NEUROLOGICAL SIGN
Neurological red flag: the numb chin Ammar Kheder,1 Nigel Hoggard,2 Simon J Hickman1 1
Department of Neurology, Royal Hallamshire Hospital, Sheffield, UK 2 Department of Neuroradiology, Royal Hallamshire Hospital, Sheffield, UK Correspondence to Dr Simon Hickman, Department of Neurology, Royal Hallamshire Hospital, Glossop Rd, Sheffield S10 2JF, UK; [email protected] Published Online First 22 November 2013
To cite: Kheder A, Hoggard N, Hickman SJ. Pract Neurol 2014;14:258–260.
258
CASE HISTORY A 51-year-old man presented to the neurology clinic with numbness affecting the left side of his chin, which had developed over a few days. Three weeks later, he reported pain affecting the left side of his face, locking of the jaw on chewing and numbness of the left side of the tongue. He worked as a maintenance fitter in a sewage works and had suffered exposure to raw sewage 2 months before his clinic visit, followed by diarrhoea and vomiting. Serological testing for leptospirosis was negative. These symptoms had settled before the numbness developed. He had no headaches or visual symptoms. His swallowing and speech were normal. He had lost 10 kg in weight over the previous 3 months. He had a strong family history of bowel carcinoma but a recent screening colonoscopy with biopsies was negative. He had never smoked and only drank alcohol occasionally. On examination, he had reduced sensation to light touch and pain over the left side of his chin and lower lip. The sensation over the rest of his face was normal; his corneal reflexes were present bilaterally and his jaw power was normal. Examination of the other cranial nerves and limbs was normal. His general physical examination was unremarkable. His full blood count, erythrocyte sedimentation rate, plasma glucose, urea and electrolytes, serum C reactive protein, calcium and liver function tests were normal apart from a raised serum alanine aminotransferase at 67 IU/L (normal 10–50). A contrast-enhanced MR scan of brain showed a single enhancing mass, measuring just over 2 cm diameter, centred on the left Meckel’s cave (figure 1). A CT scan of the skull base showed bony erosion through the petrous apex, which was also sclerotic in parts. The imaging findings were felt to be most in keeping with a metastasis. A CT scan of the chest, abdomen and pelvis showed a large heterogeneous mass within the right lobe of the liver (figure 2),
a 4.5 cm lymph node compressing the caudate lobe and the inferior vena cava, a 6 mm nodule in the left lower lobe of the lung and two further smaller nodules seen in the right lung. There was also a lytic lesion with an associated pathological fracture through the right inferior pubic ramus. A subsequent bone scan showed a focal area of increased uptake within the right inferior pubic ramus, consistent with a metastasis. A liver biopsy confirmed hepatocellular carcinoma (figure 3). Due to the extent of his disease, he was offered palliative care. During the course of his illness, he developed a left sixth nerve palsy. He died 11 months after first developing symptoms. DISCUSSION The nerves clinically affected to cause his numb chin and tongue were the left mental nerve and left lingual nerve, which are branches of the mandibular nerve. The mandibular nerve is the largest branch of the trigeminal nerve. The large sensory root proceeds from the lateral side of the trigeminal ganglion in Meckel’s cave and exits the skull through the foramen ovale. The smaller motor root passes under the ganglion and reunites with the sensory root after the foramen ovale in the infratemporal fossa. After giving off a meningeal branch and the nerve to the medial pterygoid, the mandibular nerve divides into anterior and posterior trunks. The anterior trunk contains the sensory buccal nerve and the motor branches to the rest of the muscles of mastication. The posterior trunk is mainly sensory and has three main branches: the auriculotemporal nerve; the lingual nerve and the inferior alveolar (dental) nerve. The inferior alveolar nerve passes through the mandibular foramen and travels in the mandibular canal to emerge from the mental foramen as the mental nerve, which innervates the skin of the chin and the lower lip.1 Table 1 summarises the differential diagnosis of a presentation with a numb chin.2
Kheder A, et al. Pract Neurol 2014;14:258–260. doi:10.1136/practneurol-2013-000738
NEUROLOGICAL SIGN
Figure 1
Post-gadolinium axial (A) and coronal (B) T1-weighted MRIs showing an enhancing mass in the left Meckel’s cave (arrowed).
