Slurred Speech Research Articles

Transient isolated brainstem symptoms preceding posterior circulation stroke: a population-based study

SummaryBackgroundTransient isolated brainstem symptoms (eg, isolated vertigo, dysarthria, diplopia) are not consistently classified as transient ischaemic attacks (TIAs) and data for prognosis are limited. If some of these transient neurological attacks (TNAs) are due to vertebrobasilar ischaemia, then they should be common during the days and weeks preceding posterior circulation strokes. We aimed to assess the frequency of TNAs before vertebrobasilar ischaemic stroke.MethodsWe studied all potential ischaemic events during the 90 days preceding an ischaemic stroke in patients ascertained within a prospective, population-based incidence study in Oxfordshire, UK (Oxford Vascular Study; 2002–2010) and compared rates of TNA preceding vertebrobasilar stroke versus carotid stroke. We classified the brainstem symptoms isolated vertigo, vertigo with non-focal symptoms, isolated double vision, transient generalised weakness, and binocular visual disturbance as TNAs in the vertebrobasilar territory; atypical amaurosis fugax and limb-shaking as TNAs in the carotid territory; and isolated slurred speech, migraine variants, transient confusion, and hemisensory tingling symptoms as TNAs in uncertain territory.FindingsOf the 1141 patients with ischaemic stroke, vascular territory was categorisable in 1034 (91%) cases, with 275 vertebrobasilar strokes and 759 carotid strokes. Isolated brainstem TNAs were more frequent before a vertebrobasilar stroke (45 of 275 events) than before a carotid stroke (10 of 759; OR 14·7, 95% CI 7·3–29·5, p<0·0001), particularly during the preceding 2 days (22 of 252 before a vertebrobasilar stroke vs two of 751 before a carotid stroke, OR 35·8, 8·4–153·5, p<0·0001). Of all 59 TNAs preceding (median 4 days, IQR 1–30) vertebrobasilar stroke, only five (8%) fulfilled the National Institute of Neurological Disorders and Stroke (NINDS) criteria for TIA. The other 54 cases were isolated vertigo (n=23), non-NINDS binocular visual disturbance (n=9), vertigo with other non-focal symptoms (n=10), isolated slurred speech, hemisensory tingling, or diplopia (n=8), and non-focal events (n=4). Only 10 (22%) of the 45 patients with isolated brainstem TNAs sought medical attention before the stroke and a vascular cause was suspected by their physician in only one of these cases.InterpretationIn patients with definite vertebrobasilar stroke, preceding transient isolated brainstem symptoms are common, but most symptoms do not satisfy traditional definitions of TIA. More studies of the prognosis of transient isolated brainstem symptoms are required.FundingWellcome Trust, UK Medical Research Council, Dunhill Medical Trust, Stroke Association, National Institute for Health Research (NIHR), Thames Valley Primary Care Research Partnership, and the NIHR Biomedical Research Centre, Oxford.

Therapeutic Plasma Exchange for Progressive Multifocal Leukoencephalopathy Related to Multiple Sclerosis Therapy with Natalizumab

Abstract 4386 Background:Progressive multifocal leukoencephalopathy (PML) associated with natalizumab in multiple sclerosis (MS) treatment is a complication for which therapeutic plasma exchange (TPE) has been used. We describe such a case treated with TPE. Case Report:A 54 year old woman with a past medical history of relapsing-remitting MS presented with recent onset ataxia and bilateral upper and lower extremity weakness and right hand dysmetria. Two weeks prior to presentation, she completed 57 treatments with natalizumab, a monoclonal antibody against cellular adhesion molecule α4-integrin that blocks lymphocytes from crossing the blood-brain barrier. Brain MRI showed new non-enhancing lesions in the right cerebellum and anterior left temporal lobe. Natalizumab was discontinued and investigation revealed high serum and CSF titers of JC virus DNA indicative of PML. The patient underwent a total of 3 TPE’s (1 every other day) with 3L of 5% albumin solution (1.4 plasma volume [PV]) used as replacement each TPE. After TPE, the patient initially showed improvement of neurological function and was transferred to inpatient acute rehabilitation. However, 4 days later she suffered a seizure and developed slurred speech. MRI showed that the cerebellar lesion had extended into the pons. The patient also developed dysphagia requiring nasogastric tube feeding and poorly-localized pain requiring pain management. She was treated with steroids for possible Immune Reconstitution Inflammatory Syndrome (IRIS) and discharged to hospice care for rehabilitation and pain management. The patient’s condition continued to deteriorate and she died shortly after being arriving at hospice. Conclusion:Natalizumab selectively immune suppresses the CNS putting patients at risk for PML and TPE with or without immunadsorption, can rapidly reverse the immune suppression by clearing natalizumab. Although we did not measure natalizumab concentrations in our patient, 1 report showed that a series of 3 TPE’s (1.5 PV) significantly decreased natalizumab levels while another showed that 3–5 TPE’s (1–1.5 PV) was effective. The effect of reducing natalizumab is to exchange the problem of a typically fatal opportunistic infection for the problem of an aggressive potentially injurious immune response to that virus. IRIS itself requires intense management to prevent brain injury or death and while there is no set standard of care, steroids are typically used. According to the manufacturer’s global monitoring system, the majority of natalizumab induced PML cases are treated with TPE which typically induces IRIS within days to weeks. The most recent mortality rate for all PML cases related to this drug is 22%, significantly lower than that seen with AIDS or in the oncology setting. Although our patient initially improved after TPE, the proximity of the PML to her brainstem, with or without IRIS, made her particularly vulnerable. Whether TPE may lead to more severe IRIS, as suggested by one report, requires further study. Nevertheless, given the severe consequences of PML itself, we would recommend TPE for natalizumab -induced PML. Disclosures:No relevant conflicts of interest to declare.

