Journal of Neurology, Neurosurgery, and Psychiatry, 1976, 39, 105-113

Trigeminal neuropathy with nasal ulceration:report of two cases and one necropsy

J. D. SPILLANE AND H. URICH

From the Department of Neurology, University Hospital of Wales, Cardiff,and the Institute ofPathology, The London Hospital, London

SYNOP SI s Two cases are reported of progressive trigeminal neuropathy with nasal ulceration. Onepatient developed signs of spinal cord involvement 15 years after the onset of trigeminal symptomsand died after a total course of 21 years. Necropsy revealed an unusual trigeminospinal systemdegeneration with deposition of amyloid-like substances in the affected structures. The other patientis alive eight years after the onset of symptoms, the only indication of a lesion outside the trigeminalnerve being a patch of numbness in one leg.

In a previous paper on isolated trigeminalneuropathy (Spillane and Wells, 1959) one of usdescribed a patient with total bilateral trigeminalloss accompanied by progressive ulceration ofher nose (case 15). We now wish to report thesubsequent evolution of her condition and post-mortem findings which we believe to be unique.We also wish to record another patient who dis-played similar symptoms and signs, at least inthe early stages of her disease.

CASE 1

CLINICAL HISTORY A widow, aged 59 years, was firstseen in 1950. Her illness began in 1949 with rednessand pain of both eyes. She attended an ophthalmicclinic where a diagnosis of bilateral keratocon-junctivitis (Sjogren's disease) was made. A fewmonths later a red, tender, and painful sore appearedat the edge of her left nostril. Biopsy in 1950 showeda hyperkeratotic lesion with superficial inflammationbut no evidence of neoplasm. The sore increased insize and the outer margin of the nostril was pro-gressively eroded. In January 1951 both sides of herforehead became numb; six months later her leftupper lip was numb. Thereafter, numbness spreadrapidly over the whole of the left side of her face andin August 1951 over the right side of her face. Hermouth and tongue became dry. In October 1951there was bilateral keratoconjunctivitis with smallirregular pupils and bilateral corneal opacities. Herleft nostril was ulcerated (Fig. la and b). The corneal(Accepted 19 August 1975.)

reflexes were absent and the sensations of touch,pain, and temperature were impaired in the first andsecond divisions on both sides, loss being absolutefor all modalities on the left side. There were slighterchanges in both third divisions. Deep pressure andvibration sense were normal. Taste was absent in theanterior two-thirds of the tongue. She could notappreciate the temperature of food on the left side

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FIG. 1 Case 1. Two years after onset; erosioni ofleft ala nasi, bilateral ptosis, anrd neuropathic keratitis.

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of her mouth. There was no involvemotor root of either trigeminal nervino symptom or sign of involvement elsnervous system and investigations i

negative. No evidence of syphilis,systemic lupus, or scleroderma emergeOver the years her condition stead]

By 1957 her nose was totally eroded (Fshe was profoundly anaemic and siweight. Eyesight was grossly impairecorneal opacities, she chewed and sw

FIG. 2 Case 1. Six years later; total destruction ofnose, bilateral trigeminal anaesthesia.

difficulty, and had no sense of taste. Nevertheless,she remained alert and uncomplaining; repeatedneurological examinations were otherwise negative.There was still no sign of involvement of the motorroots of the trigeminal nerves but the sensory loss

had progressed. She could scarcely detect thepresence of food in her mouth. Sensory loss was

more or less complete in both first and seconddivisions of the trigeminal nerves; it was impairedin both third divisions. Deep sensation was impaired.There was bilateral ptosis, small pupils, and loss ofsecretions from lacrimal and salivary glands. Sheretained a very feeble sense of smell.From 1958 onwards, until her death in 1970 from

bronchopneumonia at the age of 78 years, she re-

fused hospital admission and neurological examina-tions had to be made in her home. In the 1960s shebecame very thin, her jaw movements were weak and

ement of the no contraction of masticatory muscles could bee. There was palpated. There was almost total bilateral trigeminalewhere in the anaesthesia. Hearing remained normal, speechwere entirely intelligible. By the mid-'60s she was becoming un-