Among 27 patients presenting with unilateral numbness or paraesthesia of the chin, the causes were invasive dental procedures (63%), inflammation (15%) and malignancy (22%).3 In our patient, the combination of chin numbness with pain and his reported weight loss made malignancy the most likely aetiology, which was confirmed on investigation. Charles Bell first described numbness of the chin as a sign of malignancy in 1830, in a patient with mandibular metastases from breast carcinoma compressing the mandibulo labrialis (inferior alveolar) nerve.4 In 1937, Roger and Paillas confirmed that isolated mental neuropathy was a warning sign for the presence of metastases.5 The presentation of a malignancy
with a numb chin is therefore sometimes referred to as Roger’s sign. In 1963, Calverley and Mohnac coined the term ‘syndrome of the numb chin’ in their report of five cases of mental neuropathy caused by metastases.6 There is one previous case report from Japan of metastatic hepatocellular carcinoma presenting with a numb chin.7 Malignancy can lead to a numb chin through mandibular metastasis, base of skull lesions, leptomeningeal involvement in the area of the trigeminal nerve root or perineural infiltration of the mental nerve.8 MR scan with gadolinium will detect intracranial disease and leptomeningeal invasion. CT scan of the brain and mandible will show bony lesions or skull
Figure 2 Post-contrast axial CT scan of abdomen showing a large heterogeneous mass in the right lobe of the liver (arrowed).
Figure 3 Medium-power photomicrograph of a H&E-stained section from the liver biopsy. There are pleomorphic cells resembling hepatocytes showing marked mitotic activity, including abnormal mitotic figures. The appearances are those of hepatocellular carcinoma and this was confirmed by a typical immunohistochemical profile.
Kheder A, et al. Pract Neurol 2014;14:258–260. doi:10.1136/practneurol-2013-000738
259
NEUROLOGICAL SIGN Table 1 The differential diagnosis of a presentation with a numb chin Class of disease
Potential causes
Dental
Dental anaesthesia Sagittal split osteotomies Trauma Root infection Mandibular osteomyelitis Mandibular cysts Mandibular atrophy Odontogenic tumours Mandibular tumours Metastases Head and neck Breast Bronchial Gastrointestinal Melanoma Ovarian Prostate Acute lymphoblastic leukaemia Acute myeloid leukaemia Hodgkin’s disease Lymphoma, lymphosarcoma Multiple myeloma Waldenström’s macroglobulinaemia Multiple sclerosis Sarcoidosis Diabetes mellitus Sickle cell crisis Giant cell arteritis Vasculitis Connective tissue disease Syphilis Borrelia HIV Post-immunisation Radiotherapy Chemical exposure Idiopathic
Neoplasia
Neurological Systemic disease
Infections
Others
base pathology. Cerebrospinal fluid cytological examination may detect abnormal cells in carcinomatous meningitis if imaging does not show a cause.8 The presentation of malignant disease with a numb chin indicates a poor prognosis with a mean survival in one series of 14.8 months in those with a distant primary tumour and 28.2 months in those with local disease.9 Sewage workers are at risk of several bacterial, viral, fungal, helminthic and protozoal infections. The particular infection with neurological complications in sewage workers is leptospirosis.10 One study highlighted an increased mortality among sewage workers from both malignant and non-malignant liver diseases.
260
This was felt to be mostly due to excessive alcohol consumption, although some of the risks were thought to be due to occupational exposure to chemical and infectious agents.11 CONCLUSION The complaint of a numb chin is a seemingly innocuous presentation but it can be a strong indicator for the presence of malignant disease and warrants urgent investigation. Contributors AK wrote the first draft. NH interpreted the images and revised the manuscript. SJH was in clinical charge of the patient’s care and revised the final version of the manuscript. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Andrew Larner, Liverpool, UK.
