not invariable sequence of events, caused by the encroachment of tumor upon the decussating corticospinal tracts Occasionally both upper limbs are involved alone; surprisingly, there may be atrophic weakness of the hand or forearm or even intercostal muscles with diminished tendon re exes well below the level of the tumor, an observation made originally by Oppenheim which we have been able to con rm Involvement of sensory tracts also occurs; more often it is posterior column sensibility that is impaired on one or both sides, with patterns of progression similar to those of the motor paralysis Sensation of intense cold in the neck and shoulders has been another unexpected complaint, and also bands of hyperesthesia around the neck and back of the head Segmental bibrachial sensory loss has been demonstrated in a few of the cases and a Lhermitte sign (really a symptom) of electric-like sensations down the spine and limbs upon exing the neck has been reported frequently The cranial nerve signs most frequently conjoined and indicative of intracranial extension are dysphagia, dysphonia, dysarthria, and drooping shoulder (due to vagal, hypoglossal, and spinal accessory involvement); included less often are nystagmus and episodic diplopia, sensory loss over the face and unilateral or bilateral facial weakness, and a Horner syndrome The clinical course of such lesions often extends for 2 years or longer, with deceptive and unexplained uctuations With dermoid cysts of the upper cervical region, as in the case reported by Adams and Wegner, complete and prolonged remissions from quadriparesis may occur The important diagnostic procedure is MRI (Fig 31-20) and, if this is unavailable, CT myelography Tumors of the foramen magnum should be differentiated from spinal or brainstem-cerebellar multiple sclerosis, Chiari malformation with syrinx, and chronic adhesive arachnoiditis Persistent
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