Hande Alibaş

Headache During Air Travel: An Unusual Cause of Reversible Cerebral Vasoconstriction Syndrome

Reversible cerebral vasoconstriction syndrome (RCVS) is a rare condition characterized by segmental and reversible narrowing of the cerebral arteries. High altitude has been reported as an extremely rare cause for this distinctive syndrome. Here, we report a case of RCVS and propose air travel as the triggering factor. A 51-year-old female patient experienced an intense thunderclap headache during air travel, which persisted through her entire trip. On the second day after landing back home, she presented with right hemiparesis accompanying the headache at a facility different from where the authors are located. Diffusion weighted magnetic resonance imaging (DWI) and cranial magnetic resonance angiography (MRA) had revealed a pattern of restriction on the left frontoparietal cortical region and segmental vasoconstrictions that were more predominant on the posterior circulation consecutively. A diagnosis of RCVS was considered. When the patient was discharged with verapamil therapy, she had normal neurological status. Two days later, she was hospitalized at our hospital with an attack of weakness of the left leg, which lasted for 10 min. The DWI showed no infarctions. However, segmental vasoconstrictions on bilateral posterior cerebral arteries, anterior cerebral arteries (ACAs), and middle cerebral arteries were observed on cranial MRA. Her therapy regimen was changed to nimodipine and dexamethasone. On the follow-up, her left-sided weakness and thunderclap headache attacks had recurred until the last attack, which persisted. No significant blood pressure changes were observed at that time. The DWI revealed acute infarctions on ACA territory and bilateral head of the caudate nuclei. MRA showed that the vasoconstrictions were more prominent on the mentioned arteries. Her therapy was switched to intravenous nimodipine and the dose was gradually increased up to 1.5 mg/hour and dexamethasone was stopped. Under this treatment, she developed no further symptoms or infarctions. When she was discharged 1 month after her first symptom on oral From the Department of Neurology, Marmara University Faculty of Medicine, Istanbul, Turkey (H. Alibas, S. Ceferova, P. Kahraman Koytak, K. Uluc, I. Midi, N. Tuncer Elmaci, S. Aktan, and T. Tanridag).

A Case with Leukoencephalopathy with Brainstem and Spinal Cord Involvement and Elevated Lactate (LBSL) with Its Characteristic Clinical and Neuroimaging Findings

Leukoencephalopathy with brainstem and spinal cord involvement and elevated lactate (LBsL) is a recently described white matter disorder, characterized by slowly progressive cerebellar, pyramidal, and dorsal column dysfunction [1]. It is a very rare autosomal recessive mitochondrial disease; however, its unique pattern of magnetic resonance imaging (MRI) findings distinguishes it from the other leukoencephalopathies and is therefore pathognomonic [1–8]. Distinct MRI findings of nonhomogeneous cerebral white matter abnormalities with selective involvement of the brainstem and spinal cord and specific magnetic resonance spectroscopy (Mrs) abnormalities are the striking neuroradiological features of the disease. We herein report a case of LBsL.

