Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic recurrent acquired immune-mediated disease of the peripheral nerves that presents with progressive sensory and motor deficits in all four limbs. Cranial nerve involvement is not as common as in Guillain-Barre syndrome, and central nervous system involvement including optic neuritis has rarely been reported in patients with CIDP. We recently experienced a case with classic CIDP involving bilateral facial and trigeminal nerves, right lower cranial nerves, and the right optic nerve.

Facial diplegia (FD) rarely occurs as a regional Guillain-Barré syndrome (GBS) variant. A 70-yearold male presented with bifacial weakness that had started on the left side and extended to the right after several days. He was then treated using steroids and gradually improved. Serum antiganglioside antibody testing revealed positivity for anti-GM1 IgG antibodies. FD can be idiopathic, but it is an uncommon GBS variant. The ganglioside antibody test may increase the possibility of diagnosing isolated FD.

Guillain-Barré syndrome (GBS) and Miller Fisher syndrome (MFS) can present with overlapping features. A 56-year-old female developed ptosis and diplopia after an upper respiratory infection, and presented with facial palsy, dysarthria, brachial weakness, ataxia, and areflexia. Mild weakness of both legs appeared after a few days. Anti-ganglioside complex antibody were positive to IgG GM1/GQ1b and GQ1b/sulfatide antibodies. The present case suggests that the manifestation of overlap between MFS/PCB variants and GBS could be caused by antiganglioside complex antibodies.

Citations

• Overlap of Miller-Fisher Syndrome and Pharyngeal-Cervical-Brachial Variant Secondary to COVID-19 in Recurrent Guillain-Barré Syndrome: A Case Report
  Tarek Hammad, Sayeed Hossain, Amin Alayyan
  Cureus.2024;[Epub]     CrossRef

Reversible conduction block (RCB) was rare in patients with acute motor sensory axonal neuropathy (AMSAN). A-46-year-old man presented with paresthesia, weakness, diplopia, and dysarthria. Nerve conduction study (NCS) exhibited axonal changes with conduction block in motor and sensory nerves. His symptoms were rapidly progressed and recovered. Conduction block was disappeared in the follow-up NCS performed after 2 weeks. The AMSAN case with RCB showed rapid progress and rapid recovery of clinical symptoms as acute motor axonal neuropathy patients with RCB.

Citations

• Atypical Asymmetric Guillain-Barré Syndrome Overlapping with Miller-Fisher Syndrome: A Case Report
  Hwi Jung Kim, Gyu Seok Oh, Ju Kang Lee, Oh Kyung Lim, Ki Deok Park
  Journal of Electrodiagnosis and Neuromuscular Dise.2021; 23(2): 49.     CrossRef

Background
Although postural instability is one of the major symptoms of Parkinson’s disease (PD), dopaminergic treatment is ineffective for treating postural instability. Recent reports have shown that somatosensory deficit is associated with postural instability, and that somatosensory input improved postural instability. The purpose of this study is to evaluate the effects of lateral wedges for quiet standing postural control in people with PD.
Methods
Twenty-two patients who were diagnosed with PD were enrolled in this study. The participants stood on a force plate under two conditions (wedge and no wedge) with or without having their eyes open or closed. The center of pressure (COP) range and velocity were analyzed using a two-way repeated-measures analysis of variance.
Results
The range and velocity of COP in the anterioposterior and mediolateral (ML) directions were significantly improved after the patients stood on the lateral wedge with their eyes closed (p < 0.05). The range in ML direction and velocity in both directions of COP were significantly decreased when their eyes were open (p < 0.05).
Conclusions
Regardless of vision, standing on lateral wedges improved postural sway in people with PD.

Background: The aim of this study was to investigate the predictive value of snoring frequency in the diagnosis and severity of obstructive sleep apnea. Methods: Patients who underwent polysomnography with one or more of the following characteristics were included: 1) sleepiness, non-restorative sleep, fatigue, or insomnia symptoms; 2) arousal due to cessation of breathing or the occurrence of gasping or choking when waking up; and 3) habitual snoring, breathing interruptions, or both, noted by a bed partner or other observer. We analyzed the differences in clinical and polysomnographic variables between patients with and without obstructive sleep apnea and investigated the associations of those variables with obstructive sleep apnea severity. Results: One hundred ninety-three patients met the inclusion criteria, and 145 of the 193 patients were diagnosed with obstructive sleep apnea. Multiple logistic regression analysis showed that large neck circumference (p = 0.0054) and high snoring index (p = 0.0119) were independent predictors for obstructive sleep apnea. Moreover, between the obstructive sleep apnea severity groups, there was a strong tendency of difference in body mass index (p = 0.0441) and neck circumference (p = 0.0846). However, there was no significant difference in snoring frequency according to obstructive sleep apnea severity (p = 0.4914). Conclusions: We confirmed that snoring frequency is a predictor of obstructive sleep apnea. In addition, we showed for the first time that snoring frequency is not associated with obstructive sleep apnea severity, thus it is not a valuable marker for predicting obstructive sleep apnea severity.

