Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin.

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder and both clinical course and response to treatment vary widely. Because of the propensity for relapse, CIDP requires maintenance therapy after the initial response to treatment. There is no consensus regarding this in the published literature. PRESENT REPORT: A patient with CIDP was treated with oral prednisolone and cyclophosphamide pulse therapy but required repeated plasma exchange and intravenous immunoglobulin (IVIg). Treatment with ciclosporin freed the patient from repeated IVIg administration. Therapeutic responses in 14 subsequent cases including three patients who showed improvement with ciclosporin are also presented along with an algorithm of the authors' suggested protocol for treatment. CONCLUSION: Ciclosporin should be considered for patients with intractable CIDP who require repeated IVIg.

Acute motor axonal neuropathy after Mycoplasma infection: Evidence of molecular mimicry.

BACKGROUND: Patients with Guillain-Barré syndrome (GBS) after Mycoplasma pneumoniae infection often have antibodies to galactocerebroside (GalC). Electrodiagnosis may show acute inflammatory demyelinating polyneuropathy (AIDP). METHODS: The authors report a patient with acute motor axonal neuropathy (AMAN) after Mycoplasma infection and review seven cases of Mycoplasma-associated GBS. They investigated anti-GalC serology under various conditions associated with Mycoplasma infection. RESULTS: The patient had immunoglobulin (Ig)G and IgM antibodies against GM1 and GalC, which cross-reacted. During the acute phase, IgM selectively immunostained axons. The cholera toxin B-subunit and rabbit anti-GM1 IgG stained a band in the lipid extract from M pneumoniae, indicative of the presence of a GM1 epitope. Six Mycoplasma-associated GBS patients with anti-GalC antibodies had non-AIDP electrodiagnoses, whereas one with Mycoplasma-associated AIDP had no anti-GalC antibodies. Anti-GalC antibodies were positive in two of five patients who had neurologic diseases other than GBS after Mycoplasma infection and in one of 12 who had acute respiratory disease caused by M pneumoniae not followed by a neurologic disease. CONCLUSIONS: Anti-GalC antibodies in Mycoplasma-associated GBS may be an epiphenomenon. In certain cases, anti-GM1 antibodies induced by molecular mimicry with M pneumoniae may cause acute motor axonal neuropathy.

Acute facial diplegia and hyperreflexia: A Guillain-Barré syndrome variant.

Two patients with acute facial diplegia and hyperreflexia are described. Both patients had serologic evidence of preceding Campylobacter jejuni infection and antiganglioside IgG antibodies as well as other laboratory and electrophysiologic findings suggesting Guillain-Barré syndrome (GBS). IV immunoglobulin produced recovery. Hyperreflexia does not necessarily exclude the diagnosis of a GBS variant. Antiganglioside antibodies can help with diagnosis in difficult cases.

Overlapping Guillain-Barré syndrome and Bickerstaff's brainstem encephalitis associated with anti-GQ1b IgG antibody after herpes simplex virus infection.

Herpes simplex virus (HSV) is a rare, antecedent infectious agent in Guillain-Barré syndrome (GBS). We report a patient with overlapping GBS and Bickerstaff's brainstem encephalitis (BBE). The patient had a vesicular lesion on her nose. Antecedent HSV type 1 (HSV-1) infection was confirmed by isolation of the virus and detection of the presence of serum anti-HSV-1 IgM antibody during the acute phase. Her serum IgG had high anti-GQ1b antibody titer. External ophthalmoplegia has been noted in 2 of 4 reported cases of HSV-associated GBS. Herpetic brainstem encephalitis cases of poor prognosis are known, but only 2 cases of benign brainstem encephalitis secondary to HSV infection, in which there was acute ophthalmoplegia and clinical features consistent with those of BBE have been reported.

Animal model of axonal Guillain-Barré syndrome induced by sensitization with GM1 ganglioside.

Some humans develop the axonal form of Guillain-Barré syndrome after receiving bovine brain ganglioside. On sensitization with the ganglioside mixture, all of a group of rabbits injected developed high anti-GM1 IgG antibody titers, flaccid limb weakness of acute onset, and a monophasic illness course. Pathological findings for the peripheral nerves showed predominant Wallerian-like degeneration, with neither lymphocytic infiltration nor demyelination. IgG was deposited on the axons of the anterior roots, and GM1 was proved to be present on the axons of peripheral nerves. Sensitization with purified GM1 also induced axonal neuropathy, indicating that GM1 was the immunogen in the mixture. A model of human axonal Guillain-Barré syndrome has been established that uses inoculation with a bovine brain ganglioside mixture or isolated GM1. This model may help to clarify the molecular pathogenesis of the syndrome and to develop new treatments for it.

Fine specificity of anti-GQ1b IgG and clinical features.

