Original articles The numb chin R . P . M . B r u y n * a n d W. B o o g e r d * *
Introduction Summary An apparently harmless symptom, such as numbness of the chin, familiar to all who have had local aneasthesia in the dentist's chair, occasionally betrays more alarming underlying disease (table 1). Sometines, its presence is even ominous, reflecting underlying malignancy. It was even the presenting symptom of malignant disease in as much as 8 of the 19 cases 1. As such, it has been noted in nasopharyngeal carcinoma 2 and in a variety of solid 3,4 as well as leukaemic metastases to the mandible, the base of the skull, or, in a few instances, to the leptomeninge s 1'5'6. The numb chin represents loss of function of the terminal and sensory branch of the mandibular nerve, the mental nerve (fig. 1). The numb chin syndrome may be produced by any pathological process involving the mental nerve, the mandibular nerve or even the mandibular trunk 68. In the latter, the terminal sensory cutaneous distribution of the peripheral nerve is apparently affected. Most often, the syndrome appears to be caused by a metastatic or infiltrating malignancy of the jaw. Its symptoms are blunted sensation to light touch and pinprick within the area of innervation, occasionally associated with par- or dysaesthesias, or, less frequently still, by pain. The patient may experience the feeling of a thickened lip. There
An apparently innocuous complaint such as a numb chin may be associated with malignant disease, either as heralding symptom or as a manifestation of metastasis. A series of 15 patients with numbness of the chin is presented in which a malignancy was diagnosed prior to the numbness. The numbness diminished or disappeared in 66% of the patients following either systemic chemotherapy or radiotherapy. Key words: Numbness-Chin-Malignancy
is no impairment of taste. The absence of marked pain in the majority of instances strongly argues in favour of non-involvement of the Gasserian ganglion 7,9. The discrepancy between the minor inconveniency of a numb chin (or cheek) and the grave, often latent condition generating it, might lead the unwary diagnostician astray. For that reason, and because the numb chin syndrome is supposedly rare, a series of patients is presented.
* Department of Neurology Medisch Spectrum Twente, Enschede, The Netherlands, * * Department of Neurology, Antoni van Leeuwenhoek Ziekenhuis, Amsterdam, The Netherlands Address for correspondence and reprint requests: R. P.M. Bruyn, Dept. of Neurology, Oudenrijn Hospital Utrecht, van Heuven Goedhartlaan 1, 3527 CE Utrecht, The Netherlands. Accepted 31.12.90 Clin Neurol Neurosurg 1991. Vol. 93-3
187
Table 1. N o n - t u m o u r a l causes of the n u m b chin syndrome. Disorder
Author
E d e n t u l o u s elderly Syphilis
Furukawa 13 Ornsteen 43 McAlpine 44 Horowitz 27 Daly et a126 Jefferson 45 K o n o t e y - A h u l u 46
Multiple sclerosis(*) Vertebral insufficiency(*) Amyloidosis Sarcoidosis Sickle cell crisis (* *) Carotid a n e u r y s m Chemical intoxication trichlorethylene stilbamidine allopurinol Connective tissue disease
Syringobulbia (* * *)
* ** ***
Kirson and Tomaro47 Friedlander et a148 Goldstein et a122 Mitchell and Parson-Smith 49 Collard and Hargreaves 5~ Goodstein and H i m m e l f a r b 51 A s h w o r t h and Tait 33 B r u y n 34 H a g e n et a135 Blau et al 9
In these conditions, diminished nociception (pain, temperature) prevails over diminished proprioception (touch). In sickle cell anemia, mental n e u r o p a t h y starts with a pain-crisis, and leaves n u m b n e s s which m a y persist for up to a year. T h e n e u r o p a t h y is probably due to occlusive disease of the vasa n e r v o r u m , leading to nerve infarction. T h e presenting s y m p t o m was a painless, n u m b face.