sarcoidosis, steady on her feet and she often stumbled and fell.d. Sphincter functions were not affected. She com-ily worsened. plained that her legs were cold and she oftenig. 2a and b), wrapped them in a shawl and retired to bed. Theie lost much lower limbs were very thin but arterial pulsations atd because of the ankles were normal. Knee and ankle jerks weretallowed with gradually reduced and subsequently lost. Extensor

plantar reflexes were first detected in 1965 andremained until her death five years later. No sensoryabnormality was found in her lower limbs until 1966when posture and vibration could not be appreci-ated in her feet. Subsequently, vibration could not beappreciated below the knees. Superficial sensationwas impaired only distally and never profoundly. Inher upper limbs, superficial sensation did not seemto be impaired, although grips were weak and handsclumsy. There was postural and vibration loss in the

t hands; the arm reflexes were never lost. She remainedable to feed herself with difficulty until her terminalillness. She was alert and orientated until the end.

/ Thus, this strange neurological illness began in thetrigeminal sensory system and some 15 years later

§S involved the spinal cord. There were no clues to itsnature.

NECROPSY FINDINGS (West Cardiff Area LaboratoryP.M. 213/70) There were no significant abnormali-ties outside the nervous system. In particular therewas no evidence of generalized amyloidosis, multiplemyeloma, systemic lupus erythematosus, or anyother multisystemic disease.

EXAMINATION OF NERVOUS SYSTEM (L.H. P.M.4/A/71) The macroscopic appearances of the brainwere unremarkable. Transverse sections through thespinal cord showed symmetrical greyish discolora-tion in the posterior parts of the lateral columns andin the posterior columns, particularly the fasciculusgracilis.

MICROSCOPIC APPEARANCES The lesions were con-fined to the trigeminal nerve and its pathways, andto the long tracts of the spinal cord.

SENSORY ROOT OF TRIGEMINAL NERVE The entireroot from its entry into the pons to the proximity ofthe Gasserian ganglion was severely affected. Theglial part of the root was devoid of axons and myelinsheaths and showed astrocytic proliferation withfibrillary gliosis. Distal to the glio-Schwannianjunction, the root was infiltrated by an abnormalsubstance, consisting of two components, A and B,

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TABLESTAINING REACTIONS OF INFILTRATING SUBSTANCES

Method Substance Control(sporadic amyloid neuropathy)

A B

Congo red+ polarized light Weakly positive birefringent, Negative Strongly to weakly positivedichroic, polarization colour birefringent, dichroic,green polarization colour green

Sirius red+ polarized light Positive birefringent, dichroic, Negative Positive birefringent, dichroicpolarization colour green polarization colour green

Wolman's toluidine blue + polarized Purplish blue birefringent, Blue Purplish blue birefringent,light dichroic, polarization colour dichroic, polarization colour

red red

Crystal violet Gamma metachromasia (red) Beta metachromasia Gamma metachromasia (red)(purple)

Lendrum's sodium sulphate-alcian Green Khaki Greenblue-van Gieson

Thioflavin T Greenish fluorescence Greenish fluorescence Greenish fluorescence

Luxol-fast blue-cresyl violet Light blue Dark blue Light blue

'S -- ->f>...... '

.Ej . -. -Kap1' t .,jU S . . S .

with different staining reactions (Table). SubstanceA was abundant at the junction, gradually givingway to infiltration with substance B (Fig. 3).Throughout its length, the root was devoid of axonsand myelin sheaths, the individual fibres beinginfiltrated with substance B. This formed solid ortubular strands varying in thickness, with a some-what irregular outline and a tendency to coarse

FIG. 3 Case 1. Trigeminal root;infiltration ofglio-Schwannianjunction by amyloid-like material.Lighter component representssubstance A, darker substance B.Crystal violet, x 90.

beading in places (Fig. 4). The infiltration stoppedshort of the entry of the sensory root into thetrigeminal ganglion where some deposits of substanceA again became apparent. Throughout the length ofthe root the walls of blood vessels were infiltratedwith substance B.

