Determining C5, C6 and C7 myotomes through comparative analyses of clinical, MRI and EMG findings in cervical radiculopathy.

OBJECTIVE: There are many myotome charts in the literature, but few studies have presented actual data to support their identification. We aimed to determine C5/C6/C7 myotomes based on clinical and EMG data of patients with cervical spondylotic radiculopathy (CSR) having a single-root lesion confirmed by MRI. METHODS: Medical Research Council (MRC) scores and EMG findings were retrospectively reviewed for patients enrolled from our EMG database. RESULTS: Enrolled were 25 patients (10 C5, 6 C6, and 9 C7 CSR). In C5 CSR, weakness or denervation potentials in EMG, or both, were observed in the deltoid (Del) and infraspinatus (Isp) muscles for all patients, and in the biceps brachii (BB) and brachioradialis (BR) muscles for 9/10 and 8/9 patients, respectively. In C6 CSR, weakness of the wrist extensor and/or denervation of the extensor carpi radialis longus (ECRL)/extensor carpi radialis brevis (ECRB), and those of the pronator teres (PT) were observed for all patients. Weakness was not observed for any other muscle in C6 CSR. Denervation potentials of ECRL were found in 5/8 and 3/5 patients with C5 and C6 CSR, respectively, whereas those of ECRB were found in 1/5, 6/6, and 2/5 patients with C5, C6 and C7 CSR, respectively. In C7 CSR, weakness/denervation of the triceps brachii (TB) and denervation potentials of the flexor carpi radialis (FCR) were observed for all patients. Denervation potentials in PT and weakness/denervation of the extensor digitorum (ED) were observed in 2/9 and 4/9 patients, respectively. CONCLUSION: Suggested dominant myotomes are: C5 for the Del, Isp, BB, and BR, C5/6 for the ECRL, C6 > C7 for the ECRB and PT, and C7 for the TB and FCR. SIGNIFICANCE: The current study identified dominant myotomes that differ from the existing literature.

[Twenty-year follow-up study of antiglycolipid antibodies and electrophysiological findings in a 36-year-old patient with an axonal variant of Guillain-Barré syndrome].

We describe a twenty-year follow-up study of antiglycolipid antibodies and electrophysiological results in a 36-year-old man with Campylobacter jejuni-associated Guillain-Barré syndrome (GBS). The patient had a high titer of IgG antibodies to GM1 and GA1 20 years ago. Plasma exchange resulted in full recovery from a bedridden status to independent walking in three weeks, except for residual mild weakness of the bilateral extensor hallucis longus muscles and atrophy of the plantar muscles. Twenty years later, he is unable to run at full pace due to neurological sequelae, and IgG antibodies to GM1 and GA1 were still slightly positive. This case suggests that marked improvement in the acute phase does not necessarily guarantee a subsequent good quality of life (QOL). Optional treatment such as complement inhibitors in the acute phase may be required to achieve better QOL in subsets of patients with GBS.

An Atypical Case of Anti-NMDA Receptor Encephalitis: Predominant Parkinsonism and Persisting Micrographia without Oro-facial Dyskinesia.

We describe the case of a 46-year-old man with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis with prominent parkinsonism. The patient presented with psychiatric symptoms followed by epileptic seizure and parkinsonism including micrographia. Magnetic resonance imaging (MRI) revealed lesions in the bilateral medial temporal lobes and basal ganglia on fluid-attenuated inversion recovery images. His symptoms and MRI findings were ameliorated by immunotherapy but then relapsed. After retreatment, his parkinsonism gradually improved except for the micrographia. This is an atypical case of anti-NMDAR encephalitis in that the patient showed prominent and refractory parkinsonism, thus indicating that the clinical diversity of anti-NMDAR encephalitis is greater than expected.

Refractory status epilepticus caused by anti-NMDA receptor encephalitis that markedly improved following combination therapy with rituximab and cyclophosphamide.

We herein describe the case of a 48-year-old woman who presented with nonconvulsive status epilepticus refractory to antiepileptic drugs caused by anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis without any tumors. She developed nausea and psychiatric symptoms, followed by fever and an acute progressive disturbance of consciousness. On admission to our hospital, she presented with involuntary orofacial movements and central hypoventilation, and an electroencephalogram showed a generalized slow activity consistent with nonconvulsive status epilepticus. The patient's drug-resistant status epilepticus markedly improved following second-line immunotherapy with rituximab and cyclophosphamide. Physicians should consider the early initiation of second-line therapy in certain cases of anti-NMDAR encephalitis.

[Case of wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome with cerebellar ataxia and facial dysesthesia].

We report an 85-year-old man presenting with wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome with cerebellar ataxia and facial dysesthesia. He experienced an abrupt onset of double vision and exotropia of the right eye with unsteady gait and dysesthesia around upper lip. He was admitted to our hospital ten days after the onset of the double vision. On admission, he presented with WEBINO, left limb ataxia, and dysesthesia around upper lip on the right side. His exotropia was prominent on the right side. Diffusion weighted images of MRI revealed a high intensity lesion in the paramedian pontine tegmentum involving bilateral medial longitudinal fasciculus (MLF), consistent with acute ischemic lesion. Four months after the onset, the WEBINO persisted, without cerebellar ataxia and facial dysesthesia. Putative lesions of the WEBINO, cerebellar ataxia and facial dysesthesia were bilateral MLF, left superior cerebellar peduncle and trigeminothalamic tract, respectively, which were broader than the MRI lesion. Neurological examination is critical for evaluation of accurate ischemic area.

A-waves in Guillain-Barré syndrome: correlation with electrophysiological subtypes and antiganglioside antibodies.

OBJECTIVE: To investigate the relationship of A-waves with conventional electrophysiological subtypes of Guillain-Barré syndrome (GBS), as well as with anti-ganglioside antibodies. METHODS: The subjects consisted of 30GBS patients who were classified into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy, and unclassified based on the results of nerve conduction studies. "Abundant A-waves" were defined for the upper-limb nerves (median and ulnar nerves) using receiver-operator characteristic curves. The presence or absence of IgG anti-ganglioside antibodies was also noted. RESULTS: Abundant A-waves at weeks 3-6 from onset were observed in 64% of the 14 AIDP patients and 0% of 16 non-AIDP patients, and in 60% of 15 antibody-negative patients and 0% of 15 antibody-positive patients. In the earlier period, this relationship was less clear. The correlation between the conventional electrophysiological subtypes and antibodies was present, but was much weaker. CONCLUSIONS: Abundant A-waves in GBS after the acute phase were strongly associated with demyelination that was not mediated by antiganglioside antibodies, possibly through the mechanism of proximal re-excitation induced by electrical inhomogeneities due to segmental demyelination. SIGNIFICANCE: Abundant A-waves are promising as a novel reliable marker of demyelination.