Novel mutation in the ATM gene in a Malian family with ataxia telangiectasia

Dear Sirs, Ataxia telangiectasia (A-T) is a rare, autosomal recessive disorder of childhood characterized by progressive cerebellar ataxia, telangiectasia, and immune defects, and is caused by mutations in the ataxia-telangiectasia mutated (ATM) gene [1]. The incidence of the disease is about 1 in 40,000 to 100,000 births [2]. A-T has been reported worldwide [3], but reports of this disease in Africa are rare and generally limited to clinical description. We describe a Malian family with parental consanguinity (Fig. 1a) and three of ten children presenting with cerebellar symptoms in early childhood. Two patients, 14- and 10-year-old boys, had normal births and development until age 2, when they presented with progressive gait difficulty, including difficulty stopping when running and falls. They both later developed slurred speech, weakness, and decreased coordination of the upper extremities. No sensory or bladder difficulty was noted. Family history was remarkable for a grandfather who died at age 75 and had balance problems since he was a teenager. Fig. 1 Pedigree and genetic features of the family. a Pedigree of the family showing consanguinity, and unaffected (white) and affected (black) individuals. b Sequencing shows a homozygous T7985A mutation (asterisk) in ATM. c ATM protein alignment in various ... Neurological examination of both patients showed an ataxic gait, markedly reduced hand coordination, and nystagmus on fixation and lateral gaze. They had slight distal weakness and atrophy in the lower legs. Reflexes were normal to reduced, and the Babinski sign was absent. The older brother had scoliosis. Cardiologic examination and testing were normal. Brain CT scan showed cerebellar atrophy with prominent cisterna magna. Vitamin E and beta and gamma tocopherol serum levels were normal. Genetic testing for Friedreich's ataxia was negative. A follow-up clinical assessment showed oculomotor apraxia, ocular telangiectasia, and square wave jerks. The parents noted that the patients had recurrent diarrhea and upper respiratory infections. These new findings were in favor of ataxia telangiectasia (Table 1). Additional blood testing showed high alphafetoprotein (AFP) levels and low IgA, IgE, and IgG2 levels. Also, aspartate and alanine aminotransferases (AST, ALT), and C-reactive protein (CRP) were elevated in the two patients, suggesting liver dysfunction. Genetic analysis of the ATM gene identified a novel homozygous single-nucleotide substitution at position c.7985T > A (Fig. 1b), predicting the amino acid substitution V2662D. Five available unaffected siblings did not have this sequence variant. The V2662 residue lies in a predicted ATP binding domain [3] and is conserved across a broad range of vertebrate species (Fig. 1c). In addition, this non-conservative amino acid change yields a score of –3 according to the BLOSUM 62 substitution matrix [4], and was not found in 100 ethnically matched controls, suggesting that the mutation found here is likely deleterious. Table 1 Summary of clinical and laboratory findings in patients More recently, the parents noticed that their 2-year-old son also had an ataxic gait. His AFP levels were elevated, but he had no telangiectasias. This highlights the usefulness of AFP testing in the diagnosis of A-T, as previously discussed [5]. Recurrent upper respiratory infections due to immune deficiency [6] occur in ataxia telangiectasia, but the two older patients also presented with frequent diarrhea, which may represent an associated infectious disease specific to the region in Mali where the patients live. Increased cancer susceptibility has been associated with A-T [7], however, hemato-oncological examination showed no signs of malignancy in our patients. In addition, abdominal and inguinal echography showed no tumors. Although cases of A-T have been reported in populations with African ancestry [3, 8] and in North Africa [9–11], reports of this disease in sub-Saharan Africa have been limited to clinical characterization [12, 13]. We report here genetically confirmed A-T with a novel mutation in this region, and add to the global spectrum of this disease. Our study shows that hereditary neurological diseases may not be uncommon in this region of Africa, although limited expertise and lack of diagnostic tools might lead to their underestimation and neglect.