GASSERIAN GANGLION The neurones of the trig-

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FIG. 4 Case 1. Trigeminal root: infiltration along thecourse of nerve fibres and in walls of blood vessels.Thioflavin T (Mercury vapour lamp, exciter filterSchott UGJ, barrier filter Schott -50), x 200.

meninal ganglion were remarkably well preservedand only a few cells showed evidence of degenerationin the form of loss of nuclei and poor cytoplasmicstaining. Proliferation of capsule cells was seen in afew places, but there were no nodules of Nageotte or

other evidence of neuronal loss. The nerve fibreswithin the ganglion showed some degenerativechanges in the form of irregular beading, or club-shaped, spherical, or ovoid retraction bulbs. A fewargyrophilic baskets were present around neuronalcell bodies. The peripheral branches of the trigeminalnerves were not available for examination, but fibrebundles emerging from the ganglion containedabundant well myelinated axons.

MOTOR ROOT OF TRIGEMINAL NERVE Medial to theentry of the sensory root into the pons there were

skeins of well-myelinated large axons formingirregularly convoluted whorls, resembling those seen

FIG. 5 Case 1. Trigeminal root: tangled whorls oflarge calibre nerve fibres, probably derivedfrom motorroot. Glees-Marsland silver impregnation, x 120.

in traumatic neuromas of the nerve roots (Fig. 5).Their origin could not be traced with certainty, buttheir position and calibre of axons suggested thatthey were derived from the motor root.

CENTRAL TRIGEMINAL PATHWAYS The intrapontinecourse of the sensory trigeminal fibres showed totalloss of axons and myelin sheaths. No obviouslesions were seen in the motor, principal sensory, andmesencephalic nuclei of the 5th nerve. On the otherhand, the spinal trigeminal tract showed severe

degeneration and its nucleus marked, but not total,loss of neurones. In addition, the blood vessels withinthe tract and the nucleus were thickened and theirwalls infiltrated with an abnormal homogeneousmaterial showing the staining reactions of substanceB (Fig. 6). This infiltration, of variable thickness,appeared to be predominantly adventitial and didnot encroach upon the lumen of the vessels.

-%';;s a <

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*

.4'

'V

V

FIG. 6 Case 1. Nucleus ofspinal tract of trigeminalnerve: total loss of myelinatedfibres in tract and infiltrationof walls of blood vessels.Luxol fast blue-cresyl violet,x 60.

FIG. 7 Case 1. Cervical cord:degeneration of crossedpyramidal tracts and offasciculus gracilis; infiltrationof blood vessels in lateralcolumns only. Luxol fastblue-cresyl violet, x 10.

SPINAL CORD Throughout the spinal cord there wasalmost total loss of myelinated fibres in the crossedpyramidal tracts, the uncrossed tracts being un-affected (Fig. 7). On tracing the lesions into themedullary pyramids, the fibre loss became lessconspicuous and at pontine level no abnormalitycould be detected in the corticospinal tracts.Throughout the degenerate lateral columns theblood vessels showed conspicuous changes similarto those described above. The thickening andinfiltration appeared to be mainly adventitial, butsome vessels showed medial thickening with sub-intimal infiltration and possibly some narrowing ofthe lumen. The infiltrating material was almostexclusively substance B, but tiny fragments ofsubstance A were found in some vessels. These

vascular changes extended into the medullarypyramids, but not above that level.The posterior columns showed rarefaction, but

not total loss, of fibres in the lumbar segments. Thisloss became more severe at higher levels, and in thecervical segments was almost total in the fasciculusgracilis, the fasciculus cuneatus being well preserved.By contrast with the pyramidal tracts, abnormalvessels were almost completely absent in thedegenerated parts of the posterior columns.

DORSAL ROOT GANGLIA A few random posteriorroot ganglia available for examination showed nosignificant lesions.

MENINGEAL VESSELS A few vessels in the ventral cleft

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of the spinal cord showed infiltration with substanceB. The vessels over the surface of the cord showed noabnormality. A few circumferential vessels of thebrain stem showed traces of substance B in theirwalls. The blood vessels over and in the cerebralhemispheres showed no abnormality.