Patients and results
The records were reviewed from 15 patients who were diagnosed with a numb chin syndrome. All patients were known with a malignancy. Three patients had a lymphoreticular malignancy and 12 had a carcinoma (table 2). In all carcinoma patients bone metastases had been diagnosed before they presented with a numb chin. Roentgenological analysis including CT-scanning of the brain and mandibles and plain films of the mandibles as well as repeated examination of the cerebrospinal fluid (CSF) yielded no abnormalities in the 3 patients with lymphoreticular malignancy (patients 1, 2 and 5). All 3 patients experienced a widespread malignant bone marrow infiltration; the numb chin diminished or disappeared concurrent with haematological remission following systemic chemotherapy. Roentgenological analysis in the 12 carcinoma patients showed a pathological lesion in the mandible at the same site of the numbness in 5 patients (patients 3, 7, 10, 12 and 15), and a 188
lesion at the other site in 1 patient (patient 8). In 4 patients bonescan or CT-scan demonstrated lesions at the base of the skull without abnormalities in the mandibles (patients 6, 9, 11 and 13). In one patient leptomeningeal metastasis was diagnosed (patient 4), without evident lesions at the base of the skull. In this patient the numb chin disappeared after radiotherapy of the brain, including the clivus. Overall, 12 patients received treatment after the numb chin had been diagnosed: 9 patients received systemic chemotherapy and 3 patients local radiotherapy to the base of the skull; in 8 of these treated patients the numb chin diminished or disappeared, usually in about 2 months. Discussion
The numb chin syndrome was initially known as 'signe de la houppe du menton' or 'signe de Roger '1~ and it had been mentioned as early as 190911. The very first neurologist to describe the syndrome was Charles Bell in 183012,13. In the Anglosaxon medical literature, recognition of the numb chin syndrome in malignant
Figure 1. Anatomic preparation showing the inferior alveolar nerve and mental nerve.
disease is of fairly recent vintage. Even in an exhaustive survey on 5,778 patients with lymphoma and leukemia in the late fifties 14 among whom 25 patients with involvement of the trigeminal nerve were mentioned, isolated mental neuropathy was not recognised. After the princeps observations by Cuneo and Rand 7 in a single case of a malignant Schwannoma at the skull base, and by Calverley and Mohnac 15in a series of five patients (2 with pain), the status of the numb chin syndrome as a mononeuropathy of the mental nerve, reflecting underlying malignant disease became firmly established only in the last two decades 1'3'6. Metastases of solid malignancies to the mandible are rare (+ 1%) and of leukaemia and lymphoma exceptional (+ 0.5%) 14'16-18.Moreover, local secondary deposits or infiltration of the jaws will only rarely induce a numb chin syndrome 16. Once a patient presents with a numb chin, the prognosis is usually grim. The original site may be any of the viscera, with cancers of the mammary gland, kidneys, thyroid and lungs heading the list and those of stomach, cutaneous melanoma and testes bringing up the rear 16'19"21.The great majority of those presenting with a numb chin syndrome have a lifeexpectancy of less than 11/2years ~,4. This series of patients, among the largest reported, affirms that a relatively small and apparently innocuous symptom may be the harbinger of grave events.
The patient presenting with the symptoms of the numb chin syndrome essentially poses a diagnostic dilemma. From the differential diagnostic point of view, the majority of conditions that may cause it (table 1) can be readily checked. One of the first decisions the diagnostician has to make is whether the syndrome (and, for that matter, also the numb cheek and numb face syndromes) is part and parcel of a trigeminal (sensory) neuropathy. It has been reported that in a general population in about half the cases a dental cause will be found, while in four-fifths of the instances thorough diagnostic work-up will establish a definite aetiology22. Notably the numb chin syndrome must be differentiated from the mental/submental analgesia due to neuropraxia of the mylohyoid nerve during third molar extraction 23. The 'numb cheek syndrome '24, caused by involvement of the infraorbital and anterior and middle superior alveolar nerves, or 'numb face syndrome '25'26, is also not seldomly caused by a malignant lesion. About half of the series of over 60 patients with 'numb face syndrome' proved to have neoplastic disease in the cerebellopontine angle or in the skull base 27. In the condition called 'chronic benign trigeminal paresis '2831 pain precedes or accompanies sensory impairment and motor deficit is often associated, and the same obtains for lesions involving the Gasserian ganglion 32. Pure sensory 189
Table 2.
190
Pat.
