Luísa Albuquerque

Delayed leukoencephalopathy after acute carbon monoxide intoxication

Delayed leukoencephalopathy is an uncommon complication of hypoxic-ischemic events of different etiologies, including carbon monoxide intoxication. We present a case of a 40-year-old male patient who was admitted with rapidly progressive neurocognitive and behavioral deficits. There was a history of accidental carbon monoxide intoxication one month before, presenting with loss of consciousness and short hospitalization, followed by a complete clinical recovery. The imaging studies in the delayed phase depicted confluent, symmetric supra-tentorial white matter lesions in keeping with diffuse demyelinization. Restricted diffusion and metabolite abnormalities in magnetic resonance proton spectroscopy were also seen. The diagnosis of CO-mediated delayed post-hypoxic leukoencephalopathy was assumed after exclusion of other mimickers. Hyperbaric oxygen therapy was tentatively performed and the patient had a favorable clinical and radiological evolution.

Cerebral Amyloid Angiopathy Associated with Inflammation: Report of 3 Cases and Systematic Review

INTRODUCTION Cerebral amyloid angiopathy associated with inflammatory process (CAA-I) is a rare potentially treatable encephalopathy, characterized by an inflammatory response to vascular deposits of β-amyloid. We aimed to describe 3 clinical cases and perform a systematic review of all neuropathologically proved CAA-I case reports to describe its clinical and pathologic features and outcome under different treatments. METHODS We searched PubMed and Cochrane Library and screened references of included studies and review articles for additional citations. Outcome was classified at the last available follow-up by the modified Rankin Scale (mRS). RESULTS A total of 67 publications, reporting on 155 patients, were included. Mean age was 66.9 years, and 53.5% were men. The most common clinical presentation was cognitive dysfunction (48.0%) followed by headaches (38.7%), seizures (36.7%), and pyramidal signs (20.0%). Perivascular and vasculitic inflammation with granuloma was the most common pathologic pattern (27.5%). Eighty-six percent were treated with corticosteroids and 33.9% with cyclophosphamide. Forty-two percent regained independence (mRS score 0-2), whereas 20.5% were left with a severe handicap (mRS score 3-5) and 37.5% died. There were no statistically significant differences in outcome between patients treated with therapy with corticosteroids alone comparing with those treated with combination corticosteroids with cytostatic agents. CONCLUSIONS The most common clinical manifestation of CAA-I was cognitive dysfunction. The functional outcome was unfavorable in the majority of the patients, with death or severe disability in almost two third of the cases, despite treatment. No differences in outcome could be detected between patients treated with corticosteroids versus patients treated with cytostatics, combined with corticosteroids.

STN-DBS does not change emotion recognition in advanced Parkinson's disease

UNLABELLED Deep brain stimulation of the subthalamic nuclei (STN-DBS) for the treatment of levodopa-induced motor complications in advanced Parkinsons disease (APD) has been associated with neuropsychiatric disorders. It has been suggested that a postoperative decline in visual emotion recognition is responsible for those adverse events, although there is also evidence that emotional processing deficits can be present before surgery. The aim of the present study is to compare the ability to recognize emotions before and one year after surgery in APD. METHODS Consecutively operated APD patients were tested pre-operatively and one year after STN-DBS by the Comprehensive Affect Testing System (CATS), which evaluates visual recognition of 7 basic emotions (happiness, sadness, anger, fear, surprise, disgust and neutral) on facial expressions and 4 emotions on prosody (happiness, sadness, anger and fear). RESULTS In a sample of 30 patients 6 had depression or apathy at baseline that significantly increased to 14 post-surgery. There were no significant changes in the tests of identity discrimination, discrimination of emotional faces, naming of emotional faces, recognition of emotional prosody, and naming of emotional prosody after STN-DBS. The results of emotion tests could not predict the development of the neuropsychiatric symptoms. DISCUSSION This study does not support the hypothesis of an acquired change in emotion recognition, either in faces or in prosody, after STN-DBS in APD patients. Neuropsychiatric symptoms appearing after STN-DBS should not be attributed to new deficits in emotional recognition.