CASE 2

A married woman, aged 38 years, with one child wasfirst seen in 1968. No significant previous illnesseswere recorded. Onset of left-sided trigeminalneuralgia occurred in September 1967. There were notrigger phenomena; inter-paroxysmal facial achingcaused insomnia. Carbamazepine was ineffective.The skin on the affected side of the face was drierthan on the right. In January 1968 the facial painsbegan to subside but they were replaced by numbnessand paraesthesiae. There was no other neurologicalinvolvement. By February 1968 there was impair-ment of taste on the left side; tongue, gums, andcheek on the left side all felt numb. Examinationconfirmed impairment of touch, pain, and tempera-ture in the distribution of the second and thirddivisions of the left trigeminal nerve. There was nomotor trigeminal impairment. Corneal reflexes wereretained. Investigations revealed no evidence ofsyphilis, sarcoidosis, systemic lupus, or scleroderma.

Neuroradiological studies (pneumoencephalographyand vertebral angiography) were normal. Blood andspinal fluid were normal. Serum proteins and electro-phoresis were normal and the ESR was 15 mm/lh.

In December 1968 she began to notice a numbnessof her right cheek, by which time the left-sidednumbness had spread to involve the forehead andeye. The corneal reflex was absent on the left andthere was a left-sided Homer syndrome (Fig. 8a).On that side, the sensory loss was now dense and shedeveloped a neuropathic keratitis which requiredtarsorraphy. On the right side, during the followingyear, the numbness spread over the whole face. InJune 1969 further investigations were normal exceptfor a raised sedimentation rate (48 mm/lh). By early1970 she had lost 9 kg (20 lb) in weight since thebeginning of her illness. She found chewing andswallowing difficult and would lose the food in hermouth. A right tarsorraphy was necessary. When hereyes were closed she could scarcely appreciatepassive movements of the tip of the nose or of herupper lip. Taste was absent except for a fleetingsensation during the act of swallowing. There wasnow probable weakness of the masticatory muscles.ESR was 56 mm/lh; DAT 1 in 256. Prednisone 5 mgthrice daily was begun in February 1970. There wasno change during 1970 but in January 1971 earlyerosion of the left ala nasi was detected (Fig. 8b), and

FIG. 8 Case 2. (a) Fifteen months after onset; left Horner syndrome, left trigeminal sensory loss, absent leftcorneal reflex, early neuropathic keratitis, early sensory loss on right cheek. (b) Two years later; early erosionof left ala nasi. Bilateral trigeminal anaesthesia with bilateral neuropathic keratitis. (c) One year later; erosionof nasal septum and collapse of nose.

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during the course of that year the whole of the nasalseptum disintegrated and her nose collapsed (Fig.8c). No leprosy bacilli were discovered in her noseand nasal biopsy showed no evidence of granuloma.During the years 1972 and 1973 there was no newdevelopment. Her general condition was poor, herweight remained at about 50 kg (110 lb) and shewas depressed. The soft tissues of the nose shrankbut did not disappear as in case 1. She was providedwith a nasal prosthesis and she continued her workas a domestic help. Sedimentation rates were recordedduring these years at 34 and 52 mm/I h respectively.Serum proteins and electrophoresis remained nor-mal; negative antinuclear factor; positive rheumatoidscreening test, DAT 1 in 4.

Early in 1974, while in her bath, she discovered apatch of numbness on the outer side of the left legbelow the knee. It has persisted unchanged until thepresent (June 1975) and measured about 5 cm by3.8 cm (2 in. by 12 in.) and appears to be in the areasupplied by the musculocutaneous nerve. There is noalteration of the skin and no thickening of nerves.Electrodiagnostic tests were normal. The sensoryloss involves all modalities but it is not complete.Her general health has improved, she has put on5.4 kg in weight and is less depressed. She is takingprednisone 5 mg daily. Her nervous system is other-wise normal. In recent months there have beenseveral episodes of postural vertigo which havesubsided; menstruation has been irregular. Therehas been no sign of returning trigeminal sensibility.There is virtual bilateral total superficial anaes-thesia; vibration cannot be appreciated on the malaror mandibular prominences; passive movements ofthe tip of the nose and upper lips remain only feeblyappreciated.So in this case, also, we have a presentation of a

lesion in both trigenimal sensory systems, erosion ofthe nose, but so far the only indication of a lesionelsewhere in the nervous system consists of a patchof numbness on one leg.

