Abstracts of the 51St Annual Conference of the Italian Society of Neurology
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Neurological Sciences (2020) 41 (Suppl 1):S1–S347 https://doi.org/10.1007/s10072-020-04753-3 ABSTRACTS Abstracts of the 51st Annual Conference of the Italian Society of Neurology CASE REPORTS References: - L. Mandigers et al. A case report of iatrogenic deterioration of yet LISTERIA MONOCYTOGENES RHOMBENCEPHALITIS: A undiagnosed Rhombencephalitis; always be careful with corticoids. CHALLENGING DIAGNOSIS -BMC Clinical Pathology (2018);27;18:15 - Décard BF et al. Listeria rhombencephalitis mimicking a demyelin- S. M. Angelocola, M. Acciarri, R. Angeloni, P. Cardinali, F. Forconesi, I. ating event in an immunocompetent young patient. Multiple Paolino, E. Pucci Sclerosis (2017) Jan;23(1):123-125 - Bartt R. et al. Listeria and atypical presentations of Listeria in the Neurology Unit, "A. Murri" Hospital (Fermo) central nervous system. Semin Neurol. (2000);20(3):361-73 Neurolisteriosis may manifest in humans as meningitis, meningoen- cephalitis, and rhombencephalitis. Compared to other pathogens, PAINLESS WINGED SCAPULA AS THE ONSET OF A VERNET Listeria Monocytogenes (Lm) has a brainstem predilection. Contrary SYNDROME: A CASE REPORT to the more common meningitis, Lm rhombencephalitis occurs rarely and mostly in healthy and immunocompetent people. We describe the S. M. Angelocola, M. Acciarri, R. Angeloni, P. Cardinali, F. Forconesi, I. case of a 71-year-old immunocompetent woman previously in good Paolino, E. Pucci health.Aboutaweekbeforehospitaladmission,thepatientexperienced nausea, vomiting and diarrhea. Subsequently, dysarthria associated Neurology Unit, "A. Murri" Hospital (Fermo) with mild binocular diplopia appeared. At the time of admission, neu- rological examination showed dysarthria and right sixth and seventh “ ” “ ” cranial nerve palsy. The meningeal irritation signs were negative and The Jugular Foramen Syndrome (JFS) or Vernet syndrome (VS) body temperature was 36°C. She then performed a brain MRI with refers to the paralysis of the IX, X and XI cranial nerves, crossing the gadolinium, showing an extensive and clear-cut FLAIR signal alter- jugular foramen at the skull base. The main etiologies are the following: ation, affecting the pontine and mesencephalic tegmentus, without con- vascular (jugular vein thrombosis, aneurysm of internal carotid artery), trast enhancement; there was also a small focal diffusion signal alter- inflammatory (Wegener granulomatosis, Giant Cell arteritis), infectious ation located in the midbrain-pons, on the right. The thiamine dosage, (VZV, HSV), neoplastic or traumatic. We describe the case of a patient serum electrolytes and inflammatory markers were in range. During the with subacute onset of painless atrophy of the left shoulder, in particular hospitalization, she experienced worsening of vigilance and left with a winged scapula increased by the abduction of the arm. The patient hemiparesis. Because of the previous history of gastrointestinal infec- did not report prior painful symptoms, nor trauma on the shoulder and ’ tion, we made the suspiction of a post-infective Bickerstaff cervical spine. Moreover, he didn t have infectious-inflammatory epi- rhombencephalitis. The patient underwent a lumbar puncture that sodes prior to the development of hyposthenia. His symptoms were ini- showed a mild mononuclear pleiocytosis with a little increase in pro- tially attributed to a neuromuscular condition, therefore the patient initial- teins; virological tests were negative; the cultural exam didn’tshow ly performed the sampling of CPK levels and anti-AChR antibodies, with bacterial growth. We then made a therapeutic attempt with steroids negative results. VES and PCR levels were in range. An electromyogra- and a cycle of in vein immunoglobulins, without satisfactory re- phy was then scheduled. Subsequently, dysphagia and nasal voice also sults. There was a progressive further clinical worsening with appeared, and the patient was then evaluated by an otolaryngologist, who fever appearence. We then started empirical antibiotic treatment found a paralysis of the left vocal cord and a hypomobility of the soft with ceftriaxone and decided to repeat a brain RMN, which palate with reduction of the gag reflex on the left. The patient then per- showed progression of the FLAIR hyperintense lesion with wide- formed a brain and skull base CT scan with evidence of a "widening" of spread involvement of the midbrain and basal ganglia. Finally, we the jugular foramen. He then underwent an electromyography, with the repeated a CSF examination with evidence of negative culture test evidence of a lesion of the left accessory nerve with phenomena of total but positive film-array PCR for Listeria. Blood cultures showed denervation of