Reversible bilateral sensorineural hearing loss in a woman with cerebral venous thrombosis

JO N 2963 Brain MRI disclosed extensive CVT involving the superior longitudinal sinus, the straight sinus and proximal portion of both transverse sinuses. There were no visible changes in the inner ear. Laboratory examinations showed homozygosity for MTHFR A1298C, increased serum homocysteine, reduced vitamin B12 and folic acid. The patient was treated since admission with repeated therapeutic LP, furosemide and acetazolamide to control increased intracranial pressure and with low molecular weight heparin, vitamin B12 and folic acid for the CVT. Three days after admission, evaluation of hearing loss showed moderate (56–70 dB) bilateral sensorineural hearing loss, mainly affecting low frequencies (Fig. 1) with normal tympanogram and otoscopy. Both acoustic reflexes were absent ipsilaterally and contralaterally. LP performed on the same day showed an opening pressure of 43 cmH2O. Three weeks later, LP opening pressure was 26 cmH2O, hearing had improved 10–20 dB (right-left, respectively) and there was no ophthalmoparesis. Cerebral angiography showed recanalization of the dural sinuses. However, intracranial hypertension did not regress on subsequent LP and a lumboperitoneal shunt was placed three months after the first observation. One month after shunting, the audiogram (Fig. 2) of the left ear had normalized and that of the right ear showed a mild low frequency hearing loss (45–40 dB improvement, right and left). Acoustic reflexes were still absent. Visual loss persisted and papilledema had been replaced by optical nerve atrophy. CVT may have contributed to sensorineural hearing loss by inducing increased intracranial pressure. This can be explained by two mechanisms: 1. Increased pressure in the subAna C. Fonseca Luísa Albuquerque José M. Ferro

Advanced Parkinson disease patients have impairment in prosody processing.

ABSTRACT Background: The ability to recognize and interpret emotions in others is a crucial prerequisite of adequate social behavior. Impairments in emotion processing have been reported from the early stages of Parkinson’s disease (PD). This study aims to characterize emotion recognition in advanced Parkinson’s disease (APD) candidates for deep-brain stimulation and to compare emotion recognition abilities in visual and auditory domains. Method: APD patients, defined as those with levodopa-induced motor complications (N = 42), and healthy controls (N = 43) matched by gender, age, and educational level, undertook the Comprehensive Affect Testing System (CATS), a battery that evaluates recognition of seven basic emotions (happiness, sadness, anger, fear, surprise, disgust, and neutral) on facial expressions and four emotions on prosody (happiness, sadness, anger, and fear). APD patients were assessed during the “ON” state. Group performance was compared with independent-samples t tests. Results: Compared to controls, APD had significantly lower scores on the discrimination and naming of emotions in prosody, and visual discrimination of neutral faces, but no significant differences in visual emotional tasks. Conclusion: The contrasting performance in emotional processing between visual and auditory stimuli suggests that APD candidates for surgery have either a selective difficulty in recognizing emotions in prosody or a general defect in prosody processing. Studies investigating early-stage PD, and the effect of subcortical lesions in prosody processing, favor the latter interpretation. Further research is needed to understand these deficits in emotional prosody recognition and their possible contribution to later behavioral or neuropsychiatric manifestations of PD.

Effect of lesion site on serial position during list learning: a study with the CVLT.

Successful learning of supraspan word lists such as the California Verbal Learning Test (CVLT) relies more on clustering strategies than rote learning, subserved by the frontal and temporal lobes. The authors studied the effect of word sequence in CVLT learning, in 15 patients with frontal (FLL) and 15 temporal (TLL) lesions, and 33 controls. Experimental measures were: number of clusters, number of first (FI), middle (MI) and last items (LI), in learning trials and in total immediate recall. FLL disclosed significantly lower FI along learning. Clusters were similar among groups. This difficulty is discussed according to the role of frontal lobes in learning and memory.