DISCUSSION

Both cases presented with trigeminal sensoryloss which, in the first case, was bilateral fromthe outset, and in the second case becamebilateral after 15 months. Trophic erosion of thenose took place in each case. The trigeminalsensory loss was not dissociated; all superficialmodalities were involved from the outset; deeppressure and vibration became affected later.Taste was lost. Signs of involvement of thetrigeminal motor system also eventually oc-curred. No other cranial nerve was involved in

either case. A spinal cord lesion developed in thefirst case after some 15 years. In the second case,the only other sign to appear, after eight years,is a patch of numbness on the left leg. Noevidence of systemic disease was found in eithercase. The first patient died 21 years after thebeginning of her illness. The second patientremains at work as a domestic help.

In the literature of trigeminal neuropathythere are several references to its occurrence inassociation with connective tissue disease(Beighton et al., 1968; Kaltreider and Talal,1969; Gumpel, 1970a, b; Ashworth and Tait,1971 ; Lundberg and Werner, 1972; Whaley et al.,1973). In the majority of these cases the presenta-tion of illness was not with the trigeminal lesion;the latter usually appeared during the course ofthe systemic disorder. Lundberg and Wernersuggested that the neuropathy is caused byvascular lesions in the medulla because of thesparing of the corneal reflexes and the occasionalbilateral nature of the disorder of the trigeminalsensory system. Barraquer-Bordas et al. (1973)question this interpretation of the signs inbilateral cases. The relationship of unilateraltrigeminal sensory neuropathy to Bell's palsyappears to be a tenuous one (Seward, 1962;Ch'ien and Halsey, 1970; Eggleston andHaskell, 1972).

Shafar and Copp (1970) have described a casein which unilateral trigeminal sensory neuro-pathy was the only manifestation of widespreadperipheral neuropathy for a period of 10 years.It is obvious that nerve conduction studies mayreveal involvement of limb nerves when the onlyclinical feature remains numbness of the face.None of these associations was found in our

first patient on postmortem examination. Thelesions consisted of degeneration of the trige-minal root and its central pathways, and of thepyramidal tracts and posterior columns in thespinal cord. This was associated with depositionof an amyloid-like substance. The degenerationof the affected tracts was not accompanied bysignificant lesions in their cells of origin, suggest-ing a 'dying-back' mechanism. The associationof trigeminal lesions with long tract degenera-tions is unusual and we are not aware of asimilar combination reported either in genetic orsporadic system degenerations.The infiltrating substance was confined to

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connective tissue structures and involved thewalls of the blood vessels in the affected tractsin the central nervous system. In the trigeminalroot, it was apparently deposited on the endo-neurium surrounding the degenerated fibres as

well as on blood vessels, the infiltration stoppingabruptly at the glio-Schwannian junction. Thesubstance consisted of two components, differingin their staining properties, one sparse andfound predominantly at the glio-Schwannianjunction (substance A), the other abundant andwidely distributed (substance B). According tothe criteria laid down by Wolman (1971) foridentification of amyloid-like substances,material showing all the specific stainingreactions is 'amyloid', that showing only some

of them is 'pseudoamyloid'. By these criteria,our substance A represents true amyloid,substance B pseudoamyloid. Regrettably, no