CONFUSION IN THE RETURNED TRAVELLER – A RARE CAUSE OF ENCEPHALITIS

This is the only confirmed case of St Louis Encephalitis reported in the UK in 2011. The St Louis virus is a member of the single stranded RNA Flaviviridae family...

Dependence on Supratherapeutic Doses of Modafinil

To the Editor: Modafinil is a wakefulness-promoting agent used for the treatment of various sleep disorders like narcolepsy, obstructive sleep apnea, and shift work sleep disorder. Over the years it has gained popularity among prescribers due to its wakefulness-promoting efficacy and presumably lower abuse potential and has been used in the treatment of various fatigue syndromes, treatment-resistant depression, and attention-deficit/hyperactivity disorder.1,2 There is a controversy with regard to the abuse potential of modafinil, with some authors suggesting that it has low abuse potential and others suggesting that it has psychoactive and euphoric effects and leads to alterations in mood, perception, thinking, and feelings, all of which are seen with other central nervous system stimulants.1,2 The data with regard to its use in toxic doses are also limited.3–6 In our search, we could find no report of modafinil abuse or dependence in the literature. We report a case of a patient who used modafinil in supratherapeutic doses in a pattern that conformed with drug dependence. Case report. Mr A, a 53-year-old man diagnosed with schizoaffective disorder (DSM-IV criteria) for the last 33 years along with tobacco and benzodiazepine dependence syndrome (currently abstaining), presented with a history of excessive use of modafinil. Exploration of his history revealed that, 3 years prior, he consulted a psychiatrist for symptoms of fluctuating sadness of mood, lethargy, and sedation and was started on tablet modafinil, 50 mg/d, which was later increased to 100 mg/d along with tablet sertraline, 50–100 mg/d, and tablet amisulpride, 100 mg/d. The addition of modafinil made the patient feel fresh and active, and after 4–5 months modafinil was increased to 200 mg/d at the request of the patient. Over the next year, he started self-medicating with modafinil, taking 1 or 2 tablets (100–200 mg) in the afternoon or evening to overcome his boredom and fatigue, having felt a strong desire to take the same. On days when he would not take modafinil, he had strong craving and urge to do so, felt that something was missing in his life, and became irritable, felt weak, and had difficulty in concentrating on the work at hand. Over the next year, his intake of modafinil increased to 1,500–2,000 mg/d in order to experience the desired effect; he consumed 100–200 mg every 1–4 hours. Later, due to heavy doses of modafinil, he would have slurring of speech and poor attention and concentration. He continued to take modafinil against the advice of family and treating psychiatrists and would indulge in doctor shopping if he had difficulty in getting a prescription for modafinil or procuring it. With the available information, an additional diagnosis of modafinil dependence syndrome (per DSM-IV criteria for substance dependence disorder) was considered. He was counseled to taper off the dose of modafinil and was given benzodiazepines to manage the symptoms of withdrawal. The usual recommended dosage of modafinil is 200 mg/d. The previous reports of the use of modafinil in supratherapeutic or toxic doses did not describe continuous intake of the higher doses over the period.3–6 In the case reported here, the patient took 1,500–2,000 mg of modafinil per day for about 1 year. The intake of modafinil followed the typical pattern of drug dependence, which indicates its abuse potential. To conclude, our case suggests that modafinil has abuse potential and hence it should be prescribed cautiously and clinicians should be aware of its abuse potential. There is a need for further systematic studies to evaluate its abuse and dependence potential.

Pontine haemorrhage due to chondrosarcoma of the skull base.

A young adult presented with acute weakness of right side of body and slurring of speech. An initial brain CT scan showed a pontine haemorrhage, however MRI done a few days later revealed a mass in the left parapharyngeal space. Histopathology of the mass revealed that it was a chondrosarcoma. Subsequently the patient was initially treated with chemotherapy and radiotherapy and later surgery. Skull base chondrosarcomabe is to be included in the differentials of a young patient presenting with signs consistent with pontine haemorrhage.

ATP1A3 mutations in infants: a new rapid‐onset dystonia–Parkinsonism phenotype characterized by motor delay and ataxia

We report new clinical features of delayed motor development, hypotonia, and ataxia in two young children with mutations (R756H and D923N) in the ATP1A3 gene. In adults, mutations in ATP1A3 cause rapid-onset dystonia-Parkinsonism (RDP, DYT12) with abrupt onset of fixed dystonia. The parents and children were examined and videotaped, and samples were collected for mutation analysis. Case 1 presented with fluctuating spells of hypotonia, dysphagia, mutism, dystonia, and ataxia at 9 months. After three episodes of hypotonia, she developed ataxia, inability to speak or swallow, and eventual seizures. Case 2 presented with hypotonia at 14 months and pre-existing motor delay. At age 4 years, he had episodic slurred speech, followed by ataxia, drooling, and dysarthria. He remains mute. Both children had ATP1A3 gene mutations. To our knowledge, these are the earliest presentations of RDP, both with fluctuating features. Both children were initially misdiagnosed. RDP should be considered in children with discoordinated gait, and speech and swallowing difficulties.