Anti-GQ1b IgG frequently is present in sera of patients with Miller Fisher syndrome (MFS), Guillain-Barré syndrome (GBS) with ophthalmoplegia, Bickerstaff's brainstem encephalitis (BBE), and acute ophthalmoparesis (AO) in the acute phase. Why various clinical signs develop under these conditions, however, has yet to be clarified. We investigated the fine specificity of anti-GQ1b IgG and its clinical correlation in sera from 82 patients: 56 with MFS, 11 with GBS, 13 with BBE, and 2 with AO. Anti-GQ1b IgG antibodies were absorbed by GT1a in 80 (98%) of the 82 sera, by GD1b in 11 (13%), and by the other b-series gangliosides GD3, GD2, or GT1b in 24 (29%). The most frequent pattern of fine specificity was the cross-reaction with GT1a alone, seen in 56 (68%) samples. Of the 11 patients with anti-GQ1b IgG, cross-reacting with GD1b, 6 (55%) had impaired deep sense, and the association was significant (p=0.02). This is the first study to show that the fine specificity of anti-GQ1b IgG is heterogeneous and that the difference is correlated with the presence of a particular clinical sign.

Features of sensory ataxic neuropathy associated with anti-GD1b IgM antibody.

Some reports have called sensory ataxic neuropathy (SAN) associated with IgM antibody against b-series gangliosides a chronic form of Miller Fisher syndrome (MFS), but this has yet to be established. We examined five patients with SAN and eight patients with IgG anti-GQ1b-positive MFS. Only one patient with SAN complained of diplopia, whose ocular movement was not limited. The other four patients had neither diplopia nor limitation of ocular movement. All the SAN patients had severe deep sense impairment, whereas one patient with MFS showed only mild vibratory sense impairment. All sera from the SAN patients had remarkably high IgM antibody titers to the b-series gangliosides GD3, GD2, GD1b, GT1b, GQ1b, GQ1b alpha, fucosyl-GD1b, and alpha galactosyl [alpha fucosyl] GD1b. An absorption study confirmed that the anti-GQ1b antibodies cross-reacted with GD3, GD2, GD1b, and GT1b. In contrast, only two samples from the MFS patients had IgG antibody to GD3, and no sample reacted with GD2, GD1b, or GT1b. SAN has different clinical or serological features from MFS, and therefore is not a chronic form of it.

[Ataxic form of Guillain-Barré syndrome associated with anti-GD1b IgG antibody].

A 28-year-old man was admitted after developing acute onset unstable gait following acute enteritis. Neurological examination revealed mild weakness in four limbs, areflexia and ataxia. Serum obtained from the patient during the acute stage contained a high titer of anti-GD1b IgG antibody. Because the patient showed obvious cerebellar ataxia unrelated to muscle weakness, without ophthalmoplegia or proprioceptive sensory disturbance, we concluded that he had ataxic form of Guillain-Barré syndrome (GBS) (Richter, 1962). Although ataxic GBS is not an established conception, one should pay attention to the possible existence of such a rare GBS variant. It is necessary to accumulate additional case reports to clarify the association between ataxic GBS and anti-ganglioside antibodies.

[Delayed radial nerve palsy following a humeral shaft fracture].

We reported a 24-year-old man with right radial nerve palsy. He had suffered from a right humeral shaft fracture approximately three years before. The fracture was treated with intramedullary nailing. Our radiograph of the right upper limb showed callus around the fracture site. A nerve conduction study revealed conduction block of the radial nerve at the lateral side of the callus. Tinel's sign was present in the median nerve at the medial side of the callus. Surgical exploration revealed that the radial nerve trunk was compressed at the callus site. The median nerve trunk was close to the callus. We decompressed radial nerve trunk, and the patient's neurological symptoms improved gradually after the operation. Delayed radial nerve palsy has been reported only rarely, whereas acute compression of the nerve sometimes occurs after humeral shaft fractures. Morever, the median nerve also was vulnerable to compression at the site of callus. These findings underscore the importance of taking into account the possibility of delayed compression neuropathy after humeral shaft fracture.

Ataxic Guillain-Barré syndrome with anti-GQ1b antibody: relation to Miller Fisher syndrome.

The authors reviewed the medical records of seven patients with anti-GQ1b immunoglobulin G (IgG) who had no or minimal external ophthalmoplegia but showed ataxia. The clinical features of the patients were consistent with the ataxic form of Guillain-Barré syndrome (GBS). Anti-GQ1b IgG antibodies from the patients, as well as from those with Miller Fisher syndrome (MFS), were absorbed by GT1a. The finding that ataxic GBS and MFS have in common an autoantibody with the same fine specificity suggests that they form a continuous spectrum.

Epidural compression of the cauda equina caused by vertebral osteoblastic metastasis of prostatic carcinoma: resolution by hormonal therapy.

A 59 year old man with prostatic carcinoma developed epidural compression of the cauda equina caused by bony expansion from a vertebral osteoblastic metastasis. For medical reasons he could not undergo radiation or surgery. Hormonal therapy alone relieved his low back pain and restored ambulation and urinary function. Postmyelography CT showed that the bony expansion from the vertebra had completely disappeared after treatment. This is the first report of remarkable improvement due to hormonal therapy alone.