Trigeminal Neuropathy Accompanied by a Pontine Lesion on MRI

□ CASE REPORT □

Yoichi Ohnuki, Atsushi Mizuma, Tsuyoshi Uesugi and Shunya Takizawa

Abstract

A 63-year-old man presented with the loss of the sensations of pain and temperature sensation in the right facial region innervated by the trigeminal nerve (V1 to 3). He showed abnormal lesions in the pons and the trigeminal nerve on magnetic resonance imaging (MRI). He had recurrent herpes in the nasal cavity, and a history of left facial palsy. We herein present the unique MRI findings and suggest that herpes simplex infection may cause trigeminal neuropathy. This is the first reported case of dissociated trigeminal neuropathy with herpes simplex infection which was accompanied by a pontine lesion on MRI.

Key words: dissociated trigeminal neuropathy, herpes simplex virus, MRI, pontine lesion

(Intern Med 55: 1187-1189, 2016) (DOI: 10.2169/internalmedicine.55.5730)

port of a patient with dissociated trigeminal sensory neuro- Introduction pathy, which was accompanied by a pontine lesion on MRI.

The trigeminal nerve is the largest of the 12 cranial Case Report nerves. It carries the sensory input from the supratentorial dura mater, face, cornea, and the nasal, oral, and sinus mu- A 63-year-old man noticed right palatine numbness after cosa (1). The trigeminal nerve has three main branches dis- the appearance of herpes in his nasal cavity. It spread to his tal to the trigeminal ganglion: the ophthalmic (V1), maxil- right chin, temple and forehead over several days. He was lary (V2), and mandibular (V3) nerves, of which the latter admitted to our hospital on the 14th day after the onset of also carries efferent motor signals to the muscles of mastica- symptoms. He had a history of left facial palsy and recur- tion (1). In the brainstem, the trigeminal nerve has three rent herpes in the oral mucosa and nasal cavity, but no his- sensory nuclei and a motor nucleus: a principle sensory nu- tory of hypertension. There was no remarkable family his- cleus, which mediates tactile sensation; a spinal trigeminal tory. His blood pressure was 126/78 mmHg. His neurologi- nucleus (STN), which mediates pain and temperature sensa- cal examination revealed right facial dysesthesia, and the tion; a mesencephalic nucleus, which contains the unipolar impairment of pain and temperature sensation in the V1-3 first-order ganglionic cells that receive proprioceptive input region. However, his facial tactile sensation was intact and from the V3; and a motor nucleus, which lies medially to symmetrical. He also reported right dysgeusia. The other the principle sensory nucleus in the mid-pons (1). The com- cranial nerves were demonstrated normal function. An ex- plex anatomy of the trigeminal nerve makes it difficult to amination for motor function, including masseter function, identify the foci that cause trigeminal neuropathies (TNOs). was normal. A blood chemistry analysis was unremarkable, A TNO is distinct from trigeminal neuralgia (TNA), which except for the elevation of the plasma glucose (121 mg/dL), is characterized by facial pain, but without sensory impair- triglyceride (310 mg/dL) and low-density lipoprotein ment or motor weakness. In a TNO, numbness or weakness (LDL) - cholesterol (156 mg/dL) levels. The patient was usually appears due to the destruction of neurons. In TNA, negative for serum antinuclear, anti - Sjögren’s-syndrome- the neurons are usually preserved, although their myelin related antigen A (SS-A) and anti - Sjögren’s-syndrome- sheaths may be destroyed (1). We herein present the first re- related antigen B (SS-B) antibodies, as well as anti - prote-

Division of Neurology, Department of Internal Medicine, Tokai University School of Medicine, Japan Received for publication May 12, 2015; Accepted for publication July 12, 2015 Correspondence to Dr. Yoichi Ohnuki, [email protected]