Adult-onset Alexander disease with an R66Q mutation in GFAP presented with severe vocal cord paralysis during sleep

Adult-onset Alexander disease (AOAD) is clinically characterized by slowly progressive bulbar palsy, pyramidal signs, spastic paresis and cerebellar ataxia with atrophy of the medulla oblongata and upper cervical cord, and the glial fibrillary acidic protein (GFAP) gene has been identified to be the causative gene for Alexander disease [3]. So far, some reports have mentioned that AOAD presents with sleep apnea [1, 4, 5, 8–10, 12], but the apnea mechanism is not sufficiently known. Here, we report an AOAD patient presented with vocal cord paralysis during sleep. This is the first report indicating that AOAD presented with vocal cord abductor paralysis, followed by the rapid progression of vocal cord dysfunction. A 61-year-old woman was admitted to our hospital because of gait disturbance over a 4-year period. She has been described as ‘‘round shouldered’’ since childhood and would snore loudly while sleeping since her early 30 s. Her family history revealed that even her mother snored loudly and died because of unknown reason at 54 years of age. The patient had a short neck and cervical kyphosis. Neurological examination revealed slurred speech, spasticities of all extremities, and bilateral extensor Babinski signs. MRI findings revealed atrophy of the upper cervical cord and medulla oblongata. Axial imaging of the medulla oblongata showed multiple T2-weighted high-intensity lesions. Her cerebral blood flow on ECD-SPECT was reported previously [7]. Mutational analysis revealed a previously reported heterozygous missense mutation at exon 1 of GFAP, c.197G[A, which involved the substitution of glutamine for arginine at the 66th codon (R66Q) (Fig. 1a) [11]. We confirmed the diagnosis of AOAD. Polysomnography revealed obstructive sleep apnea syndrome with 292 apneic episodes at night (245 obstructivetype and 47 mixed-type). Although her mean percutaneous oxygen saturation during wakefulness was 94 %, her mean saturation during sleep was 84 %. Her apnea–hypopnea index (AHI) was 52.2/h indicating severe apnea syndrome. To investigate the mechanism of her apnea, we examined her laryngeal movement during wakefulness and sleep induced by intravenous administration of diazepam [6]. Awake-laryngofiberscopy showed mild vocal cord abduction restriction during inspiration. After intravenous diazepam administration (5 mg), the vocal cords showed paradoxical movement with obstruction during inspiration. To avoid fatal outcome Electronic supplementary material The online version of this article (doi:10.1007/s00415-012-6540-4) contains supplementary material, which is available to authorized users.

Abstract 223: Acute Ischemic Stroke in a 23-Year-Old Patient with Verrucous Endocarditis, Prothrombin Heterozygote Mutation, and a Patent Foramen Ovale: A Case Report and Literature Review