Hemifacial Spasm Revealing Contralateral Peripheral Facial Palsy

Dear Editor, Peripheral facial nerve palsy (PFP) is a benign and selflimiting disease. Its etiology is usually unknown and it is known as idiopathic facial palsy or Bell palsy. However, it can cause transient or long lasting severe motor dysfunction and involuntary movement disorders (1). A 58-year-old female was admitted to our neurology polyclinic with a slip in her mouth, dysfunction of tasting, and difficulty in closing her left eye. A facial nerve examination revealed asymmetry of the frontal muscles and oral commissures and weakness in closing her left eye. Findings suggested left PFP with a grade 4 HouseBrackmann score. Otoscopic examination of the same side was normal. In biochemical tests, blood glucose level was 145 mg/dL, which was higher than normal. The patient had not been previously diagnosed as having diabetes mellitus. Diet and diet and regular measurement of her blood glucose levels were advised. Oral methylprednisolone with a dosage of 64 mg/ day was initiated. While oral steroid treatment was going on, physiotherapy was initiated at the second week of the palsy and lasted for 4 weeks. During follow-up, her glycated hemoglobin ‘HbA1c’ was found as 8% (normal: <5.7%) and oral anti-diabetic treatment was initiated. The patient was admitted to the neurology polyclinic with involuntary movements involving the right side of her face. Neurologic examination revealed partial improvement of left PFP. She could close her left eye and better pull the left commissure of her mouth (grade 2 House-Brackmann score). On the other hand, involuntary movements showing clonic and sometimes tonic patterns involving the periocular muscles, cheek and lips of the right side were observed suggesting hemifacial spasm (HS). Contrast-enhanced magnetic resonance imaging of cranium showed normal parenchymal and vascular structures. The patient was considered as having ‘contralateral HS on PFP’. Electromyography (EMG) showed lower compound muscle action potential (CMAP) amplitude on the left facial nerve compared with the right side (facial nerve CMAP amplitude recording on musculus nasalis was 1.8 mV on the right and 0.7 mV on the left; facial nerve CMAP amplitude recording on musculus orbicularis oculi was 1.1 mV on the right and 0.2 mV on the left). Needle EMG revealed motor unit potentials showing chronic denervation and reinnervation findings in the muscles innervated by left facial nerve. The findings were suggestive of chronic partial axonal damage in the regeneration period in the left facial nerve. Surface electrode recordings on the contralateral musculus levator anguli oris and musculus orbicularis oculi showed findings suggestive of HS. Movement disorders occurring contralateral to peripheral nerve palsies have been reported rarely in the literature including an increase in spontaneous blink frequency and muscle activity similar to blepharospasm (1). HS, as a such movement disorder, has been reported very infrequently in the literature. Bonnet et al. (2) reported a patient with a prior HS who developed contralateral idiopathic PFP. Ekmekçi et al. (3) discussed HS-PFP as a crossed

Cutaneous silent period in myofascial pain syndrome

Introduction: An increased response to painful stimuli without spontaneous pain suggests a role of central hyperexcitability of pain pathways in the pathogenesis of myofascial pain syndrome (MPS). In this study we aimed to test the hypothesis that spinal pain pathways are affected in MPS. We used cutaneous silent period (CSP) parameters to demonstrate the hyperexcitability of spinal pain pathways in MPS. Methods: Twenty‐nine patients diagnosed with MPS and 30 healthy volunteers were included in the study. The CSP recordings were performed in the right upper and left lower extremities. Results: In both upper and lower extremities, patients had prolonged CSP latencies (P = 0.034 and P = 0.049 respectively) and shortened CSP durations (P = 0.009 and P = 0.008, respectively). Discussion: Delayed and shortened CSP in MPS patients implies dysfunction in the inhibitory mechanism of the spinal/supraspinal pain pathways, suggesting central sensitization in the pathogenesis of MPS and supporting our research hypothesis. Muscle Nerve 57: E24–E28, 2018

Medial plantar-to-radial amplitude ratio: does it have electrodiagnostic utility in distal sensory polyneuropathy?

Purpose of the study: We proposed a new electrophysiological parameter medial plantar (MP)-to-radial amplitude ratio (MPRAR), similar to sural-to-radial amplitude ratio (SRAR), in the diagnosis of distal sensory polyneuropathy (DSP), based on the concept that distal nerves are affected more and earlier than proximal nerves in axonal neuropathies. We aimed to investigate the diagnostic sensitivity of this parameter in diabetic DSP, together with sensitivities of SRAR and MP nerve action potential (NAP) amplitude. Materials and Methods: In 124 healthy controls and 87 diabetic patients with clinically defined DSP and normal sural responses, we prospectively performed sensory nerve conduction studies (NCS), and evaluated the MP NAP amplitude, MPRAR and SRAR values. We determined the lower limits of normal (LLN) of these parameters in the healthy controls and calculated their sensitivities and specificities in detecting DSP in diabetic patients. Results: MP nerve amplitude and MPRAR values were significantly lower in the patient group, compared to controls. However, SRAR values did not differ significantly between the two groups. The LLN of MP NAP amplitude was found to be 4.1 μV. The cutoff values for SRAR and MPRAR were determined as 0.24 and 0.16, respectively. MPRAR was abnormal in 21.8% of patients. However, the most sensitive parameter in detection of DSP was MP NAP amplitude, which showed a sensitivity of 31% and a specificity of 100%. Conclusions: Although MPRAR is more sensitive than SRAR in detecting DSP, it does not provide additional diagnostic yield to the assessment of MP NCS alone in diabetic DSP patients with normal sural responses.

Progressive dysarthria and dysphagia in an otherwise healthy girl

Fig. 1. (A) Note the left unilateral atrophy and deviation of the tongue. (B) Coronal fluid-attenuated inversion recovery MRI conforming almost complete replacement of the left genioglossus muscle by fat tissue (arrow)