the trapezius muscle and partial denervation of the the presence of Lm. Specific antibiotic treatment was then started sternocleidomastoid muscle. The remaining muscles belonging to the but the clinical picture was very poor and the patient died after a brachial plexus were normal, as well as motor and sensory nerve conduc- few days. Our attempt is to hilight the importance of considering tion studies. A brain and cervical MRI with angio sequences showed an a possible infectious nature of brainstem lesions even in presence of non expansive process at the level of the left jugular foramen with a enhancing MRI alterations, negative CSF cultures and without systemic polylobulated appearance and irregular contrast enhancement. This lesion and laboratory signs of infection. If Listerial infection is not considered or showed a mass effect with erosive phenomena at the skull base. The ruled out with specific tests, late diagnosis and thereby late initiation of patient then underwent the mass removal; the definitive histological ex- target antibiotic treatment will allow the disease to run its devastating amination revealed a chondrosarcoma. Vernet Syndrome is a rare condi- natural course. The administration of corticosteroids or i.v. immunoglob- tion, attributable to various etiologies, which must always be kept in mind ulins would accelerate the progression of the disease. when we are faced with the involvement of multiple cranial nerves S2 Neurol Sci (2020) 41 (Suppl 1):S1–S347 (especially bulbar). Some pathologies that enter into differential diagnosis A (VERY) EARLY ONSET PARKINSONISM are: brachial plexopathy, myasthania gravis, cranial inflammatory multineuropathy and other syndromes involving more than one cranial S. Aramini1,S.Satolli1, R, De Micco1, A.L. Torella2, F. Del Vecchio nerve (e.g. Collet-Sicard, Tapia, Avellis, Shmidt). If the onset is acute or Blanco2,V.Nigro2, G. Tedeschi1,A.Tessitore1 subacute, Parsonnage Turner syndrome must be excluded. References: 1Department of Advanced Medical and Surgical Science, University of - P. Doneddu et al. Neuralgic amyotrophy mimicking Vernet syn- Campania “Luigi Vanvitelli” (Napoli); 2UOSD Medical Genetics, – drome. Journal of the Neurological Sciences (2016);362:230 231 Department of Precision Medicine, University of Campania "Luigi - J. M Das et al. Jugular Foramen Syndrome (Vernet). NBCI Vanvitelli" (Napoli) Bookshelf (2020) A 51 years-old woman was admitted to our Neurology department, complaining of the poor control of her Parkinson’sdisease’s(PD) EARLY COMBINED PERCUTANEOUS AORTIC symptoms. Her history started back when she was 11 years old, with VALVULOPLASTY AND CAROTID ENDARTERECTOMY IN A an abrupt onset of gait disturbances and a dystonic posture in the PATIENT WITH MINOR ISCHEMIC STROKE AND HIGH left lower limb. She then progressively developed bilateral arm PERIOPERATIVE RISK tremor and axial rigidity. She was diagnosed with PD at the age of 24, and then started dopaminergic medication, which was still ongoing when she was referred to our Clinic. Her personal history A. Antonioni, N. Merli, R. Simonetti, A. Braccia, C. Azzini, A. De Vito also revealed a psychiatric disorder treated with neuroleptics since the patient was about 20 years old. Her father presented cognitive Neurology Department, Sant'Anna Hospital, University of Ferrara impairment. At our clinical examination, she was presenting with (Ferrara) unpredictable motor and non-motor levodopa-induced fluctuations, namely dystonic postures during the OFF-state and lower limbs Objectives: Here, we present the case of L.B., a 68-year-old Caucasian dyskinesia during the ON-state. The presence of a fixed dystonic male patient who reported a sudden onset of right upper limb weakness posture of left lower limb and facial-faucial-finger myoclonus were and transient visual disturbance. also detected. We performed a brain MRI, showing diffuse cerebral Materials and methods: Therefore, he was referred to ER of S. Anna atrophy; Dopamine Trasporter (DAT) SPECT revealed absent tracer University Hospital of Ferrara. His medical history included moderate aortic activity in the putamen of both hemispheres. We also performed an valve stenosis due to previous rheumatic heart disease at early age, dyslipid- extensive neuropsychological evaluation showing multiple cognitive emia, hypertension and not significant epi-aortic trunks atherosclerosis. deficit (MMSE: 21.31) and mood disturbances. Given the early Arrived at the Hospital on May 24th 2020, neurological evaluation document- symptoms onset, the presence of motor and non-motor features ed just a slight weakness of right upper limb (NIHSS 1); accordingly, he and the family history, we performed a next generation sequencing performed urgent brain CT, showing no acute lesions, and he was admitted genetic testing, which revealed