Sex
Age
Primary tumor
Numb chin
1
F
17
Burkitt's lymphoma (3-'86) Recurrence (9-'86)
9-'86
L + R
2
F
25
A L L ('88)
6-'89 9-'89
L R
3
F
32
Breast (3-'89) Bone metastases (11-'89)
2-'90
L
4
F
33
Breast ('88) Bone metastases (11-'89)
2-'90
L
5
M
39
A M L ('88)
4-'89
R
6
F
45
Breast (3-'89) Bone metastases (4-'89)
5-'90
L
7
M
50
Prostate (2-'88) Bone metastases (9-'89) Liver metastases ('89)
11-'88 L
8
F
51
Breast (8-'84) Bone metastases (7-'89)
1-'90
L
9
F
54
Breast ('79) Bone metastases (7-'90) Liver metastases (8-'90)
7-'90
L+R
10
M
56
Coloncarcinoma ('80)
8-'90
L
11
F
57
Breast ('81) Bone metastases ('82) Liver metastases ('87)
12-'86 R
12
F
62
Breast ('73) Bone metastases ('78) Epidural metastasis ('84) L4-L5
12-'84 R
13
F
67
Breast ('81) Bone metastases ('84)
7-'85 9-'85
14
F
70
Breast ('72) Bone metastases (4-'88) Epidural metastases
11-'89 R
15
F
72
Breast ('82) Bone metastases
3-'89
L L + R
L + R
Table 2 (continuation) Investigations
Therapy
Result
CT-brain: normal CT-mandible: normal CSF: normal
Chemotherapy
Complete remission Numbness disappeared
CT-brain: normal CSF: normal
Chemotherapy
Numbness L disappeared R persisted
Bonescan: metastasis at clivus and mental protuberance of the mandible
Chemotherapy
Numbness disappeared
CT-brain: cerebellar metastasis CSF: malignant cells
Radiotherapy
Numbness disappeared (4-'90) Died (6-'90) Cause: extracranial tumoractivity
X-mandible: normal CSF: normal
Chemotherapy
Numbness disappeared
CT-brain: metastasis at the base of the skull CSF: normal
Chemotherapy
Numbness persisted
Bonescan: metastases at the base of the skull and left mandible
Radiotherapy
Died (4-'89)
X-mandible: metastasis at the right side CSF: normal
Chemotherapy
Numbness disappeared (3-'90)
CT-brain: normal Bonescan: metastases at the base of the skull
Withdrawn
Died (9-'90) Cause: extracranial tumoractivity
Radiotherapy
Numbness persisted
Bonescan: diffuse metastases at the base of the skull CSF: normal
Chemotherapy
Numbness disappeared (2:87) Died (9-'87) Cause: extracranial tumoractivity
X-mandible: pathologic fracture right mandible
Chemotherapy
Numbness disappeared Died (11-'86) Cause: extracranial tumoractivity
CT-brain: metastasis at the base of the skull CSF: normal
Chemotherapy
Died (11-'85) Cause: extracranial tumoractivity
CSF:normal
Withdrawn
Died (12-'89) Cause: extracranial tumoractivity
X-mandible: metastases at the mental foramen L + R
Withdrawn
Numbness persisted Died (6-'89) Cause: extracranial tumoractivity.
Bonescan: metastasis at the 9 left mandible CSF: normal
All = acute lymphocytic leukemia AML = acute myelogenous leukemia
L = left R = right CSF = cerebrospinal fluid
191
t r i g e m i n a l n e u r o p a t h y w i t h o u t p a i n or m o t o r deficit is n o t i n f r e q u e n t l y seen in c o n n e c t i v e tissue disease or g r a n u l o m a t o s i s 3335. H o w e v e r , despite r e a s s u r i n g r e p o r t s o n the b e n i g n n a t u r e of a n isolated s e n s o r y t r i g e m i n a l n e u r o p a t h y 9,36,37, the physician s h o u l d n o t b e disc h a r g e d f r o m the r e s p o n s i b i l i t y to carry o u t a full w o r k - u p for such a n a p p a r e n t l y m i n o r a n d i n n o c u o u s c o m p l a i n t . E x c l u d i n g n e o p l a s i a is the m o s t i m p o r t a n t task as. Massey et al I e v e n w e n t so far as to state: ' n o n t r a u m a t i c m e n t a l n e u r o p a t h y is r a r e l y c a u s e d by b e n i g n lesions', a n d ' s h o u l d i n i t i a t e the search for c a n c e r ' . Following complete neurological examina t i o n i n c l u d i n g a precise e x a m i n a t i o n of trigemiHal f u n c t i o n s a n d local i n s p e c t i o n a n d p a l p a t i o n i n c l u d i n g the oral cavity, r o e n t g e n o l o g i c a l exa m i n a t i o n is i n d i c a t e d : p l a i n films of the m a n dible a n d a b o n e s c a n , or a l t e r n a t i v e l y , in case o f b o n e m e t a s t a s e s , C T s c a n n i n g of the base of the skull a n d m a n d i b l e s . C o m p u t e d t o m o g r a p h y a p p e a r s to b e the m o s t a c c u r a t e diagnostic m e t h o d in visualizing m a l i g n a n t osseous infiltration. S o m e t i m e s , these i m a g i n g t e c h n i q u e s m a y fail to d e m o n s t r a t e a causative lesion. I n o u r e x p e r i e n c e , this p a r t i c u l a r l y occurs in patients with a l y m p h o r e t i c u l a r m a l i g n a n c y ; in these i n s t a n c e s , the lesion is p r e s u m a b l y caused by diffuse b o n e m a r r o w infiltration. O f s o m e a d d i t i o n a l , t h o u g h n o t diagnostic v a l u e m a y b e E M G e x a m i n a t i o n , i n c l u d i n g the b l i n k reflex, m a s s e t e r reflex, the m a s s e t e r i n h i b itory reflex 39"41, a n d S S E P of the m e n t a l n e r v e 42. E x a m i n a t i o n of the C S F is g e n e r a l l y u n r e w a r d i n g 1, b u t in o u r view still r e c o m m e n d e d b e c a u s e of the i m p o r t a n t i m p l i c a t i o n s in (the rare) case of l e p t o m e n i n g e a l infiltration.