Citations

• Validity Analysis of Neck Circumference as a Screening Test for Hypoxia Occurrence in Patients Undergoing Sedative Endoscopy
  Hyun-Ji Song, Jiyun Kim
  Healthcare.2022; 10(4): 679.     CrossRef

Background
Viruses can cause either meningitis or encephalitis. It is unclear why some people suffer from aseptic meningitis, and others acquire aseptic encephalitis when infected with the same viral pathogens. The aim of this study was to compare demographic and laboratory factors between patients with aseptic meningitis and encephalitis. Methods: The demographic and laboratory differences were analyzed according to age, sex, diabetes, hypertension, C-reactive protein in the blood, white blood cell and protein in the cerebrospinal fluid, and glucose ratio (cerebrospinal fluid/blood). Additionally, we analyzed the nation-wide differencesin age between the patients with aseptic meningitis and those with encephalitis in Korea. Results: The patients with aseptic encephalitis were older, more likely to have hypertension, and had higher levels of C-reactive protein than did the patients with aseptic meningitis. However, the numbers of white blood cells in the cerebrospinal fluid were significantly higher in the patients with meningitis than in the patients with encephalitis. Multivariable analysis revealed that age >49 years, hypertension and a C-reactive protein level >5.81 mg/dL were independent and significant variables in the prediction of aseptic encephalitis. Additionally, the patients with aseptic encephalitis were older than those with aseptic meningitis in the nation-wide Korean database. Conclusions: Older age, hypertension, and higher levels of C-reactive protein are useful factors for the prediction of aseptic encephalitis.

A 77-year-old man developed acute vertigo and unsteady gait. Neurological examination revealed spontaneous left-beating nystagmus in the primary position. He fell to the left when walking without support. Magnetic resonance imaging showed an acute infarction involving the right parieto-temporal lobe. Although the vertigo and unsteady gait are most often associated with vestibular disorders involving the infratentorial structures, those may occur in cerebral infarction of the parieto-temporal lobe.

We encountered a case of pituitary apoplexy who presented with isolated headache and vomiting without visual disturbance or ophthalmoplegia. The cerebrospinal fluid examination was compatible with aseptic meningitis. A computed tomography revealed slightly high density in the pituitary fossa and suprasella area, but the signal change was very faint. Our case suggests that clinicians should take into account the possibility of pituitary apoplexy without visual disturbance or ophthalmoplegia, when aseptic meningitis is suspected.

It was sometimes difficult to differentiate between acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) and subacute inflammatory demyelinating polyneuropathy (SIDP). The CNS involvement of these polyneuropathies has rarely reported in the literature. We present the case of a 42-year-old man who developed rapidly developing inflammatory demyelinating polyneuropathy followed by right optic neuritis. This case showed progressive motor weakness and sensory dysfunction with time to nadir at 8 weeks, demyelination in nerve conduction study, no other etiology of neuropathy, no relapse during follow-up of 18 months, good response to steroid and complete recovery which favor SIDP more than A-CIDP. We experienced the case of SIDP associated with optic neuritis.

Background
Transcranial magnetic stimulation (TMS) is a non-invasive diagnostic method particularly suited to investigation the long motor tracts. The clinical value of TMS in most spinal cord diseases has still to be made. Diagnostic value of magnetic motor evoked potential (MEP) parameters in intramedullary spinal cord lesions was investigated.
Methods
MEP elicited by TMS was recorded in 57 patients with clinically and radiologically defined intramedullary myelopathy. Twenty five patients with cervical myelopathy (CM) and 32 thoracic myelopathy (TM) were included. Recordings were performed during resting and minimal voluntary contraction at both abductor pollicis brevis (APB) and tibialis anterior (TA) muscles. Stimulation threshold(ST), amplitude, and central motor conduction time (CCT) were measured at resting and facilitated conditions. CCT was calculated by two means; central motor latency (CML)-M using magnetic transcranial and root stimulation, and CML-F using electrical F-wave study. The results were compared between patient groups and 10 normal control group.
Results
Facilitated mean ST recorded at TA was elevated in both CM and TM compared with control group. Resting mean CML-M at TA was significantly prolonged in both CM and TM, and CML-M was absent or delayed in 37.1% of CM and 8% of TM at APB with facilitation. Facilitated mean MEP amplitude at ABP was lower in CM than in TM, while MEP/M ratios were not different significantly between groups.
Conclusions
Magnetic motor evoked potential has diagnostic value in intramedullary myelopathy and localizing value in differentiating between CM and TM by recording at APB and TA. It is a noninvasive way to investigate the functional status of motor tracts of spinal cord.

Ischemic monomelic neuropathy and myopathy are rare complications of peripheral arterial occlusive disease. We reporta case of ischemic monomelic neuropathy of the right sural, common peroneal and posterior tibial nerves and ischemic myopathyof the right tibialis anterior resulting from the occlusion of the right common femoral arteries despite successfulrevascularization. Ischemic monomelic neuropathy and myopathy can occur as a result of occlusion of the specific peripheralartery.

Progressive systemic sclerosis (PSS) is a multi-systemic disorder characterized by abundant fibrosis of the skin, blood vessels, and visceral organs. But it rarely affects the peripheral nervous system. We report a 36-year-old man of painful trigeminal neuropathy as a complication of PSS. He was referred from Rheumatology for the evaluation of abruptly developed bilateral facial pain. He had facial hyperesthesia and paresthesia on neurologic examinations. In the blink reflex, ipsilateral and contralateral R1 and R2 responses were not detected during bilateral supraorbital stimulation. But normal latency and CMAP amplitude of facial NCV were found. Under the impression of trigeminal neuropathy caused by PSS, steroid therapy was tried, and his clinical symptoms and electrophysiologic findings were improved. PSS could be the cause of the painful trigeminal neuropathy.

Underlying neuropathies combined with amyotrophic lateral sclerosis (ALS) cast doubt on the diagnosis of ALS when present.Abnormal sural nerve conductions were found in 3 patients with clinically definite ALS. Pathologically demyelinating, axonal, or vasculitic neuropathy was suggested respectively. High dose oral corticosteroid had no effect and clinical courses were deteriorating in all the patients.The causes of combined neuropathies were unclear. Possibility of direct consequence of ALS, concomitant neuropathies, or rare variants of ALS should be considered in these cases.