Intravenous immunoglobulin for cranial polyneuropathy associated with Campylobacter jejuni infection.

This study reports the efficacy of i.v. immunoglobulin in a patient with cranial polyneuropathy resulting from Campylobacter jejuni infection who had high titers of serum IgG antibodies against gangliosides GD1a and GT1b in the acute phase. Treatment with i.v. immunoglobulin (400 mg/kg/day x 5 days) led to rapid partial resolution of his neurologic manifestations, but complete recovery was not obtained until 6 months later. The present case suggests that i.v. immunoglobulin therapy prevents further progression of the disease but that it may not shorten the clinical course of cranial polyneuropathy in some cases associated with Campylobacter jejuni infection.     

A case of multiple cranial neuropathy with positive antinuclear antibody responded to steroid]

A 56-year-old woman was admitted to our hospital because of bilateral ptosis, total ophthalmoplegia, bilateral facial palsy and left hypoglossal nerve palsy. Antinuclear antibody (ANA) showed high titer of 1280 but other data were normal. With oral prednisolone therapy (40 mg/day), the symptoms improved gradually but ANA titer did not show any significant change. We thought multiple cranial neuropathy of the patient was due to autoimmune disease such as collagen disease or cranial nerve type of Guillain-Barré syndrome from her symptoms and positive ANA. In contrast to other reported cases of multiple cranial neuropathy with positive ANA, in our patient ANA titer was high, only motor cranial nerves were impaired, and total ophthalmoplegia was found. Steroid therapy was useful in accordance with other reports. Although our patient has no symptoms related to collagen diseases, a long follow-up of this patient is considered to be necessary.     

A case of Cogan's syndrome associated with multiple cranial neuropathy

A 52-year-old woman was admitted to our hospital because of disturbance of right visual acuity and double vision. At 38-year-old she became deaf bilaterally and experienced many vertigo attacks. She was diagnosed as Ménière disease. At 45-year-old vertigo attacks disappeared. At 47-year-old right peripheral facial nerve palsy developed transiently with interstitial keratitis and episcleritis of the both eyes. Oral adrenocorticosteroid therapy produced an improvement of interstitial keratitis and episcleritis. On admission, ophthalmological examination revealed bilateral interstitial keratitis and episcleritis, right retrobulbar optic neuritis and she was proven to have bilateral sensorineural deafness by otologist. Neurological examination revealed right abducens nerve paresis. Laboratory examinations revealed slightly increased erythrocyte sedimentation rate. CRP was positive. Serological tests for syphilis were negative. CSF showed mildly elevated protein level. Orbital CT scans revealed the swelling of right optic nerve. Cerebral MRI showed multiple high spotty areas in left thalamus, bilateral basal ganglia and deep white matter in T2 weighted images. After treatment with adrenocorticosteroid, right optic neuritis and abducens nerve paresis improved together with bilateral interstitial keratitis and episcleritis. Multiple cranial neuropathy may develop with Cogan's syndrome.     

Multifocal motor neuropathy and Campylobacter jejuni reactivity

In some patients, Campylobacter jejuni infection has been associated with the development of multifocal motor neuropathy (MMN) and high titers of antiganglioside antibodies. The authors measured anti-C. jejuni antibodies by ELISA and immunoblot in 20 patients with MMN, and correlated their presence with antiganglioside reactivity and a history of recent diarrhea. Only one patient had high titers of anti-C. jejuni antibodies, indicating that C. jejuni is unlikely to be involved in the pathogenesis of MMN in most patients.     

A case of recurrent cranial neuropathy presenting as recurrent Tolosa-Hunt syndrome]

A 64-year-old woman, with history of hypertension and arteriosclerosis, developed left painful ophthalmoplegia in July, 1988. Neurological examination proved abnormality of the third cranial nerve innervation, otherwise normal. No systemic illness was present. With corticosteroid therapy, the symptoms regressed and completely disappeared in 3 months. In January, 1990, right painful ophthalmoplegia appeared. Neurological examination revealed involvement of right sixth nerve and first branch of the right fifth nerve. With corticosteroid therapy, the symptoms completely regressed in several weeks. In April, 1990, she developed severe pain in the right side of the face. The facial pain disappeared rapidly with corticosteroid therapy, but reappeared following quit of steroid. She complained of severe pain of the right face, the territory of first and second branch of the right fifth nerve, but neurological examination was negative. With corticosteroid therapy, the pain disappeared remaining with mild tingling sensation on the right face, but during the tapering of corticosteroid in August, a severe peripheral type right facial palsy developed. Corticosteroid therapy resumed and the facial palsy regressed almost completely in ten days. Our case suggests that THS might be a variant of so-called recurrent cranial neuropathy.     