7 CUNEOHM, RANDCW.Tumor of the left gasserian ganglion associated with enlargement of the mandibular nerve. A review of the literature and case report. J. Neurosurg. 1952; 9:423-31. 8
14
15 16 17 18 19 20
21
22
23
24 25
MASSEY EW, MOORE J, SCHOLD SC. Mental neuropathy from systemic cancer. Neurology 1981; 31:1277-81. 2 MAWDSLEYC. InternationalSymposium on some aspects of Neurology. In: R.F. Robertson, ed. Livingstone, Edinburgh, 1968:85. 3
HORTON J~ MEANS EDs CUNNINGHAM TJ~ OLSON KB. T h e
numb chin in breast cancer. J. Neurol. Neurosurg. Psychiat. 1973; 36:211-6. 4 ROHRER MD, COLYER J. Mental nerve paresthesia: symptom for a widespread skeletal metastatic adenocarcinoma. J. Oral Surg. 1981; 39:442-5. 5 NOBLERMP. Mental nerve palsy in malignant lymphoma. Cancer 1969; 24:122-7. 6 RUBINSTEINMK. Cranial mononeuropathy as the first sign of intracranial metastasis. Ann. Intern. Med. 1969; 70:49-54. 192
WILLIAMS HM, DIAMOND H D , CRAVER LF, PARSONS H.
Neurological complications of lymphomas and leukemias. Springfield, Ill.: Charles C. Thomas, 1959: 4-8, 73-85. CALVERLEY CD, MOHNAC AM. Syndrome of the numb chin. Arch. Intern. Med. 1963; 112:819-21. CASH CO, ROYER RO , DAHLIN DC. Metastatic tumors of the jaws. Oral Surg. 1961; 14:897-905. CLAUSEN F, PAULSEN H. Metastatic carcinoma to the jaws. Acta Pathol. Microbiol. Scand. 1963; 57:361-74. VAN DER KWAST WAM, VAN DER WAAL I. J a w metastases. Oral Surg. 1974; 37:850-57. CASTIGLIANO SG, ROMINGER CJ. Metastatic malignancy of the jaws. Am. J. Surg. 1953; 87:496-507. VREBOS JER, MASSON JK, HARRISON E G . Metastatic carcinoma of the mandible with primary tumor in the lung. Am. J. Surg. 1961; 102:52-7. MYALL RWT, MORTON T H H , WORTHINGTON PH. M e l a n -
metastatic to the mandible. Report of a case. Int. J. Oral Surg. 1983; 12:56-9. GOLDSTEIN NP, G1B1LISCO JA, RUSHTON JG. Zrigeminal neuropathy and neuritis: a study of etiology with emphasis on dental causes. J. Amer. Med. Ass. 1963; 184:45862. ROBERTSGOD,HARRISM. Neurapraxia of the mylohyoid nerve and submental analgesia. Brit. J. Oral Surg. 1973; 11:110-13. CAMPBELLWW. The numb cheek syndrome: A sign of infraorbital neuropathy. Neurology 1986; 36:421-3. BLACKWOODHJJ. Metastatic carcinoma of the mandibular condyle. Oral Surg. 1956; 9:1318-23. DALY D D , LOVE JG, DOCKERTY MB. Amyloid tumor of the Gasserian ganglion. J. Neurosurg. 1957; 14:347-52. HOROWITZSH. Isolated facial numbness. Clinical significance and relation to trigeminal neuropathy. Ann. Intern. Med. 1974; 80:49-53. HILLTR. TWOcases of trigeminal neuropathy. Proc. Roy. Soc. Med. 1954; 47:914-5. HUGHESB. Chronic benign trigeminal paresis. Proc. Roy. Soc. Med. 1958; 51:529-31. SP1LLANE JO, WELLS CEC. Isolated trigeminal neuropathy. A report of 16 cases. Brain 1959; 82:391-416. SEWARDMHE. Anaesthesia of the lower lip. A problem in differential diagnosis. Brit. Dental J. 1962; 113:423-6. JEFFERSONG. The trigeminal neurinomas with some remarks on malignant invasion of the Gasserian ganglion. Clin. Neurosurg. 1955; 1:11-54. oma
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P. The dissemination of tumors of the head and neck via the cranial nerves. Radiol. Clin. Nth. Amer. 1970; 8:445-61. 9 BLAU JN, HARRIS M, KENNETF S. Trigeminal sensory neuropathy. New Engl. J. Med. 1969; 281:873-6. 10 ROGER H, PAILLAS J. L e signe du mentonnier r6v61ateur (paresth6sie et anesth6sie unilat6rale) d'un processus n6oplasique m6tastatique. Rev. Neurol. (Paris) 1937; 68:751-2. 11 SCHLESINGERH. Typische Neuritis des N. alveolaris inferior infolge einer Carcinommetastase im Unterkiefer. Mitt. Ges. inn. Mediz. Wien 1909; 8:841-8. 12 FURUKAWAT. Charles Bell's description of numb chin syndrome. Neurology 1988; 38:331. 13 FURUKAWAT. Numb chin syndrome in the elderly. J. Neurol. Neurosurg. Psychiat. 1990; 53:173.