Background Stroke is the third leading cause of death and the leading cause of severe, long-term disability in the United States. Since the etiology of stroke in young adults is more heterogeneous, making a diagnosis is often a challenge requiring extensive clinical investigation. We report a case of acute ischemic stroke in a 23 year-old patient with multiple risk factors. Case Report A 23 year-old Spanish female with a history of tobacco abuse was brought to the Emergency room after she had a sudden onset of left-side weakness, slurring of speech, and lost of consciousness for a couple of minutes. Physical examination: left upper and lower extremities were completely flaccid with left facial weakness. MRI of head confirmed a right middle cerebral artery regional acute ischemic stroke. Transesophageal Echocardiogram revealed small granular densities on the mitral leaflets that consistent with atypical verrucous endocarditis and a patent foramen ovale (PFO). Hypercoagulable evaluation confirmed prothrombin gene mutation heterozygote. The erythrocyte sedimentation rate (ESR) was 75 mm/hour. All other tests were either negative or in normal range. Anticoagulation therapy was started with Heparin and then switched to Coumadin with an INR target of 2.5. Aspirin was also initiated. Discussion The differential diagnosis in young people (under 50 years) for potential etiology of ischemic stroke is broader than that for older adults. Atypical causes are more prevalent in this population, including cardiogenic cerebral embolus, hypercoagulable states, and autoimmune disease. Verrucous endocarditis is small thrombotic and nonbacterial lesions on the heart valves and endocardium, occurring frequently in systemic lupus erythematosus (SLE). However, hypercoagulable states and malignancy are often associated with the formation of verrucous endocarditis. Lesions are usually clinically silent. An embolic stroke may be the first feature to suggest the diagnosis. Since there was no evidence in malignancy, SLE, and antiphospholipid syndrome for our patient, hypercoagulable states should be considered as one of the risks for verrucous endocarditis. About 1-2% of the general population is heterozygous for the prothrombin gene mutation. In contrast, in Spain rates of 6% have been reported. It carries a 2 to 4 times increase in venous thrombosis and also increases the risk of arterial thrombosis (stroke and heart attack). Additionally, studies also show that inherited thrombophilic disorders in the pathogenesis of ischemic stroke might relate to congenital heart diseases such as Prothrombin G20210A variant and Factor V Leiden G1691A mutation might be associated with PFO. The prevalence of PFO in patients who have stroke of unknown cause may be about 40%. PFO also increases the rate of paradoxical thromboembolic stroke. This is particularly true in patients who have had a stroke at an age less than 50 years. Since our patient presents multiple risks for recurrent ischemic stroke, life-long anticoagulant therapy should be considered. Conclusion Acute ischemic stroke in a 23-years-old patient with verrucous endocarditis, prothrombin heterozygote mutation, and a PFO, but absent SLE has been rarely reported. Beside, the authors conclude that hypercoagulable testing and echocardiogram should be performed in young patients with stroke in order to provide us a better understanding of the etiology and the best treatment options.

Tapia Syndrome, Resulting from Internal Carotid Artery Dissection in the Setting of Yoga and Review the Localization and Mechanism of Injury to the Vagal and Hypoglossal Nerves (P03.025)

Objective: To describe a case of Tapia syndrome, resulting from internal carotid artery dissection (ICAD) in the setting of yoga. Background ICAD is an uncommon cause of stroke, accounting for 2–3% of all strokes. The absence of cerebral ischemic symptoms may lead to a delay in the diagnosis. Yoga is becoming increasingly popular. It is unclear if yoga practitioners are aware of the mechanism of ICAD. Design/Methods: Case report. Results: A sixty-eight year old gentleman with a medical history significant for GERD presented with slurred speech for five days and difficulty swallowing for 1 day. The patient had started practicing yoga, for left shoulder pain, approximately three weeks prior to admission. The yoga regimen included hyperflexion of the neck. He first noticed slurred speech along with neck discomfort five days prior to admission. This persisted without progression and on the day of admission he had sudden onset of trouble with swallowing both solid and liquid food. There were no other focal neurological symptoms noticed at this time. His general physical exam was unremarkable. His neurological exam was significant for hypophonia and dysphonia, dysarthria, dysphagia, especially with liquids, tongue deviation to the left, without atrophy or fasciculations, and a symmetric gag reflex. A left vocal cord paralysis was noticed on endoscopy. The remainder of the neurological exam was unremarkable. Magnetic resonance imaging revealed a significant decrease in flow and caliber of lumen of the cervical portion suggestive of left ICAD. Conclusions: ICAD should be considered in the differential of mononeuritis multiplex involving lower cranial nerves. ICAD can be caused by hyperflexion of the neck. Increasing awareness of the mechanism of ICAD among yoga practitioners will help avoid extreme postures involving the neck and decrease the incidence of an avoidable cause of neurologic dysfunction and stroke. Disclosure: Dr. Arcot has nothing to disclose. Dr. Liff has received personal compensation for activities with E-Rewards Medical. Dr. Horowitz has nothing to disclose.

Stroke Associated with Severe Cerebral Vasospasm after Petroclival Meningioma Resection (P05.263)