REFERENCES 1 Williams PL, Warwick R, Dyson M, et al. Gray’s Anatomy. 37th edn. Edinburgh: Churchill Livingstone, 1989:1103–7. 2 Laurencet FM, Anchisi S, Tullen E, et al. Mental neuropathy: report of five cases and review of the literature. Crit Rev Oncol Hematol 2000;34:71–9. 3 Kalladka M, Proter N, Benoliel R, et al. Mental nerve neuropathy: patient characteristics and neurosensory changes. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:364–70. 4 Furukawa T. Charles Bell’s description of numb chin syndrome. Neurology 1988;38:331. 5 Roger H, Paillas J. Le signe du mentonnier ( paresthésie et anesthésie unilatérale) révélateur d’un processus néoplasique métastatique. Rev Neurol (Paris) 1937;2:751–2. 6 Calverley JR, Monhac AM. Syndrome of the numb chin. Arch Intern Med 1963;112:819–21. 7 Yamashiro T, Nakamatsu K, Nakamura A, et al. A case of metastatic hepatocellular carcinoma with numb chin syndrome as the initial manifestation. J Jpn Stoma Soc 2011;60:7–11. 8 Lossos A, Siegal T. Numb chin syndrome in cancer patients: etiology, response to treatment, and prognostic significance. Neurology 1992;42:1181–4. 9 Sanchis JM, Bagán JV, Murillo J, et al. Mental neuropathy as a manifestation associated with malignant processes: its significance in relation to patient survival. J Oral Maxillofac Surg 2008;66:995–8. 10 Al-Batanony MA, El-Shafie MK. Work-related health effects among wastewater treatment plants workers. Int J Occup Environ Med 2011;2:227–44. 11 Wild P, Ambroise D, Benbrik E, et al. Mortality among Paris sewage workers. Occup Environ Med 2006;63:168–72.
Kheder A, et al. Pract Neurol 2014;14:258–260. doi:10.1136/practneurol-2013-000738
Neurological red flag: the numb chin Ammar Kheder,1 Nigel Hoggard,2 Simon J Hickman1 1
Department of Neurology, Royal Hallamshire Hospital, Sheffield, UK 2 Department of Neuroradiology, Royal Hallamshire Hospital, Sheffield, UK Correspondence to Dr Simon Hickman, Department of Neurology, Royal Hallamshire Hospital, Glossop Rd, Sheffield S10 2JF, UK; [email protected] Published Online First 22 November 2013
To cite: Kheder A, Hoggard N, Hickman SJ. Pract Neurol 2014;14:258–260.
258
CASE HISTORY A 51-year-old man presented to the neurology clinic with numbness affecting the left side of his chin, which had developed over a few days. Three weeks later, he reported pain affecting the left side of his face, locking of the jaw on chewing and numbness of the left side of the tongue. He worked as a maintenance fitter in a sewage works and had suffered exposure to raw sewage 2 months before his clinic visit, followed by diarrhoea and vomiting. Serological testing for leptospirosis was negative. These symptoms had settled before the numbness developed. He had no headaches or visual symptoms. His swallowing and speech were normal. He had lost 10 kg in weight over the previous 3 months. He had a strong family history of bowel carcinoma but a recent screening colonoscopy with biopsies was negative. He had never smoked and only drank alcohol occasionally. On examination, he had reduced sensation to light touch and pain over the left side of his chin and lower lip. The sensation over the rest of his face was normal; his corneal reflexes were present bilaterally and his jaw power was normal. Examination of the other cranial nerves and limbs was normal. His general physical examination was unremarkable. His full blood count, erythrocyte sedimentation rate, plasma glucose, urea and electrolytes, serum C reactive protein, calcium and liver function tests were normal apart from a raised serum alanine aminotransferase at 67 IU/L (normal 10–50). A contrast-enhanced MR scan of brain showed a single enhancing mass, measuring just over 2 cm diameter, centred on the left Meckel’s cave (figure 1). A CT scan of the skull base showed bony erosion through the petrous apex, which was also sclerotic in parts. The imaging findings were felt to be most in keeping with a metastasis. A CT scan of the chest, abdomen and pelvis showed a large heterogeneous mass within the right lobe of the liver (figure 2),