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27 28 29 30
31 32
Trigeminal neuropathy i n c o n tissue disease. Neurology 1971; 21:609-14. 34 BRUVNGW. Angiopathic neuropathy in collagen vascular disease. In: Vinken PJ, Bruyn GW, Klawans HL, eds. Handbook of Clinical Neurology. Elsevier Sci. Publ., Amsterdam, Vol. 51, ch. 24, 1987:445-60. 35 H A G E N N A , S T E V E N S J C , M I C H E T J R . C J . Trigeminal sensory neuropathy associated with connective tissue disease. Neurology 1990; 40:891-6. 36 E G G L E S T O N D J , H A S K E L L R . Idiopathic trigeminal sensory neuropathy. Practitioner 1972; 208:649-55. 37 FISHERCM. Trigeminal sensory neuropathy. Arch. Neurol. 1983; 40:591-2. 38 THRUSHDC, SMALLM. HOW benign a symptom is facial numbness? Lancet 1970; 2:851-4. 39 A U G E R R G , M C M A N I S P G . Trigeminal sensory neuropathy associated with decreased oral sensation and impairment of the masseter inhibitory reflex. Neurology 1990; 40:759-63. 40 L E A N D R I M~ P A R O D I CI~ R I G A R D O S t F A V A L E E . Early scalp responses evoked by stimulation of the mental nerve. Neurology 1990, 40:315-20. 41 N A K A S H I M A K , T A K A H A S H I K~ A Z U M I T , I S H I D A G . Exteroceptive suppression of the masseter and temporalis muscles produced by electrostimulation of the mental nerve in Parkinson's disease. Acta Neurol. Scand. 1990; 81:407-10.
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ASHWORTH B, TAIT GBW. nective
Somatosensory evoked potentials to electrical stimulation of the mental nerve. Brit. J. Oral Maxiliofacial Surg. 1987; 25:300-7. 43 ORNSTEEN AM. Isolated bilateral trigeminal nerve disease presumably syphilitic in origin. J. Nerv. Merit. Dis. 1931, 74:297-300. 44 M C A L P I N E D , L U M S D E N C E , A C H E S O N E D . Multiple sclerosis, a reappraisal. Livingstone, Edinburgh, 1965:121. 45 JEFFERSONG. Sarcoidosis of the nervous system. Brain 1957; 80:540-56. 46 KONOTEY-AHULUFI. Mental nerve neuropathy: A complication of sickle-cell crisis. Lancet 1972; 2:388-9. 47 K I R S O N L E , T O M A R O A J . Mental nerve paresthesia secondary to sickle-cell crisis. Oral Surg. Oral Med. Oral Pathol. 1979; 48:509-12. 48 F R I E D L A N D E R A H , G E N S E R L , S W E R D L O F F M . Mental nerve neuropathy: A complication of sickle-cell crisis. Oral Surg. 1980; 49:15-7. 49 M I T C H E L L A B S , P A R S O N S - S M I T H B G . Trichloroethylene neuropathy. Brit. Med. J. 1969; 1:422-3. 50 C O L L A R D P, H A R G R E A V E S W . Neuropathy after stilbamidine. Lancet 1947; 2:686-8. 51 G O O D S T E I N D R , H I M M E L F A R B R . Allopurinol-induced mandibular ncuropathy. Abbreviated case report. Oral Surg. 1975; 40:51-2.
42
GODFREY RM, MITCHELL KW.