1187 Intern Med 55: 1187-1189, 2016 DOI: 10.2169/internalmedicine.55.5730

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Figure. (A)(C)(E)(G) A T2-weighted image shows hyperintense lesions on the right ventral side of the pons and the right trigeminal nerve. (B)(D)(F)(H) A post-gadolinium T1-weighted image shows the partial enhancement of the abnormal lesion in pons. (A) to (D) were performed on the day of admission. (E) to (H) were performed 1 month after admission. T2-weighted images show a high signal region in the pons and the right trigeminal nerve, which was enhanced by the administration of gadolinium. inase 3 neutrophil cytoplasmic antibody (PR3 - ANCA), formed on the 42nd day after the onset of symptoms (Fig- myeloperoxidase neutrophil cytoplasmic antibody (MPO- ure E-H). Although the abnormal enhancement remained, ANCA) and cyclic citrullinated peptide (CCP) antibodies. the patient’s right facial dysesthesia showed an almost com- The protein and glucose levels and the cell counts in the pa- plete recovery within 6 weeks after the onset of symptoms. tient’s cerebrospinal fluid were within the normal limits. We The impairment of his sensation of pain and temperature re- did not perform an electromyogram. A T2-weighted image covered within 10 weeks. on 1.5 T MRI showed a high signal region in the pons and the right trigeminal nerve, which was enhanced on the T1- Discussion weighted image by the administration gadolinium (Fig- ure A-D), with no other remarkable findings. Spinal MRI We herein present the case of a patient with dissociated showed a cervical and lumbar spinal canal stenosis. A chest sensory trigeminal neuropathy in whom MRI revealed ab- X-ray and abdominal computed tomography showed no ab- normalities of the right ventral side of the pons and the right normal findings. On the 16th day after the onset of symp- trigeminal nerve. toms, an enzyme immunoassay (EIA) IgG titer for herpes The sensory root of the trigeminal nerve passes back- simplex virus (HSV) in a serum sample was elevated to wards below the superior petrosal sinus and tentorium cere- 44.4. A cerebrospinal fluid (CSF) sample showed a titer of belli, and after entering the pons, divides into upper and 0.29. The patient’s serum titer subsequently reached 81 at 5 lower roots (2). The upper root (the main sensory tract) ends weeks after the onset of symptoms. The IgM titer for HSV partly in a nucleus situated in the pons, while the lower root was negative. The IgG titer for varicella zoster virus (VZV) (the spinal trigeminal tract) descends through the pons and was 19.4 (positive) in serum, and less than 0.2 (negative) in medulla oblongata, and ends in the upper part of the sub- CSF. The IgM titer was negative for VZV. A polymerase stantia gelatinosa of Rolando. The main sensory tract medi- chain reaction (PCR), which was performed to analyze the ates tactile sensation, while the spinal tract mainly mediates HSV genome in a serum sample, was negative. The patient pain and temperature sensation (2). Thus, this patient was diagnosed with trigeminal neuropathy based on the showed sensory dissociation. The spinal trigeminal nucleus presence of the herpes infection in his nasal cavity, and was and tract receive primary afferent fibers from the trigeminal, treated with acyclovir for 7 days. His right facial hemidyses- facial, glossopharyngeal, and vagal nerves (3). Thus, our pa- thesia improved by the end of the acyclovir therapy. We did tient not only had facial sensory impairment, but also dys- not administer steroids. The patient’s dysgeusia disappeared geusia. within 4 weeks after the onset of symptoms. MRI was per- Sensory trigeminal neuropathy is well known as a com-

1188 Intern Med 55: 1187-1189, 2016 DOI: 10.2169/internalmedicine.55.5730

Table. Previous Reports on Trigeminal Neuropathy Due to Herpes Simplex Accompanied by an Intramedullary Lesion on MRI.

reference age of impairment dissociated dysgeusia abnormality on MRI course number onset divisions neuropathy left intramedullary trigeminal 8 61 y/o V3 no data no data no data nerve root left intramedullary spinal resolved over 3 41 y/o V1-3 - + trigeminal nucleus and tract 3 weeks right intramedullary spinal resolved over our case 63 y/o V1-3 + + trigeminal nucleus and tract 4 weeks

mon complication of autoimmune connective tissue and in- the enhancement of the lesion on MRI was not uniform flammatory diseases, such as mixed connective tissue dis- (Figure B, D). We could not determine whether our patient’s ease, progressive systemic sclerosis and multiple sclero- herpes infection was caused by HSV type 1 from serum or sis (1, 4, 5). However, infection is not considered to be a CSF examinations, however, the effectiveness of acyclovir is common mechanism of trigeminal neuropathy (TNO) (1). consistent with this as a possible cause. Connective tissue diseases based on the results of the blood We presented the case of a patient with dissociated analysis and multiple sclerosis was excluded based on the trigeminal sensory neuropathy and unique MRI findings MRI findings. Consequently, the patient was diagnosed with concerning the foci responsible for the impairment. a herpes infection. Although herpes simplex virus has been implicated in many cases of presumed idiopathic Bell’s The authors state that they havenoConflictofInterest(COI). palsy, in which patients typically exhibit acute peripheral facial nerve palsy, ipsilateral TNO may be seen in up to 25% References of patients with Bell’s palsy (6). There are only two reported cases of herpes infection-related pure trigeminal sen- 1. Smith JH, Cutrer FM. Numbness matters: A clinical review of sory neuropathy in which an intramedullary lesion was ob- trigeminal neuropathy. Cephalalgia 31: 1131-1144, 2011. served on MRI (3, 7, 8) (Table): one had extensive sensory 2. Gray H. 5e. The trigeminal nerve. Anatomy of the human body. 1918. disturbances involving the three divisions of the trigeminal 3. Umehara T, Oka H, Toyoda C, Mochio S. Pearls & Oy-sters: nerve; the other had dysgeusia, and was similar to our case. Trigeminal neuropathy associated with herpes labialis. Neurology Only our case had dissociated sensory impairment - in other 79: e173-e175, 2012. words, they all had disturbances of the sensations of touch, 4. Mori K, Iijima M, Koike H, et al. The wide spectrum of clinical pain and temperature, but our case is the first report of dis- manifestations in Sjögren’s syndrome-associated neuropathy. Brain 128: 2518-2534, 2005. sociated trigeminal sensory neuropathy, which was appar- 5. Farrell DA, Medsger TA Jr. Trigeminal neuropathy in progressive ently caused by HSV and in which an abnormal pontine le- systemic sclerosis. Am J Med 73: 57-62, 1982. sion was observed on MRI. Interestingly, our patient, who 6. Benatar M, Edlow J. The spectrum of cranial neuropathy in pa- showed dissociated sensory impairment, had enhanced le- tients with Bell’s palsy. Arch Intern Med 164: 2383-2385, 2004. sions not only in the pons, but also in the trigeminal nerve 7. Gominak S, Cros D, Paydarfar D. Herpes simplex labialis and trigeminal neuropathy. Neurology 40: 151-152, 1990. tract. It is unclear why the patient’s sensory impairment was 8. Suzuki N, Mizuno H, Nakashima I, Itoyama Y. Herpes labialis in dissociated; however, we speculate that only the lower root multiple sclerosis with a trigeminal lesion. Intern Med 50: 259, was damaged at the end of trigeminal nerve tract, because 2011.

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