Chronic Inflammatory Demyelinating Polyneuropathy Combined With Limited Cutaneous Systemic Sclerosis

Chronic inflammatory demyelinating polyneuropathy (CIDP) has been rarely reported in systemic sclerosis (SSc). We describe the clinical, electrophysiological, and pathologic findings in a 73-year-old female with established limited cutaneous SSc who later developed CIDP. The patient had progressive limb weakness, sensory loss, and slow nerve conduction velocities. Sural nerve biopsy showed evidence of demyelination and remyelination. This case demonstrates that chronic demyelinating polyneuropathy can be an unusual manifestation of SSc, presumably resulting from an immune-mediated process.

Acute Peripheral Neuropathy, as One of Early Manifestations in Crohn's Disease

Acute neuropathic complications rarely develop in patients with inflammatory bowel disease. A case of acute peripheral neuropathy that developed only 4 weeks after a diagnosis of Crohn's disease (CD) is reported herein. Peripheral neuropathy can be a rare extraintestinal manifestation of CD. Early recognition and proper management of the neurologic complications are crucial for preventing the associated morbidity.

A Case of Postural Orthostatic Tachycardia Syndrome Associated with Migraine and Fibromyalgia

Postural orthostatic tachycardia syndrome (POTS) refers to the presence of orthostatic intolerance with a heart rate (HR) increment of 30 beats per minute (bpm) or an absolute HR of 120 bpm or more. There are sporadic reports of the autonomic nervous system dysfunction in migraine and fibromyalgia. We report a case of POTS associated with migraine and fibromyalgia. The patient was managed with multidisciplinary therapies involving medication, education, and exercise which resulted in symptomatic improvement. We also review the literature on the association between POTS, migraine, and fibromyalgia.

Cervical Myelopathy Resulting From Non-Rheumatoid Atlantoaxial Subluxation

Degenerative pannus arising from the atlanto-axial articulation is a rare entity, which is associated with chronic atlanto-axial instability in degenerative arthropathies. Due to cervical instability and the mass effect on the spinal cord, it can produce severe neck pain and compressive myelopathy. We report a case of cervical myelopathy resulting from non-rheumatoid atlanto-axial subluxation. The posterior stabilization provided excellent neurological improvement and pannus regression. Early recognition of this condition is important as the clinical condition will deteriorate without surgical interventions.

Moraxella Meningoencephalitis: Case Report and Review of the Literature

Moraxella is an aerobic, Gram-negative coccobacillus, which is rarely associated with serious and invasive infections. Because of its rarity, the clinical significance and appropriate therapy for infections due to Moraxella are not well understood. We report a case of meningoencephalitis caused by Moraxella. The patient presented with fever and confusion and was successfully treated with beta-lactam and aminoglycoside antibiotics. We also review 8 previously published cases of Moraxella meningitis or meningoencephalitis.

Development of Korean Neuropathic Pain Questionnaire for Neuropathic Pain Screening and Grading: A Pilot Study

BACKGROUND: The pain-screening questionnaire is a self-reported description of the intensity and nature of pain. This study aimed to develop the Korean Neuropathic Pain Questionnaire (KNPQ) and to assess its reliability and validity regarding the diagnosis of neuropathic pain. METHODS: Four screening tools and two rating scales were translated and modified to develop the preliminary KNPQ. Following a development phase and a pilot study, we generated the final 25-item version of the KNPQ. Each item was rated on a numerical scale of 0-10. The validation procedure was performed in 62 patients with neuropathic pain (21 with central pain and 41 with peripheral pain) and in 34 patients with nonneuropathic pain. The internal consistency between items was assessed to determine the reliability of the KNPQ, and its concurrent validity was determined by evaluating the relationship between the Visual Analogue Scale (VAS) and KNPQ scores. RESULTS: The KNPQ was not influenced by age, sex, or pain duration. The 25-item questionnaire demonstrated high internal consistency. The total score of the KNPQ was correlated with the global pain intensity on a VAS. These items were able to differentiate neuropathic pain from nonneuropathic pain with a sensitivity of 84% and a specificity of 44% (when using a cut-off point of 46). CONCLUSIONS: The newly developed KNPQ may be used for the initial screening of neuropathic pain patients. However, it cannot be used to differentiate central neuropathic pain from peripheral neuropathic pain.

Piriformis Syndrome Following Transarterial Embolization of Bilateral Uterine Arteriovenous Malformation

Piriformis syndrome is a rare entrapment neuropathy in which the sciatic nerve is compromised by the piriformis muscle or other local structures. We report a case of sciatic and gluteal nerve lesions with infarction of the piriformis muscle following internal iliac artery embolization of the bilateral uterine vascular malformation. The surgical intervention revealed anatomical variation of the right sciatic nerve. To our knowledge, this is the first case of piriformis syndrome following endovascular treatment of uterine vascular malformation in Korea.

Platelet Function Assay for Clopidogrel and Ticlopidine in Patients With Ischemic Stroke

BACKGROUND: The rapid platelet function assay (RPFA) has recently been developed and used to monitor the antiplatelet effects on the P2Y12 ADP receptor. We describe the platelet response to clopidogrel and ticlopidine using the RPFA and identify the clinical factor related to laboratory resistance in patients with ischemic stroke. METHODS: Of the 172 outpatients with ischemic stroke or transient ischemic attack (TIA) enrolled in this study, 86 were taking clopidogrel (75 mg/day) and 86 were taking ticlopidine (500 mg/day). Demographic data, vascular risk factors, stroke subtypes, and the results of blood tests were recorded. Inhibition is described as the percentage change from baseline aggregation, and is calculated from the P2Y12 reaction unit (PRU) and the base PRU on the RPFA. Those patients who displayed ineffective aggregation-inhibition (inhibition <20%) on the RPFA were defined as nonresponders. RESULTS: The response of platelet aggregation-inhibition to clopidogrel and ticlopidine exhibited a variable distribution (PRU; coefficient of variability, 0.477). Ineffective platelet inhibition was detected in 25.6% of the clopidogrel group and 3.5% of the ticlopidine group (p<0.001). In addition to clopidogrel, TIA and diabetes exhibited significantly higher ineffective platelet inhibition in a univariate analysis. In the multivariate analysis, clopidogrel and TIA remained significant, and diabetes fell to borderline significance (p=0.061). CONCLUSIONS: The response to clopidogrel and ticlopidine can vary between patients. Platelet inhibition is lower for clopidogrel than for ticlopidine on the platelet function test in patients with ischemic stroke. The clinical impact of these results remains uncertain; further investigations are needed.

Compressive Tibial Neuropathy Induced by Myxoid Liposarcoma in the Popliteal Fossa

Tibial nerve compression occurs most often at the ankle. A proximal tibial nerve entrapment at the popliteal fossais rare because of the deep location of the nerve. We report a case of tibial neuropathy caused by a myxoid liposarcoma at the popliteal fossa, which was diagnosed by electrophysiologic studies, magnetic resonance imaging and an open excisional biopsy. This case illustrates that tumors should be considered when patients present with an entrapment neuropathy of the proximal tibial nerve.

Oculomotor Neuromyotonia with Lid Lag

A 52-year-old woman developed episodic diplopia during a period of six months. Between the episodes, depression of the right eye was mildly impaired and lid lag was evident in the right eye on downward gaze. On resuming the primary position after prolonged leftward gaze, she developed a right esotropia and limitation of abduction, elevation, and depression in the right eye. Lid lag may be a sign suggesting oculomotor neuromyotonia in patients with episodic diplopia. The isolated lid lag without lid retraction suggests primary aberrant oculomotor regeneration as a mechanism of this phenomenon.