Biopsy-negative, varicella zoster virus (VZV)-positive giant cell arteritis, zoster, VZV encephalitis and ischemic optic neuropathy, all in one

A 72-year-old man developed clinical features of giant cell arteritis (GCA) and ipsilateral ophthalmic-distribution zoster, followed within 2 weeks by VZV encephalitis and 2 months later by ischemic optic neuropathy. Temporal artery biopsy was histopathologically negative for GCA, but contained VZV antigen and VZV DNA in multiple non-contiguous (skip) areas. The collective clinical and laboratory findings revealed a remarkably close temporal association of zoster, multifocal VZV vasculopathy with temporal artery infection, biopsy-negative VZV-positive GCA and VZV encephalitis.

Giant cell arteritis with symptomatic intracranial stenosis and endovascular treatment

Giant cell arteritis (GCA) is the commonest systemic vasculitis in adults. It is characterized by granulomatous inflammation in the wall of medium size and large arteries. Classically, it affects external carotid branches, namely, superficial temporal artery. It is a rare cause (3–4 %) of transient ischaemic attacks, and stroke due to carotid and vertebral extracranial stenosis [1, 2]. There are scarce reports of GCA with intracranial stenosis and stroke [3, 4]. Here, we report on a 65-year-old black man with a medical history of hypertension, dyslipidemia, congenital 6-phosphogluconate dehydrogenase, and GCA confirmed by temporal artery 3 months before, presented to emergency department with sudden language and right motor deficit, while withdrawing corticosteroid treatment but still under prednisolone (PDN) 50 mg/daily, acetylsalicylic acid (AAS) 100 mg/daily and lisinopril 5 mg/daily. On neurological examination, there was anomic dysphasia, right hemiparesis and homolateral upper limb ataxia. Laboratory examination showed an elevated erythrocyte sedimentation rate (ESR) (57 mm/h) but autoimmune, cerebrospinal fluid and hemoglobin electrophoresis studies were normal. Diffusion-weighted MRI showed acute left middle cerebral artery watershed infarcts (Fig. 1a) and right posterior inferior cerebellar artery (PICA) ischemia. Doppler ultrasonography (US) of the temporal arteries showed bilateral periluminal hypoechogenic halo. Cervical US and Transcranial Doppler (TCD) suggested distal preocclusive stenosis of the left internal carotid artery (ICA) and a severe stenosis of the cavernous segment of the right ICA. Angio-MRI showed the progressive left ICA stenosis from its origin, with pre-occlusive stenosis in the cavernous and supraclinoid segments; occlusion of V4 segments of both vertebral arteries and of the inferior third of the basilar artery (Fig. 1b). Furthermore, conventional angiogram showed a segmental stenosis of the right ICA petrous segment (the flow came from the anterior circulation bilaterally; the posterior circulation fed via the right posterior communicating; there was a reverse flow in the basilar artery filling the left anterior inferior cerebellar artery and PICA) (Fig. 1c, d). Systemic and brain PET-CT showed decreased uptake of 18FDG in parietal and left occipital cortices and right cerebellar hemisphere; increased uptake in the territory of right superficial temporal and left posterior auricular arteries (Fig. 1f); thoracic and abdominal aorta included iliac and femoral branches and a cardiac MRI (with aortic arch) were unremarkable. Treatment was switched to clopidogrel 75 mg/daily, simvastatin 20 mg/daily, and PDN 1 mg/kg/daily and he was discharge. One month later, he developed recurrent aphasia following iatrogenic hypotension and syncope. ESR was 9 mm/h and TCD was stable. Brain MRI disclosed a new acute stroke, adjacent to the previous lesion. Angioplasty was performed in the supraclinoid segment of left ICA and D. Neutel T. P. e Melo L. Albuquerque Neurology Department, Santa Maria Hospital, CHLN, Lisbon, Portugal

