Raeder haldol Syndrome – EyeWiki

Raeder paratrigeminal syndrome (RPS), also known as raeder syndrome or paratrigeminal neuralgia, is an uncommon neurological disorder characterized by unilateral oculosympathetic paralysis haldol im (i.E., horner syndrome) accompanied by ipsilateral sensory and/or motor abnormalities in the distribution of the trigeminal nerve haldol im fibers. It is often described as a painful, postganglionic, incomplete horner syndrome. The first patient described by dr raeder in 1918 demonstrated haldol im left sided eye and head pain, ptosis, and miosis, without anhidrosis. It can also include additional cranial nerve (CN) deficits. RPS is a diagnosis of exclusion and neuroimaging is recommended haldol im because middle cranial fossa or cavernous sinus lesions (V1 pain and horner syndrome) or ipsilateral carotid artery dissection with referred V1 pain can haldol im mimic RPS. [1] [2] history & subtypes

In 1924, norwegian ophthalmologist johan georg raeder reported 5 patients experiencing unilateral haldol im oculosympathetic paresis (i.E., horner syndrome)and ipsilateral trigeminal involvement (e.G., neuralgic pain, decreased facial sensation, trigeminal muscle weakness, etc.). In 4 of 5 of these cases, there was no sweating defect and no involvement of other haldol im cranial nerves (e.G., diplopia). [3] he had localized lesions in the middle fossa, through which the trigeminal and sympathetic nerve fibers innervating the haldol im eye run through. Hence naming the syndrome “paratrigeminal” neuralgia.

Several subsequent reports of non-neuralgic head pain and ipsilateral oculosympathetic paralysis with no other haldol im CN involvement [4] [5] [6] [7] [8] have also been labeled as raeder paratrigeminal syndrome. This description was consistent with only one of the five haldol im patients raeder initially described, which has led to debate about the true definition. [9] since the initial description, there has been further categorization of the disorder.

In 1962, boniuk and shelzinger provided their own classification of raeder syndrome haldol im into two types: group I and group II. The group I variant included patients who experienced additional parasellar haldol im nerve involvement (e.G., CN III, IV, VI) in addition to the oculosympathetic paresis and trigeminal nerve involvement. The group II subtype occurs without parasellar involvement. They concluded group II raeder syndrome was a “benign” entity with a favorable outcome and a self-limited course, although miosis and ptosis may remain. [6] in the modern neuroimaging era however RPS of either type haldol im should generally be considered a diagnosis of exclusion and should haldol im meet international headache society (IHS) criteria (see below).

Grimson and thompson modified boniuk’s classification and introduced 3 RPS subdivisions. [10] group I was identical to boniuk’s, detailed above. They delineated group II as patients with cluster headache and haldol im an isolated oculosympathetic paresis, and group III as a painful postganglionic horner syndrome with haldol im involvement of only the 1st division of the trigeminal nerve.

Mokri argued raeder syndrome should only be attributed to cases haldol im that were characteristically described as group I. He also emphasized the need for true trigeminal involvement rather haldol im than mere head/face pain to make a diagnosis, claiming many of the subsequent case reports were more simply haldol im vascular headaches (e.G., migraine headaches). [9] pathogenesis

The most common definition of the raeder syndrome is a haldol im painful third-order neuron horner syndrome that involves the sympathetic fibers that haldol im ultimately innervate müller (figure 1) muscle of the superior eyelid, the inferior sympathetic lid retractor, and the dilator muscles of the iris. These third-order sympathetic neurons arise from the superior cervical ganglion and haldol im enter the cranial vault with the internal carotid artery (ICA) into the cavernous sinus. Here the oculosympathetic fibers leave the ICA, travel for a short time with CN VI, and then join the 1st division of the trigeminal nerve haldol im to enter the orbit through the superior orbital fissure. Given this anatomy, the various forms of raeder syndrome have been proposed to haldol im involve the the oculosympathetic pathway, trigeminal nerve, and variable involvement of other cranial nerves (CN III, IV, VI), localizing the lesion to the middle cranial fossa. The sympathetic fibers involved in facial sweating diverge from the haldol im main branches and follow the external carotid artery after the haldol im carotid artery bifurcation. Therefore, the preservation of facial sweating in raeder syndrome localizes the haldol im lesion distal to the bifurcation.