193
Introduction Summary An apparently harmless symptom, such as numbness of the chin, familiar to all who have had local aneasthesia in the dentist's chair, occasionally betrays more alarming underlying disease (table 1). Sometines, its presence is even ominous, reflecting underlying malignancy. It was even the presenting symptom of malignant disease in as much as 8 of the 19 cases 1. As such, it has been noted in nasopharyngeal carcinoma 2 and in a variety of solid 3,4 as well as leukaemic metastases to the mandible, the base of the skull, or, in a few instances, to the leptomeninge s 1'5'6. The numb chin represents loss of function of the terminal and sensory branch of the mandibular nerve, the mental nerve (fig. 1). The numb chin syndrome may be produced by any pathological process involving the mental nerve, the mandibular nerve or even the mandibular trunk 68. In the latter, the terminal sensory cutaneous distribution of the peripheral nerve is apparently affected. Most often, the syndrome appears to be caused by a metastatic or infiltrating malignancy of the jaw. Its symptoms are blunted sensation to light touch and pinprick within the area of innervation, occasionally associated with par- or dysaesthesias, or, less frequently still, by pain. The patient may experience the feeling of a thickened lip. There
An apparently innocuous complaint such as a numb chin may be associated with malignant disease, either as heralding symptom or as a manifestation of metastasis. A series of 15 patients with numbness of the chin is presented in which a malignancy was diagnosed prior to the numbness. The numbness diminished or disappeared in 66% of the patients following either systemic chemotherapy or radiotherapy. Key words: Numbness-Chin-Malignancy
is no impairment of taste. The absence of marked pain in the majority of instances strongly argues in favour of non-involvement of the Gasserian ganglion 7,9. The discrepancy between the minor inconveniency of a numb chin (or cheek) and the grave, often latent condition generating it, might lead the unwary diagnostician astray. For that reason, and because the numb chin syndrome is supposedly rare, a series of patients is presented.
* Department of Neurology Medisch Spectrum Twente, Enschede, The Netherlands, * * Department of Neurology, Antoni van Leeuwenhoek Ziekenhuis, Amsterdam, The Netherlands Address for correspondence and reprint requests: R. P.M. Bruyn, Dept. of Neurology, Oudenrijn Hospital Utrecht, van Heuven Goedhartlaan 1, 3527 CE Utrecht, The Netherlands. Accepted 31.12.90 Clin Neurol Neurosurg 1991. Vol. 93-3
187
Table 1. N o n - t u m o u r a l causes of the n u m b chin syndrome. Disorder
Author
E d e n t u l o u s elderly Syphilis
Furukawa 13 Ornsteen 43 McAlpine 44 Horowitz 27 Daly et a126 Jefferson 45 K o n o t e y - A h u l u 46
Multiple sclerosis(*) Vertebral insufficiency(*) Amyloidosis Sarcoidosis Sickle cell crisis (* *) Carotid a n e u r y s m Chemical intoxication trichlorethylene stilbamidine allopurinol Connective tissue disease
Syringobulbia (* * *)
* ** ***
Kirson and Tomaro47 Friedlander et a148 Goldstein et a122 Mitchell and Parson-Smith 49 Collard and Hargreaves 5~ Goodstein and H i m m e l f a r b 51 A s h w o r t h and Tait 33 B r u y n 34 H a g e n et a135 Blau et al 9
In these conditions, diminished nociception (pain, temperature) prevails over diminished proprioception (touch). In sickle cell anemia, mental n e u r o p a t h y starts with a pain-crisis, and leaves n u m b n e s s which m a y persist for up to a year. T h e n e u r o p a t h y is probably due to occlusive disease of the vasa n e r v o r u m , leading to nerve infarction. T h e presenting s y m p t o m was a painless, n u m b face.
Patients and results
The records were reviewed from 15 patients who were diagnosed with a numb chin syndrome. All patients were known with a malignancy. Three patients had a lymphoreticular malignancy and 12 had a carcinoma (table 2). In all carcinoma patients bone metastases had been diagnosed before they presented with a numb chin. Roentgenological analysis including CT-scanning of the brain and mandibles and plain films of the mandibles as well as repeated examination of the cerebrospinal fluid (CSF) yielded no abnormalities in the 3 patients with lymphoreticular malignancy (patients 1, 2 and 5). All 3 patients experienced a widespread malignant bone marrow infiltration; the numb chin diminished or disappeared concurrent with haematological remission following systemic chemotherapy. Roentgenological analysis in the 12 carcinoma patients showed a pathological lesion in the mandible at the same site of the numbness in 5 patients (patients 3, 7, 10, 12 and 15), and a 188
lesion at the other site in 1 patient (patient 8). In 4 patients bonescan or CT-scan demonstrated lesions at the base of the skull without abnormalities in the mandibles (patients 6, 9, 11 and 13). In one patient leptomeningeal metastasis was diagnosed (patient 4), without evident lesions at the base of the skull. In this patient the numb chin disappeared after radiotherapy of the brain, including the clivus. Overall, 12 patients received treatment after the numb chin had been diagnosed: 9 patients received systemic chemotherapy and 3 patients local radiotherapy to the base of the skull; in 8 of these treated patients the numb chin diminished or disappeared, usually in about 2 months. Discussion
The numb chin syndrome was initially known as 'signe de la houppe du menton' or 'signe de Roger '1~ and it had been mentioned as early as 190911. The very first neurologist to describe the syndrome was Charles Bell in 183012,13. In the Anglosaxon medical literature, recognition of the numb chin syndrome in malignant
Figure 1. Anatomic preparation showing the inferior alveolar nerve and mental nerve.