Mycoplasma pneumoniae causing nervous system lesion and SIADH in the absence of pneumonia

A patient was admitted for fever and acute respiratory failure (ARF), rapidly progressive tetraparesis, delirium, behavioral abnormalities, and diplopia. Leukocytosis and a rise in C-reactive protein were present. A syndrome of inappropriate anti-diuretic hormone secretion (SIADH) was also diagnosed. Lumbar puncture yielded colorless CFS with mononuclear pleocytosis and protein rise. Electrodiagnosis revealed demyelinating polyneuropathy and axonal degeneration. Serum IgG and IgM for mycoplasma pneumoniae (MP) was consistent with acute infection, and erythromycin was started with rapid resolution of symptoms. Contrarily to most reports, an associated respiratory disease was not present and SIADH in association with MP has been reported only once, in a patient without direct central nervous system (CNS) involvement. Differential diagnosis and possible pathogenic mechanisms are discussed.

Recovery after copper-deficiency myeloneuropathy in Wilson's disease.

Wilson’s disease (WD) treatment focuses on removing excess copper from the body and preventing reaccumulation, but there are reports of neuropathy and myeloneuropathy (MN) linked to copper deficiency (CD) induced by excessive depletion [1]. Here we report on a WD patient with MN associated with longstanding CD. The patient is a 36-year-old male diagnosed with WD at 20 years old, having displayed generalized dystonia, bradykinesia and freezing of gait. Clinical stability was achieved with zinc sulphate 150 mg/ day and trientine 500 mg/day. In 2006, he developed low urinary copper (24 h urine copper = 128 lg/24 h, recommended 200–500 lg/24 h; serum copper 6.35 lg/dL) [6], which worsened in the following years. In 2008 he was switched to D-penicillamine 600 mg/day due to drug availability problems. Soon after, he developed a nephrotic syndrome with focal segmental glomerular sclerosis which resolved with corticosteroid therapy and switching back to trientine. In February 2011, he developed subjective numbness of both hands and feet and over the following months worsening of gait with frequent falls. In July 2011 neurological examination revealed de novo algic hypoesthesia of the limbs in a ‘‘glove and stocking’’ pattern, postural and vibratory hypoesthesia of the lower limbs and gait ataxia. Analytical studies revealed low serum and urine copper (serum copper 13.3 lg/dL, urine copper 40.5 lg/24 h), low ceruloplasmin (3.0 mg/dL), normal zinc levels (14.7 lmol/ L) and anemia (Hb 8.2 g/dL). Electromyography with conduction velocities revealed a mixed sensory-motor peripheral neuropathy and spinal MRI showed signs of posterior dorsal cord myelopathy (Fig. 1). Other causes of MN were excluded: both vitamin E and B12 levels were normal, and serological tests for HIV, syphilis and autoantibodies were negative. Therefore, a causal relationship between CD and MN was considered as probable. Trientine (500 mg/day) and zinc sulfate (330 mg/day) were substituted for zinc acetate 100 mg daily, which was progressively reduced and stopped in January 2012, due to persistent CD. Copper deficiency resolved by March 2012 (24 h urinary copper = 97 lg/24 h, recommended B100 lg/24 h for zinc monotherapy) [6]. Parallel improvement of sensorial ataxia allowed recovery of walking capacity without assistance. Spinal MRI showed mild regression of myelopathy signs (Fig. 1). Conduction velocities studies disclosed slight improvement in amplitude of sensory action potentials and conduction velocities (Table 1). At this point, zinc acetate 150 mg/day was reintroduced, and his condition remains stable 1 year after treatment. T. Teodoro (&) D. Neutel P. Lobo I. Conceição M. M. Rosa L. Albuquerque J. J. Ferreira Department of Neurology, Hospital de Santa Maria (CHLN, EPE), Lisbon, Portugal e-mail: tiagoteodoro@gmail.com