a 4.5 cm lymph node compressing the caudate lobe and the inferior vena cava, a 6 mm nodule in the left lower lobe of the lung and two further smaller nodules seen in the right lung. There was also a lytic lesion with an associated pathological fracture through the right inferior pubic ramus. A subsequent bone scan showed a focal area of increased uptake within the right inferior pubic ramus, consistent with a metastasis. A liver biopsy confirmed hepatocellular carcinoma (figure 3). Due to the extent of his disease, he was offered palliative care. During the course of his illness, he developed a left sixth nerve palsy. He died 11 months after first developing symptoms. DISCUSSION The nerves clinically affected to cause his numb chin and tongue were the left mental nerve and left lingual nerve, which are branches of the mandibular nerve. The mandibular nerve is the largest branch of the trigeminal nerve. The large sensory root proceeds from the lateral side of the trigeminal ganglion in Meckel’s cave and exits the skull through the foramen ovale. The smaller motor root passes under the ganglion and reunites with the sensory root after the foramen ovale in the infratemporal fossa. After giving off a meningeal branch and the nerve to the medial pterygoid, the mandibular nerve divides into anterior and posterior trunks. The anterior trunk contains the sensory buccal nerve and the motor branches to the rest of the muscles of mastication. The posterior trunk is mainly sensory and has three main branches: the auriculotemporal nerve; the lingual nerve and the inferior alveolar (dental) nerve. The inferior alveolar nerve passes through the mandibular foramen and travels in the mandibular canal to emerge from the mental foramen as the mental nerve, which innervates the skin of the chin and the lower lip.1 Table 1 summarises the differential diagnosis of a presentation with a numb chin.2
Kheder A, et al. Pract Neurol 2014;14:258–260. doi:10.1136/practneurol-2013-000738
NEUROLOGICAL SIGN
Figure 1
Post-gadolinium axial (A) and coronal (B) T1-weighted MRIs showing an enhancing mass in the left Meckel’s cave (arrowed).
Among 27 patients presenting with unilateral numbness or paraesthesia of the chin, the causes were invasive dental procedures (63%), inflammation (15%) and malignancy (22%).3 In our patient, the combination of chin numbness with pain and his reported weight loss made malignancy the most likely aetiology, which was confirmed on investigation. Charles Bell first described numbness of the chin as a sign of malignancy in 1830, in a patient with mandibular metastases from breast carcinoma compressing the mandibulo labrialis (inferior alveolar) nerve.4 In 1937, Roger and Paillas confirmed that isolated mental neuropathy was a warning sign for the presence of metastases.5 The presentation of a malignancy
with a numb chin is therefore sometimes referred to as Roger’s sign. In 1963, Calverley and Mohnac coined the term ‘syndrome of the numb chin’ in their report of five cases of mental neuropathy caused by metastases.6 There is one previous case report from Japan of metastatic hepatocellular carcinoma presenting with a numb chin.7 Malignancy can lead to a numb chin through mandibular metastasis, base of skull lesions, leptomeningeal involvement in the area of the trigeminal nerve root or perineural infiltration of the mental nerve.8 MR scan with gadolinium will detect intracranial disease and leptomeningeal invasion. CT scan of the brain and mandible will show bony lesions or skull
Figure 2 Post-contrast axial CT scan of abdomen showing a large heterogeneous mass in the right lobe of the liver (arrowed).
Figure 3 Medium-power photomicrograph of a H&E-stained section from the liver biopsy. There are pleomorphic cells resembling hepatocytes showing marked mitotic activity, including abnormal mitotic figures. The appearances are those of hepatocellular carcinoma and this was confirmed by a typical immunohistochemical profile.