material containing substance A was availablefor electron microscopy. Its fibrillary structurecan only be inferred from its optical propertiessuch as birefringence and dichroism. The ultra-structure of substance B was homogeneous andwould therefore be designated by some authori-ties as 'hyaline', a term reserved by Wolman formaterial showing none of the staining reactionsof amyloid. The relationship between amyloidand pseudoamyloid, or amyloid and hyaline,remains obscure. It is, however, of interest thatCoimbra and Andrade (1971a) observed deposi-tion of hyaline as well as fibrillary amyloid inearly cases of the Portuguese variant of familialamyloid neuropathy and suggested that thehyaline material might be a precursor of trueamyloid.The distribution of amyloid-like substances in

our case was unusual and has no counterpart inany known type of amyloidosis. It bears no

resemblance to any form of amyloid neuropathy,familial or sporadic (reviewed by Henson andUrich, 1970 and by Urich, 1976). Two unusualcases deserve a brief mention, that of Daly et al.(1957) and of Borghi and Tagliabue (1961). Inboth these cases, amyloid deposits were found inGasserian ganglia removed surgically for thetreatment of trigeminal neuralgia. At the time ofoperation these patients showed no evidence ofsystemic amyloidosis or peripheral neuropathy,and their trigeminal symptoms were unilateral.In the absence of follow-up studies the noso-

logical position of this localized Gasserianamyloidosis remains uncertain. It is also ofinterest that hyaline bodies, mainly related toblood vessels, were found in the dorsal rootganglia in cases of hereditary sensory radicularneuropathy studied by Denny-Brown (1951) andby van Bogaert (1953), but not in other similarcases. The material on Denny-Brown's casestained with Congo red.

In the central nervous system amyloid deposi-tion is common in the 'congophilic angiopathy'in the cerebral cortex of old people, sometimesassociated with senile dementia of the Alzheimertype (Corsellis and Brierley, 1954). In his ex-tensive studies on senile amyloidosis of thecardiovascular system, Schwartz (1969) em-phasized this involvement of cortical vessels,but did not mention similar changes in anyother part of the central nervous system.The strict association of the vascular

amyloidosis or pseudoamyloidosis with tractdegenerations in our case suggests a causalrelationship between the two processes. It couldbe argued that the vascular lesions were theprimary event, and the tract degenerationsrepresented a summation effect of multipleischaemic lesions leading to Wallerian degenera-tion. This problem has its counterpart inperipheral amyloid neuropathy, which, inKernohan and Woltman's (1942) view, repre-sented an ischaemic neuropathy due to oblitera-tion of the vasa nervorum. Doubts on thisinterpretation were cast when Dyck and Lambert(1969) demonstrated a selective loss of un-myelinated axons and when Coimbra andAndrade (1971b) suggested that fibre degenera-tion preceded the deposition of amyloid. Simi-larly, Schwartz's view that amyloidosis was thecause of senile dementia was refuted by Wis-niewski and Terry (1973). In our case, it wouldappear unlikely that a primary vascular diseaseshould be confined to well-defined tracts. Therewas no evidence that the patency of the vesselswas significantly impaired and there were nofocal areas of ischaemic necrosis. We thereforeprefer to interpret the findings as a primarysystem degeneration and the deposition ofamyloid-like substances as an epiphenomenon.

It remains to be explained why these vascularchanges are not seen more frequently in systemdegenerations. It is well known that amyloid

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deposition may be due to generalized distur-bances, such as excess of circulating light chainimmunoglobulins, or to strictly local causes,such as observed in certain types of tumours.In addition, it may be suggested that in somecases the interplay of local and general factorsmay be responsible both for the deposition andfor the localisation of amyloid. To quoteSchwartz (1969) '. . . amyloid is precipitatedwhen a morbid factor circulating in the blood,lymph, or cerebrospinal liquor, joins a compoundlocated in diseased structural elements of tissuesand cells'. Perhaps the lesions in our caserepresent an example of senile amyloidosislocalized in degenerating nervous tissue.

There is at present no indication that thecondition of our second patient is due to asimilar combination or distribution of lesions.So, while trigeminal neuropathy with progressivenasal ulceration forms a distinct clinical syn-drome, it would be premature to suggest that itconstitutes a nosological entity.

We are indebted to Dr N. G. Sanerkin for his necropsyfindings and for referring the material for furtherexamination.

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