disease is of fairly recent vintage. Even in an exhaustive survey on 5,778 patients with lymphoma and leukemia in the late fifties 14 among whom 25 patients with involvement of the trigeminal nerve were mentioned, isolated mental neuropathy was not recognised. After the princeps observations by Cuneo and Rand 7 in a single case of a malignant Schwannoma at the skull base, and by Calverley and Mohnac 15in a series of five patients (2 with pain), the status of the numb chin syndrome as a mononeuropathy of the mental nerve, reflecting underlying malignant disease became firmly established only in the last two decades 1'3'6. Metastases of solid malignancies to the mandible are rare (+ 1%) and of leukaemia and lymphoma exceptional (+ 0.5%) 14'16-18.Moreover, local secondary deposits or infiltration of the jaws will only rarely induce a numb chin syndrome 16. Once a patient presents with a numb chin, the prognosis is usually grim. The original site may be any of the viscera, with cancers of the mammary gland, kidneys, thyroid and lungs heading the list and those of stomach, cutaneous melanoma and testes bringing up the rear 16'19"21.The great majority of those presenting with a numb chin syndrome have a lifeexpectancy of less than 11/2years ~,4. This series of patients, among the largest reported, affirms that a relatively small and apparently innocuous symptom may be the harbinger of grave events.
The patient presenting with the symptoms of the numb chin syndrome essentially poses a diagnostic dilemma. From the differential diagnostic point of view, the majority of conditions that may cause it (table 1) can be readily checked. One of the first decisions the diagnostician has to make is whether the syndrome (and, for that matter, also the numb cheek and numb face syndromes) is part and parcel of a trigeminal (sensory) neuropathy. It has been reported that in a general population in about half the cases a dental cause will be found, while in four-fifths of the instances thorough diagnostic work-up will establish a definite aetiology22. Notably the numb chin syndrome must be differentiated from the mental/submental analgesia due to neuropraxia of the mylohyoid nerve during third molar extraction 23. The 'numb cheek syndrome '24, caused by involvement of the infraorbital and anterior and middle superior alveolar nerves, or 'numb face syndrome '25'26, is also not seldomly caused by a malignant lesion. About half of the series of over 60 patients with 'numb face syndrome' proved to have neoplastic disease in the cerebellopontine angle or in the skull base 27. In the condition called 'chronic benign trigeminal paresis '2831 pain precedes or accompanies sensory impairment and motor deficit is often associated, and the same obtains for lesions involving the Gasserian ganglion 32. Pure sensory 189
Table 2.
190
Pat.
Sex
Age
Primary tumor
Numb chin
1
F
17
Burkitt's lymphoma (3-'86) Recurrence (9-'86)
9-'86
L + R
2
F
25
A L L ('88)
6-'89 9-'89
L R
3
F
32
Breast (3-'89) Bone metastases (11-'89)
2-'90
L
4
F
33
Breast ('88) Bone metastases (11-'89)
2-'90
L
5
M
39
A M L ('88)
4-'89
R
6
F
45
Breast (3-'89) Bone metastases (4-'89)
5-'90
L
7
M
50
Prostate (2-'88) Bone metastases (9-'89) Liver metastases ('89)
11-'88 L
8
F
51
Breast (8-'84) Bone metastases (7-'89)
1-'90
L
9
F
54
Breast ('79) Bone metastases (7-'90) Liver metastases (8-'90)
7-'90
L+R
10
M
56
Coloncarcinoma ('80)
8-'90
L
11
F
57
Breast ('81) Bone metastases ('82) Liver metastases ('87)
12-'86 R
12
F
62
Breast ('73) Bone metastases ('78) Epidural metastasis ('84) L4-L5
12-'84 R
13
F
67
Breast ('81) Bone metastases ('84)
7-'85 9-'85
14
F
70
Breast ('72) Bone metastases (4-'88) Epidural metastases
11-'89 R
15
F
72
Breast ('82) Bone metastases
3-'89
L L + R
L + R
Table 2 (continuation) Investigations
Therapy
Result
CT-brain: normal CT-mandible: normal CSF: normal
Chemotherapy
Complete remission Numbness disappeared
CT-brain: normal CSF: normal
Chemotherapy
Numbness L disappeared R persisted