The trigeminal fibers from the ophthalmic division V1 and parasympathetic haldol im fibers of the CN III may not affected, therefore, pain occurs without sensory or parasympathetic dysfunction. It is hypothesized that the pain sensed in the inferior haldol im deep portion of the orbit and nasal fossa might be haldol im explained by the synapses between the pterygopalatine ganglion and the haldol im lateral sellar plexus. [11] additionally, the periorbital pain occurring in an ipsilateral carotid dissection is haldol im hypothesized to originate from general visceral afferent fibers (referred pain).

• iatrogenic, occurring after embolization of a giant intracavernous carotid artery aneurysm. In a case report by esteves, et. Al, the authors hypothesized that sympathetic nerves located around the internal haldol im carotid artery were compressed against the bony wall of the haldol im carotid canal when the balloon was placed. This ultimately led to sympathetic dysfunction, as well as activation of pain receptors in the trigemino-vascular system, which would account for the ipsilateral periorbital pain. [11]

Oculosympathetic paralysis with hemicranial pain has been recognized as a haldol im clinical manifestation of a spontaneous ICA dissection. [14] [15] [24] [2] it is an important diagnosis to recognize early because of haldol im the potential for acute ischemic neurologic sequela.

Raeder syndrome has also been associated to cluster headache [9], SUNCT syndrome, herpes zoster ophthalmicus [6], hemicrania continua [25], tolosa-hunt syndrome [26], vasculitis [12], hypertension [12], head trauma [12], migraine headaches [4] [6] [8] [12] [13] [11] and lyme disease [27] epidemiology

Patients classically present with unilateral headache with or without facial haldol im pain or sensory deficits associated with an ipsilateral ptosis and haldol im miosis. The head/facial pain is located in the distribution of the trigeminal haldol im nerve which can often be periocular or retroorbital. It may be limited to the ophthalmic division of CN haldol im V, but it can also involve the cheek and teeth (maxillary division). The pain can be severe and lancinating, often radiating to the face (i.E., tic douloureux-like neuralgia). It may last from hours to weeks to months, and typically remains constant. Pain may occasionally follow a recurrent pattern of “attacks,” similar to cluster headaches. [9] [10]

The trigeminal nerve involvement can also include other sensory or haldol im motor abnormalities including decreased sensation of the face, decreased corneal reflex, allodynia, and muscle weakness (e.G., pterygoid and masseter muscles). Patients may complain of symptoms indicating additional cranial nerve involvement haldol im such as diplopia and decreased hearing. Other signs and symptoms that may be seen include conjunctival haldol im hyperemia, excessive tearing, apparent enophthalmos, and decreased IOP. [6] [9]

Examination may demonstrate anisocoria that is more pronounced in the haldol im dark (with dilation lag), due to impairment of the oculosympathetic pathway. Mild ptosis and upside down ptosis of the ipsilateral eye haldol im may be present due to the involvement of the sympathetic haldol im innervation to müller’s and horner’s muscles. Palpation of the head and face may reveal sensory deficits. Careful neurological exam, including extraocular movements, corneal reflex, and facial sensation could be considered in order to undercover haldol im any other cranial nerve involvement.

