Luciana Leon Cejas

Acute brachial plexus neuropathy with involvement of cranial nerves IX, X, XI and XII.

INTRODUCTION Acute brachial plexus neuropathy is characterized by acute onset of shoulder girdle and arm pain, followed by weakness of the shoulder and arm muscles. It affects primarily nerves of the upper trunk of the brachial plexus and the long thoracic nerve. Cranial nerve involvement is an infrequent association and implies a diagnostic challenge. We report a unique case of acute brachial plexus neuropathy with involvement of the cranial nerves IX, X, XI and XII. CASE REPORT Fifty six year-old woman who developed acute dysphonia, dysphagia and left shoulder pain, followed, six days later, by left arm weakness. Needle examination showed only fibrillation potentials and positive sharp waves in the left deltoid muscle. MRI of the brachial plexus shows enlargement of the trunks, cords and terminal branches, with mild gadolinium enhancement. DISCUSSION This case illustrates the unique presentation of neuralgic amyotrophy with involvement of nerves outside the brachial plexus, and the importance of MRI for diagnosis, in the absence of electrophysiologic involvement.

Intraneural perineuriomas: diagnostic value of magnetic resonance neurography: León Cejas et al

Intraneural perineurioma (IP) is an under‐recognized hypertrophic peripheral nerve tumor. It affects young patients involving frequently the sciatic nerve and its branches and presents with a progressive, painless and predominantly motor deficit. Magnetic resonance neurography (MRN) is a useful tool to localize the lesion, evaluate its extension, and discriminate between different etiologies. We reviewed the clinical records of 11 patients with pathologically confirm IP. Eight patients were males with mean age 19 years. Initial complains were unilateral steppage (seven patients), bilateral steppage (one patient), unilateral gastrocnemius wasting (one patient), unilateral thigh atrophy (one patient), and unilateral hand weakness (one patient). Nine patients had mild painless sensory loss. Examinations revealed involvement of sciatic nerve extending into the peroneal nerve (eight patients), posterior tibial nerve (one patient), radial nerve (one patient), and femoral nerve (one patient). MRN revealed enlargement of the affected nerve isointense on T1‐weighted, hyperintense on T2 fat‐saturated images, and with avid enhancement on post‐contrast imaging. In all patients, a nerve biopsy confirmed the diagnosis. MRN allows early and non‐invasive identification of this tumor and is a key tool providing localization and differential diagnosis in patients with slowly progressive focal neuropathies.

BLOOD PRESSURE EVOLUTION IN YOUNG PATIENTS WITH ACUTE ISCHEMIC STROKE: A new model for understanding the natural course of spontaneous hypertension?

ABSTRACT Background: Approximately 80% of patients suffering an acute ischemic stroke develop transient hypertension. The physiopathological mechanism remains unclear. Due to the lack of vascular risk factors, young adults could be a useful model for understanding blood pressure (BP) evolution in this setting. Methods: Patients between 18 and 55 years old admitted with an acute ischemic stroke were included. BP was evaluated during the following 48 h. Hypertension was defined as: systolic blood pressure (SBP) ≥140 mmHg or diastolic blood pressure (DBP) ≥90 mmHg. Patients were divided into two groups: RF group included those who had a previous vascular risk factor and/or other medical condition known to affect the autonomic function; noRF group included patients without any of the previously stated conditions. Results: Thirty-nine patients were included: mean age: 44.26 years old, 21 were males (53.8%). Mean SBP and DBP at admission were 139.77 ± 5.35 mmHg (range: 90–243) and 84.44 ± 3.02 mmHg (range: 60–128), respectively; 43.58% patients were hypertensive at admission. SBP decreased significantly during the first 48 h (p = 0.044) for the entire population. RF group has higher SBP (p = 0.009) and DBP (p = 0.011) at admission than the noRF group. Conclusion: Most patients were normotensive at admission and BP fell spontaneously despite BP at admission. Young patients without medical conditions that could alter the autonomic system function could be a useful model for understanding acute hypertension in ischemic stroke.

S47. Sensory Guillain Barré syndrome

Introduction The classic Guillain Barre syndrome (GBS) is characterized by motor weakness, hyporreflexia, but limited sensory deficits. Sensory variants involving either small or large fibers or both are unusual and represent a diagnostic challenge. Methods We described 6 patients presenting with the sensory variant of GBS and retrospectively analyzed the clinical and electrophysiological findings of patients fulfilling the criteria for Sensory GBS according to Oh et al. criteria. Results Six patients were identified (mean age 38 years: range 15–54 years). Four had a previous infection. They all consulted due to distal painful paresthesias and allodynia. On examination the 6 patients presented normal strength and normal cranial nerves through the course of the disease with reduced knee and ankle reflexes in 3 patients. Distal hyperesthesia to pinprick was identified in 3 and one of them additionally had hyperhidrosis and constipation. Two additional patients presented hypoesthesia to pinprick and temperature. One patient had distal proprioceptive sensory loss with sensory ataxia. CSF albumin cytological dissociation was present in 3 patients. Nerve conduction studies (NCS) identified a sensory motor demyelinating neuropathy in 2 patients. Among the 4 with normal NCS, 2 had abnormal cold and warm threshold in their QST evaluation. All patients received symptomatic treatment for the neuropathic pain and only two IvIg therapies. Longstanding pain, fatigue or both were persistent findings in 5 patients after a mean follow up of 6 months. Conclusion The sensory variant of GBS is both an infrequent presentation and a diagnostic challenge. Longstanding pain and fatigue are common persisting findings.

RAPIDLY PROGRESSIVE DEMENTIA SECONDARY TO AUTOIMMUNE ENCEPHALITIS: NEUROPSYCHOLOGICAL PROFILES AND RESPONSE TO IMMUNOTHERAPY

P2-313 IS CUED RECALL OF SHARED AUTOBIOGRAPHICAL KNOWLEDGE USEFUL IN CLINICAL DEMENTIA ASSESSMENT? Claudia Frankenberg, Maren Knebel, Christina Degen, Nadeshda Andrejeva, Petra Wetzel, Lina Sidonija Gorenc-Mahmutaj, Sabrina Dominique Navratil, Inga Meyer-K€uhling, Britta Wendelstein, Johannes Schr€oder, Section of Geriatric Psychiatry, University Hospital Heidelberg, Heidelberg, Germany; Interdisciplinary Ageing Research, Faculty of Educational Sciences, Goethe University, Frankfurt am Main, Germany; Institute of Gerontology, University of Heidelberg, Heidelberg, Germany. Contact e-mail: Claudia.Frankenberg@med.uni-heidelberg.de