A case of multiple cranial nerve palsy with severe dysphagia due to herpes zoster infection]

A case of multiple cranial nerve palsy by herpes zoster was reported. A 79-year-old man showed fever, sore throat, and dysphagia. No vesicle was noted at ear and pharynx. The patient developed, later, left peripheral facial nerve palsy. The cerebrospinal fluid revealed pleocytosis with increased protein. The viral antibody titer of herpes zoster was significantly elevated both in cerebrospinal fluid and in serum. The left facial palsy was slightly improved. But his dysphagia didn't improve during at least 10 months after the onset. Among the cranial nerves, trigeminal and facial nerves are the most commonly affected by herpes zoster. But there are a few cases of the 9th and 10th cranial nerve involvement in the literature. However, dysphagia has rarely been reported in these previous cases, only four cases developed severe dysphagia like the present patient. All of these cases including our case were over sixty years old, while cases with slight dysphagia were under sixty years old. No other differentiating factor is noted between these two groups with regard to sites of vesicles, findings of cerebrospinal fluid and mode of therapy.     

Serological evidence for infection with Campylobacter jejuni/coli in patients with multifocal motor neuropathy

Campylobacter jejuni/coli (CJC) infection has been implicated in the immunopathogenesis of Guillain-Barré syndrome (GBS), acute motor axonal neuropathy (AMAN), and Miller Fisher syndrome (MFS). However, its role in chronic immune mediated neuropathies such as multifocal motor neuropathy (MMN) or chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is less clear. Anti-ganglioside antibodies are associated with chronic motor neuropathies such as MMN and IgM anti-GM1, and IgM anti-asialo GM1 antibodies have been shown to cross-react with CJC lipopolysaccharides. Molecular mimicry between CJC and IgG anti-GM1 antibodies has also been suggested. Therefore we have performed a retrospective assessment of anti-CJC-specific IgG, IgM, and IgA antibodies in a cohort of seven patients with clinical and electrophysiologically definite MMN. The control group consisted of 140 healthy blood donors with no history of enteric illnesses. We found elevated titres of anti-CJC-specific IgG in 5 of 7 patients, IgM in 3 of 7 and IgA in 1 of 7. At least 1 anti-CJC antibody was elevated in 6 of 7 patients, and 3 patients had elevations of both IgG and IgM antibodies. Three patients had significantly elevated titres of anti-ganglioside antibodies without a clear relationship to the anti-CJC titres. Therefore antibodies specific for CJC were found more frequently than expected in patients with MMN. Prior or ongoing infection with CJC may play a role in the actiopathogenesis of MMN.     

Fisher's syndrome associated with Campylobacter jejuni infection

A patient developed typical features of Fisher's syndrome after an infection with Campylobacter jejuni as ascertained by positive serology both in the blood and spinal fluid. This may be the first case of this association described.     

Guillain-Barré syndrome preceded by diarrhea with the infection of Campylobacter jejuni]

We report a 77-year-old woman with Guillain-Barré syndrome following Campylobacter jejuni infection. She was admitted complaining of mild weakness in the left leg. Seven days before, she had severe diarrhea, which continued several days. After admission, the weakness soon worsened resulting in tetraparesis, and the respiration became so impaired that she was supported by the artificial ventilator. Deep tendon reflexes were absent in four limbs, and no sensory disturbance was noted. CSF contained the protein as low as 20.4 mg/dl in concentration; no pleocytosis was seen. Muscle action potentials evoked by the stimulation of nerves were markedly reduced in amplitude, but conduction velocity along the nerve was decreased mildly. Campylobacter jejuni was detected by bacterial culture of the stool; the antibody was positively raised against this bacteria in serum. By 90 hospitals days, she was restored to spontaneous respiration.     

Anti-GM1b IgG antibody is associated with acute motor axonal neuropathy and Campylobacter jejuni infection

Anti-GM1 and anti-GM1b antibodies are frequently present in patients with Guillain-Barré syndrome (GBS) and accordingly, the two antibodies often coexist in the same patient. In order to study clinical and laboratory features of anti-GM1b-positive GBS, we analyzed the data of patients with anti-GM1b IgG antibody but no anti-GM1 IgG antibody. Of 86 consecutive patients, 10 had anti-GM1b antibody alone and frequently had acute motor axonal neuropathy (AMAN, 80%) and Campylobacter jejuni infection (60%). Of 10 patients with anti-GM1 antibody alone, four had AMAN, and two had C. jejuni infection. These results showed that GM1b could be a target molecule of autoantibody in the AMAN form of GBS subsequent to C. jejuni infection.     

A case of herpes zoster associated with multiple cranial nerve involvement]

A 74-year-old man who had suffered from right herpes zoster ophthalmicus developed ipsilateral multiple cranial nerve involvement two weeks later. He showed right visual disturbance, total ophthalmoplegia and peripheral facial palsy. Pleocytosis and increased protein were found in CSF. Titer of VZV antibody increased in serum and CSF. CT and MRI demonstrated no abnormal findings in the brain stem. Within a month, peripheral facial palsy improved. Severe extra-ophthalmoplegia began to improve after three months, and moderately recovered. After two and a half year, visual disturbance and mydriasis showed no improvement. In this case, we speculate that localized leptomeningitis caused multiple cranial nerve involvement.     