Objective: We report a rare case of stroke associated with delayed severe vasospasm after resection of petroclival meningioma. Background Diffuse cerebral vasospasm is a frequent complication following aneurysmal subarachnoid hemorrhage or after severe traumatic brain injury; however, symptomatic cerebral vasospasm following intracranial tumor resection is a rare, and underrecognized condition. Design/Methods: A 55-year-old woman with a petroclival meningioma, presented with intractable seizures and decreased right hand dexterity, and underwent resection of the mass She underwent a nine hour and was discharged home on POD#7 with mild slurred speech and IV nerve palsy. Patient was readmitted on POD#11 for evaluation of four days of progressively worsening slurred speech and lethargy. Results: MRI showed patchy acute infarcts in the left posterior frontal, parietal and occipital lobes, perisylvian region, corpus callosum splenium, mesial temporal lobe, hypothalamus, and bilateral pontomesencephalic junctions. MRA was significant for diffuse vasospasm, including:1) severe stenosis of terminal ICAs bilaterally,2) significant stenosis in the proximal ACA A1, MCA M1, and ACA A2 segments bilaterally 3) moderate-severe stenosis in both vertebral arteries and severe stenosis in mid basilar artery and proximal PCA.Four-vessel angiogram confirmed the findings. Patient was subsequently treated with balloon angioplasty and intrarterial nicardipine, with marked improvement in symptoms. Conclusions: In conclusion, although vasospasm is rare following tumor resection, its existence has been well documented in an increasing number of cases. There is no clear inciting factor for this phenomenon; it is evident factors are likely implicated in the development of the vasospasm including blood in basal cisterns, vessel manipulation, and tumor-related inflammatory mediators. It appears that the interval for the occurrence of vasospasm after tumor resection follows that seen after aneurysmal SAH. One needs to have a high index of suspicion for vasospasm as a cause of neurological deterioration postoperatively after brain tumor resection so that prompt diagnosis and aggressive management is instituted. Disclosure: Dr. Chmayssani has nothing to disclose. Dr. Murthy has nothing to disclose. Dr. Ghosh has nothing to disclose. Dr. Bershad has nothing to disclose. Dr. Suarez has received personal compensation for activities with Bristol-Myers Squibb/Sanofi and Boehringer Ingelheim as a speaker. Dr. Suarez has received research support from the Haas Trust and NINDS.

Acute bilateral medial medullary infarct with hypoplastic vertebral artery

The patient was a 60-year-old man who had hypertension or ten years and was not on antihypertensive medication. He ad history of transient ischaemic attack 15 years ago. He was a on-smoker and did not drink alcohol. On the day of admission, e presented with sudden onset of “choking sensation” followed y vomiting and slurring of speech. Within an hour, he became rowsy and was not moving the left upper limb and left lower imb. On arrival to casualty, he was noted to have inspiratory strior. His blood pressure was 204/100 mmHg with pulse rate of 5/min (regular). He was afebrile, but was tachypnoeic with oxyen saturation of 97% on high flow mask (with 15 L of oxygen). His apillary blood glucose was 8.5 mmol/L. In view of respiratory disress with stridor, he was intubated and ventilated in intensive care nit. On minimal sedation, he was able to open both eyes sponaneously and was able to obey one step command. There was eft gaze preference with left horizontal nystagmus. There was no symmetry on elevation of soft palate but his gag reflex was weak

Reversible Parainfectious Bilateral “Striatal Necrosis”

Bilateral striatal necrosis is usually associated with either endogenous or exogenous toxins, and with poor neurodevelopmental outcomes. We describe two patients with acute bilateral striatal clinical syndrome and magnetic resonance signal changes who made a complete clinical and radiologic recovery within 3 months. After an uneventful pregnancy, normal birth, and normal development, both boys presented at ages 3 and 5 years, respectively, after a viral illness with slurring of speech, bradykinesia, and an extrapyramidal movement disorder. On examination, both manifested bilateral cog wheel rigidity, with a broad-based gait and flexor plantar response. Cranial magnetic resonance imaging in both children indicated bilateral, symmetric, high signal changes in the lentiform nucleus, predominately in the putamen, with sparing of the globus pallidi bilaterally. The brain parenchyma was otherwise normal. Neurometabolic investigations produced normal results in both patients. The pathogenesis is uncertain, but could be immune-mediated. Both children, at 3-year and 1-year follow-ups, respectively, are doing well neurologically and academically. Our patients demonstrate that abnormal imaging findings during acute stages do not preclude good clinical and radiologic recovery.

A child with mutism after bilateral thalamic infarction

The occurence of mutism after thalamic lesions has rarely been observed before adulthood. We report a 6.5-year-old girl who presented with sudden mutism with a decreased level of alertness. Her MRI revealed a T2-fluid attenuated inversion recovery hyperintensity in both thalami, which suggested bithalamic infarction in the territory of the thalamo-perforating arteries and, more specifically, the para-median territories. Mutism was followed by a deficit of speech initiation with slow and slurred speech. Her speech returned to normal at 3 months after the onset of symptoms. Thus mutism could be the equivalent of akinetic mutism limited to the speech sphere.

Plasmapheresis and Cyclophosphamide therapy in an eight year old girl with cerebral manifestation of systemic lupus erythematodes