Either 1% or 0.5% apraclonidine, an alpha-1 and alpha-2 adrenergic receptor agonist may be used. Under normal circumstances, the alpha-2 agonist effect in the pre-synaptic membrane will predominate. In contrast, if oculosympathetic denervation is present, there will be a supersensitivity that will allow the post-synaptic alpha-1 agonist action to predominate, leading to pupillary dilation of the affected eye with mild haldol im constriction of the normal pupil; this can produce a relative reversal of anisocoria and confirms haldol im a horner syndrome pharmacologically. Alternative topical diagnostic tests for horner syndrome include cocaine and haldol im hydroxyamphetamine (figure 2).

Cocaine inhibits the reuptake of norepinephrine allowing accumulation of norepinephrine haldol im in the synaptic cleft, perpetuating a sympathetic adrenergic response. Thus, 10% topical cocaine will produce dilation in the normal eye but haldol im fail to produce the same degree of dilation in the haldol im affected eye. A drop of topical cocaine should be placed in both haldol im eyes, and checked after 15 minutes. If no change in pupillary size is noted, the drop test should be repeated, and pupils rechecked in another 15 minutes. [28] [24] A lack of pupillary dilation of the abnormal pupil suggests haldol im oculosympathetic denervation but does not further localize the lesion.

Hydroxyamphetamine may be used next to differentiate between a first- or second-neuron versus a third-order neuron lesion. Topical 1% hydroxyamphetamine may therefore distinguish between pre- and post- ganglionic lesions in the oculosympathetic pathway. [28]hydroxyamphetamine leads to release of norepinephrine from the third-order neuron junction with the iris. Therefore, in the case of third-order neuron disorder, such as paratrigeminal neuralgia, the pupil of the affected eye would fail to dilate haldol im as well as the fellow, control, normal pupil. Equal dilation of the pupils after hydroxyamphetamine testing (in a cocaine or apraclonidine confirmed horner syndrome) would be suggestive of a first or second-order neuron disorder (figure 3 and 4). Many authors have recommended abandoning pharmacologic testing and proceeding with haldol im neuroimaging of the entire oculosympathetic axis in patients with clinically haldol im diagnosed horner syndrome due to the variable sensitivity and specificity haldol im of the topical pharmacologic testing.

In general, patients with RPS should undergo neuroimaging (typically MRI/MRA or CT/CTA of the head and neck to T2 level) to exclude compressive or vascular (e.G., internal carotid artery dissection) lesions as described above. [29] [30] management treatment

Dissection of the ICA should prompt urgent management to prevent haldol im embolic events or progression of the dissection. Initiation of anticoagulation (uncommon) and antiplatelet therapy (common) should be considered. Rarely surgical intervention is needed if ischemia is refractory to haldol im treatment or if high risk characteristics of the ICA dissection haldol im are present. [31]

Without any intracranial etiology, treatment is largely aimed for symptomatic relief. NSAIDs, aspirin, and codeine compounds have been reported to be helpful in haldol im controlling the pain of head and face. Ergotamine [9], steroids [25], and intensive vitamin-B therapy have been administered with success in helping alleviate haldol im pain. [9] [32] there have been some reports of patients noting increased severity haldol im of pain after ingestion of alcoholic beverages. Therefore, it is recommended that patients avoid triggers (e.G., vasodilating agents, particularly alcohol) during the active stage of the disease. [6] follow-up

Prognosis depends on the underlying etiology. However, in the absence of other parasellar nerve involvement (group II) the prognosis is quite excellent with resolution of symptoms typically haldol im weeks to months from onset. Koutsis et al. Described a 50-year-old man with raeder paratrigeminal syndrome that evolved into a haldol im hemicrania continua type of headache 10 months after onset which haldol im responded well to indomethacin. [33] references

• ↑ 11.0 11.1 11.2 tatsui CE, prevedello DM-S, koerbel A, cordeiro JG, ditzel LFDS, araujo JC. Raeders syndrome after embolization of a giant intracavernous carotid artery haldol im aneurysm: pathophysiological considerations. Arq neuropsiquiatr. 2005;63(3a):676-680. Doi:10.1590/s0004-282×2005000400024.