A case of acute sensory neuropathy associated with cytomegalovirus infection]

We report a non-compromised patient with acute sensory neuropathy (ASN) developed following cytomegalovirus (CMV) hepatitis. A 67-year-old man admitted to a hospital because of acute hepatic dysfunction accompanying fever and skin eruption. One month later, when hepatic function normalized, numbness and clumsiness started acutely first in the right upper limb next to all the extremities. He found difficulty in walking in a couple of weeks. One month after the commencement of neurological illness, he was referred to us. On examination, he had sensory limb ataxia. His gait was wide-based, and Romberg sign was positive. Position sense was severely diminished in the extremities. Skin sensation was also attenuated distally, while no motor weakness was noted. Tendon reflexes were almost absent. Nerve conduction studies revealed absent sensory potentials in all but the left median nerve, in which amplitude was 5.5 microV with sensory conduction velocity of 40.7 m/s. Motor conduction studies, on the other hand, appeared normal except for a slight focal delay in the right ulnar nerve across the elbow. Mild increase in F-wave latencies was noted. A sural nerve specimen taken two months after the neurological onset showed a marked decrease in myelinated fiber density and active fiber degenerations accompanying axonal sproutings. Sj?gren syndrome and paraneoplastic neuropathy were excluded serologically and by comprehensive imaging techniques. Although IgM anti-CMV antibody was not detected, serum IgG anti-CMV antibody was positive and significantly increased during the neurological illness. The intrathecal antibody synthesis of IgG anti-CMV antibody was suggested by a low serum/cerebrospinal fluid (CSF) antibody ratio and a high CSF IgG index. From these observations, it was strongly suggested that acute hepatitis and subsequent ASN were associated with CMV infection in this patient. Although some cases with post-infectious ASN have been previously reported, this is the first report of ASN preceded by CMV infection.     

A case of multiple sclerosis associated with myelin associated glycoprotein neuropathy]

A 28-year-old woman had developed chronic, recurrent, visual disturbance (bilateral), and girdle sensation at Th 5-6. She was admitted to our hospital because of left visual disturbance, distal limb weakness on right side, and numbness of four extremities. The neurological examination revealed decreased visual acuity of the left eye with abnormality of the optic disk, moderate muscle weakness of the right upper and lower extremities, absent tendon reflexes and paresthesia on distal portions of the four limbs. Laboratory examinations disclosed the titration of anti-myelin associated glycoprotein (MAG) antibody (IgM) and CSF protein was elevated (104 mg/dl). Motor nerve conduction studies revealed conduction block in more than one nerve. The conduction velocities in the upper and lower extremities were all diminished. P100 latency was prolonged by flash visual evoked potential (VEP) studies. N13-N20 interpeak latency of somatosensory evoked potential (SEP) of median nerve was also prolonged. She was treated with steroid pulse therapy, followed by an oral dose of 30 mg/day of prednisolone. Her symptoms resolved completely three months later, and multifocal conduction block subsided on electrophysiological study. There are some cases of multiple sclerosis with multifocal conduction block, but such a case is very rare in Japan. We discussed the pathogenic mechanisms of these conditions, and we conclude that we must take notice of demyelinating neuropathy in multiple sclerosis and that nerve conduction studies are useful for detecting them.     

A case of Ramsay Hunt syndrome associated with local meningitis, multiple cranial neuropathy, and the second cervical nerve involvement]

A 76-year-old man with herpetic vesicle in the right auricle developed ipsilateral 5th, 6th, 7th, and 8th cranial nerves involvement and pain in the dermatome of the second cervical nerve. The CSF study revealed elevated opening pressure up to 220 mmH2O, and pleocytosis up to 151 cells/mm3. Ninety-nine percent of the CSF cells were mononuclear cells. CSF protein was 47 mg/dl, and CSF glucose was 62 mg/dl. On the 24th hospital day the CSF cells decreased to 13/mm3 with 100% mononuclear cells. Titer of varicella-zoster virus (VZV) antibody was significantly elevated in CSF. Brain MRI and ABR demonstrated no abnormality. Although disorders of 5th and 6th cranial nerves and second cervical nerve improved, mild facial nerve palsy lasted and hearing disturbance showed no recovery. There are only seven cases of Ramsay Hunt syndrome associated with external ophthalmoplegia in the literature. However, un like the present case, none of these cases presented disorders of upper cervical nerves. In this case, we speculate that spreading of reactivated VZV caused local meningitis and multiple cranial nerve involvement as well as the second cervical nerve.