An eight year old girl suffering from systemic lupus erythematodes (SLE) presented with fever and developed a slurred and slowed speech, followed by hemiparesis of the right side, tremor of the arms and an instable gait. Diagnosis of SLE was done two years ago during an episode of muscle pain and polyarthralgia. From December 2008 until August 2009 the girl has received corticosteroids, naproxen and methotrexate. The cerebral magnetic resonance imaging (MRI) showed multiple T2-weighted hyperintense lesions in the cerebellum, periventricular on the right hemisphere, putamen bilateral and white matter of the frontal lobe. The blood results revealed a consumption of the complement system with decreased values of C3, C4 and CH50, increases of serum inflammatory factors, high values of antinuclear antibodies and of antibodies against dsDNA. Antibodies against SSA (Ro), SSB (La), u1RNP and SM have also been positive for a long time. Antibodies against phospholipids were not detected. High doses of corticosteroids (30 mg/kg/day methylprednisone) have been applied for three days and were followed by an apparent improvement of the hemiparesis. Speech worsened and an organic psychosyndrome developed with confusion, aggressiveness and a loss of short term memory and orientation. Plasmapheresis therapy on three consecutive days was performed and was followed by a prompt improvement of the organic psychosyndrome. After the second plasmapheresis procedure the girl developed an insult with a seizure. An MRI showed new hyperintense T2 lesions in the occipito-parieto-temporal area and in the frontal lobe in the white matter without restricted diffusion. Most of the known former lesions showed a dramatic improvement. Nine days after plasmapheresis and seven days after the first cyclophosphamide therapy the patient showed an impressive clinical improvement. Four weeks after the first symptoms the hyperintense lesions were in regression and the complement levels were within normal limits. The immunosuppressive therapy consisted of daily corticosteroids and intravenous cyclophosphamide in four weekly intervals and a continuous anticoagulation has been applied. Now ten months after the severe cerebral manifestation of SLE, the girl is well and going to school without any neurological handicap.

A longitudinal study of neurocysticercosis through CT scan of the brain

Objective To find out the role of CT scan in the diagnosis of neurocysticercosis (NC) and also to get an idea of the nature and extent of this disease in a definite geographical ethnic location. Methods CT scan of the brain of altogether 4 762 persons residing in a congested area of central Kolkata, was performed between 2004 and 2010, in an imaging centre in central Kolkata (Calcutta), of whom 1 114 (23.39%) suspected patients were investigated for NC, having complaints of convulsion (472), headache (272), right sided weakness (98), loss of consciousness (84), left sided weakness (61), abnormal behavior (48), slurring of speech (34), difficulty with keeping balance (33) and dementia (12). Results NC was detected in 45, 1, 0, 2, 1, 0, 0, 1 and 0, respectively consisting 4.48% (50/1 114) of the suspected cases varying from as low as 2 (in 2006) to as high as 23 (in 2005) in different years indicating this parasitic infection is perennial. The age of patients varied from 6 to 59 years of whom 28 (56%) were vegan. We were also able to detect 4 types of lesions viz. calcified lesions (14), small hypodense i.e. vesicle (12), bright central spot i.e. vesicular lesion with central spot (2) and ring enhancement (22). For 3 patients, further imaging investigation (MRI) was needed to confirm the diagnosis. We calculated the specificity and sensitivity of CT scan of brain diagnosing NC which was found to be 64% and 100%, respectively. Conclusions Although, the sample may not be the representatives of the whole community, the findings suggest that cysticercosis is a significant but under-recognized public health concern in Kolkata.