Kheder A, et al. Pract Neurol 2014;14:258–260. doi:10.1136/practneurol-2013-000738
259
NEUROLOGICAL SIGN Table 1 The differential diagnosis of a presentation with a numb chin Class of disease
Potential causes
Dental
Dental anaesthesia Sagittal split osteotomies Trauma Root infection Mandibular osteomyelitis Mandibular cysts Mandibular atrophy Odontogenic tumours Mandibular tumours Metastases Head and neck Breast Bronchial Gastrointestinal Melanoma Ovarian Prostate Acute lymphoblastic leukaemia Acute myeloid leukaemia Hodgkin’s disease Lymphoma, lymphosarcoma Multiple myeloma Waldenström’s macroglobulinaemia Multiple sclerosis Sarcoidosis Diabetes mellitus Sickle cell crisis Giant cell arteritis Vasculitis Connective tissue disease Syphilis Borrelia HIV Post-immunisation Radiotherapy Chemical exposure Idiopathic
Neoplasia
Neurological Systemic disease
Infections
Others
base pathology. Cerebrospinal fluid cytological examination may detect abnormal cells in carcinomatous meningitis if imaging does not show a cause.8 The presentation of malignant disease with a numb chin indicates a poor prognosis with a mean survival in one series of 14.8 months in those with a distant primary tumour and 28.2 months in those with local disease.9 Sewage workers are at risk of several bacterial, viral, fungal, helminthic and protozoal infections. The particular infection with neurological complications in sewage workers is leptospirosis.10 One study highlighted an increased mortality among sewage workers from both malignant and non-malignant liver diseases.
260
This was felt to be mostly due to excessive alcohol consumption, although some of the risks were thought to be due to occupational exposure to chemical and infectious agents.11 CONCLUSION The complaint of a numb chin is a seemingly innocuous presentation but it can be a strong indicator for the presence of malignant disease and warrants urgent investigation. Contributors AK wrote the first draft. NH interpreted the images and revised the manuscript. SJH was in clinical charge of the patient’s care and revised the final version of the manuscript. Competing interests None. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Andrew Larner, Liverpool, UK.
REFERENCES 1 Williams PL, Warwick R, Dyson M, et al. Gray’s Anatomy. 37th edn. Edinburgh: Churchill Livingstone, 1989:1103–7. 2 Laurencet FM, Anchisi S, Tullen E, et al. Mental neuropathy: report of five cases and review of the literature. Crit Rev Oncol Hematol 2000;34:71–9. 3 Kalladka M, Proter N, Benoliel R, et al. Mental nerve neuropathy: patient characteristics and neurosensory changes. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2008;106:364–70. 4 Furukawa T. Charles Bell’s description of numb chin syndrome. Neurology 1988;38:331. 5 Roger H, Paillas J. Le signe du mentonnier ( paresthésie et anesthésie unilatérale) révélateur d’un processus néoplasique métastatique. Rev Neurol (Paris) 1937;2:751–2. 6 Calverley JR, Monhac AM. Syndrome of the numb chin. Arch Intern Med 1963;112:819–21. 7 Yamashiro T, Nakamatsu K, Nakamura A, et al. A case of metastatic hepatocellular carcinoma with numb chin syndrome as the initial manifestation. J Jpn Stoma Soc 2011;60:7–11. 8 Lossos A, Siegal T. Numb chin syndrome in cancer patients: etiology, response to treatment, and prognostic significance. Neurology 1992;42:1181–4. 9 Sanchis JM, Bagán JV, Murillo J, et al. Mental neuropathy as a manifestation associated with malignant processes: its significance in relation to patient survival. J Oral Maxillofac Surg 2008;66:995–8. 10 Al-Batanony MA, El-Shafie MK. Work-related health effects among wastewater treatment plants workers. Int J Occup Environ Med 2011;2:227–44. 11 Wild P, Ambroise D, Benbrik E, et al. Mortality among Paris sewage workers. Occup Environ Med 2006;63:168–72.
Kheder A, et al. Pract Neurol 2014;14:258–260. doi:10.1136/practneurol-2013-000738