Bonescan: metastasis at clivus and mental protuberance of the mandible
Chemotherapy
Numbness disappeared
CT-brain: cerebellar metastasis CSF: malignant cells
Radiotherapy
Numbness disappeared (4-'90) Died (6-'90) Cause: extracranial tumoractivity
X-mandible: normal CSF: normal
Chemotherapy
Numbness disappeared
CT-brain: metastasis at the base of the skull CSF: normal
Chemotherapy
Numbness persisted
Bonescan: metastases at the base of the skull and left mandible
Radiotherapy
Died (4-'89)
X-mandible: metastasis at the right side CSF: normal
Chemotherapy
Numbness disappeared (3-'90)
CT-brain: normal Bonescan: metastases at the base of the skull
Withdrawn
Died (9-'90) Cause: extracranial tumoractivity
Radiotherapy
Numbness persisted
Bonescan: diffuse metastases at the base of the skull CSF: normal
Chemotherapy
Numbness disappeared (2:87) Died (9-'87) Cause: extracranial tumoractivity
X-mandible: pathologic fracture right mandible
Chemotherapy
Numbness disappeared Died (11-'86) Cause: extracranial tumoractivity
CT-brain: metastasis at the base of the skull CSF: normal
Chemotherapy
Died (11-'85) Cause: extracranial tumoractivity
CSF:normal
Withdrawn
Died (12-'89) Cause: extracranial tumoractivity
X-mandible: metastases at the mental foramen L + R
Withdrawn
Numbness persisted Died (6-'89) Cause: extracranial tumoractivity.
Bonescan: metastasis at the 9 left mandible CSF: normal
All = acute lymphocytic leukemia AML = acute myelogenous leukemia
L = left R = right CSF = cerebrospinal fluid
191
t r i g e m i n a l n e u r o p a t h y w i t h o u t p a i n or m o t o r deficit is n o t i n f r e q u e n t l y seen in c o n n e c t i v e tissue disease or g r a n u l o m a t o s i s 3335. H o w e v e r , despite r e a s s u r i n g r e p o r t s o n the b e n i g n n a t u r e of a n isolated s e n s o r y t r i g e m i n a l n e u r o p a t h y 9,36,37, the physician s h o u l d n o t b e disc h a r g e d f r o m the r e s p o n s i b i l i t y to carry o u t a full w o r k - u p for such a n a p p a r e n t l y m i n o r a n d i n n o c u o u s c o m p l a i n t . E x c l u d i n g n e o p l a s i a is the m o s t i m p o r t a n t task as. Massey et al I e v e n w e n t so far as to state: ' n o n t r a u m a t i c m e n t a l n e u r o p a t h y is r a r e l y c a u s e d by b e n i g n lesions', a n d ' s h o u l d i n i t i a t e the search for c a n c e r ' . Following complete neurological examina t i o n i n c l u d i n g a precise e x a m i n a t i o n of trigemiHal f u n c t i o n s a n d local i n s p e c t i o n a n d p a l p a t i o n i n c l u d i n g the oral cavity, r o e n t g e n o l o g i c a l exa m i n a t i o n is i n d i c a t e d : p l a i n films of the m a n dible a n d a b o n e s c a n , or a l t e r n a t i v e l y , in case o f b o n e m e t a s t a s e s , C T s c a n n i n g of the base of the skull a n d m a n d i b l e s . C o m p u t e d t o m o g r a p h y a p p e a r s to b e the m o s t a c c u r a t e diagnostic m e t h o d in visualizing m a l i g n a n t osseous infiltration. S o m e t i m e s , these i m a g i n g t e c h n i q u e s m a y fail to d e m o n s t r a t e a causative lesion. I n o u r e x p e r i e n c e , this p a r t i c u l a r l y occurs in patients with a l y m p h o r e t i c u l a r m a l i g n a n c y ; in these i n s t a n c e s , the lesion is p r e s u m a b l y caused by diffuse b o n e m a r r o w infiltration. O f s o m e a d d i t i o n a l , t h o u g h n o t diagnostic v a l u e m a y b e E M G e x a m i n a t i o n , i n c l u d i n g the b l i n k reflex, m a s s e t e r reflex, the m a s s e t e r i n h i b itory reflex 39"41, a n d S S E P of the m e n t a l n e r v e 42. E x a m i n a t i o n of the C S F is g e n e r a l l y u n r e w a r d i n g 1, b u t in o u r view still r e c o m m e n d e d b e c a u s e of the i m p o r t a n t i m p l i c a t i o n s in (the rare) case of l e p t o m e n i n g e a l infiltration.
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