Transient Ischemic Attack Associated With Trazodone Therapy

To the Editor: Trazodone is a tetracyclic antidepressant commonly prescribed for insomnia. We report the emergence of neurologic symptoms suggestive of transient ischemic attack (TIA) in a patient following the initiation of trazodone. To the best of our knowledge, no reports of such an occurrence have been published. Case report. Mr A, a 40-year-old man with a history of mood disorder not otherwise specified (DSM-IV criteria), was admitted to a psychiatric inpatient unit in 2010 for suicidal ideation and depressed mood. He developed depressive symptoms about a year prior to this presentation when he got laid off from his job of over 4 years. Mr A had attempted suicide twice in the preceding year (the first time, he attempted to asphyxiate himself, and the second time, he overdosed on hydrocodone tablets) and was hospitalized at a psychiatric inpatient unit on both occasions. During his most recent admission (2 months prior to this presentation), he was started on fluoxetine treatment, which he discontinued after discharge. The stressors precipitating his current admission included homelessness, unemployment, financial constraints, and impending divorce. He endorsed depressed mood, decreased interest, poor energy, sleep difficulties, poor appetite, and suicidal ideation with a plan to stab himself. He denied auditory or visual hallucinations, psychotic symptoms, and drug or alcohol abuse. He had experimented with cocaine and marijuana during high school. There was no evidence of homicidal ideation or delusions. His medical history was significant for irritable bowel syndrome and tinnitus. Findings of his physical examination were unremarkable, and he was not taking any medications at the time of presentation. He did not tolerate valproic acid and citalopram initiated during this admission. He tolerated lithium carbonate 300 mg 3 times daily but complained of insomnia. He reported a past history of nonresponse to zolpidem. Five days after the admission, he was started on trazodone 50–100 mg at bedtime as needed for insomnia. Twelve hours later, he developed slurred speech, neck pain, facial tingling sensations, vertigo, dizziness, lack of coordination, muscle weakness, blurred vision, and diplopia. His serum lithium level was 0.5 mEq/L. An immediate computed tomography scan of the head was performed, which was negative for hemorrhage or ischemia. He was unable to walk and required wheelchair support. We discontinued trazodone, which led to rapid resolution of all his neurologic symptoms, except for his dizziness, within 24 hours. Neurologic examination on day 2 revealed no abnormalities, but the neurologist recommended magnetic resonance imaging of the brain and angiography of the neck to rule out stroke; findings of both tests were within normal limits. He received a single 325-mg dose of aspirin and was started on meclizine 25 mg/d. His dizziness improved on day 2 after cessation of trazodone therapy. We decided to continue lithium since the serum lithium level was low and all his symptoms resolved. A temporal relationship between drug initiation and emergence of neurologic symptoms and, most importantly, complete remission on discontinuation suggests TIA possibly as an adverse event following trazodone use. The literature shows that serotonergic drugs cause cerebral vasoconstriction. Paroxetine, a selective serotonin reuptake inhibitor (SSRI), has caused TIA and stroke.1–3 It was postulated that paroxetine and other SSRIs may result in changes of the vasculature and subsequent ischemic events in predisposed patients.1 Normally, serotonin secreted by platelets stimulates 5-HT2A receptors, which mediate vasoconstriction. This is counterbalanced by the release of the vasodilator nitric oxide upon serotonin stimulation of endothelial 5-HT1 receptors. In addition to their serotonergic activity, SSRIs such as paroxetine weakly inhibit norepinephrine reuptake and nitric oxide production, which increases the risk of cerebrovascular events.1 The combination of lithium, trazodone, and paroxetine as well as the combination of lithium, trazodone, and bupropion has been implicated in stroke.2 It is possible that the combination of lithium and trazodone in our patient has a role in the evolution of vasoconstriction, given their serotonergic effects. Similarly, ergot derivatives, amphetamine, and cocaine cause stroke due to their sympathomimetic and serotonergic effects.4 Trazodone possibly increases the serotonin release from platelets by its antagonistic and reuptake inhibitor effect at serotonin receptor, which may cause vasoconstriction. Trazodone has an effect on vascular and coagulation system like that of SSRIs, which may implicate that trazodone is associated with cerebrovascular accidents. Our case emphasizes that when vasoconstriction is suspected, serotonergic drugs should be discontinued.

Pontine stroke presenting as isolated facial nerve palsy mimicking Bell's palsy: a case report

IntroductionIsolated facial nerve palsy usually manifests as Bell's palsy. Lacunar infarct involving the lower pons is a rare cause of solitary infranuclear facial paralysis. The present unusual case is one in which the patient appeared to have Bell's palsy but turned out to have a pontine infarct.Case presentationA 47-year-old Asian Indian man with a medical history of hypertension presented to our institution with nausea, vomiting, generalized weakness, facial droop, and slurred speech of 14 hours' duration. His physical examination revealed that he was conscious, lethargic, and had mildly slurred speech. His blood pressure was 216/142 mmHg. His neurologic examination showed that he had loss of left-sided forehead creases, inability to close his left eye, left facial muscle weakness, rightward deviation of the angle of the mouth on smiling, and loss of the left nasolabial fold. Afferent corneal reflexes were present bilaterally. MRI of the head was initially read as negative for acute stroke. Bell's palsy appeared less likely because of the acuity of his presentation, encephalopathy-like imaging, and hypertension. The MRI was re-evaluated with a neurologist's assistance, which revealed a tiny 4 mm infarct involving the left dorsal aspect of the pons. The final diagnosis was isolated facial nerve palsy due to lacunar infarct of dorsal pons and hypertensive encephalopathy.ConclusionThe facial nerve has a predominant motor component which supplies all muscles concerned with unilateral facial expression. Anatomic knowledge is crucial for clinical localization. Bell's palsy accounts for around 72% of facial palsies. Other causes such as tumors and pontine infarcts can also present as facial palsy. Isolated dorsal infarct presenting as isolated facial palsy is very rare. Our case emphasizes that isolated facial palsy should not always be attributed to Bell's palsy. It can be a presentation of a rare dorsal pontine infarct as observed in our patient.

Dysautonomia From Bilateral Carotid Artery Dissection

The mechanisms of dysautonomia from carotid artery dissection were discussed recently in Circulation . A case was presented of a 49-year-old woman with a history of hypertension and vasovagal syncope who developed abrupt slurred speech and left hemiplegia.1 She had a right internal carotid artery dissection and right cerebral hemispheric infarction involving the insular cortex. During her hospitalization, the patient had episodes of severe bradycardia that Dulay et al1 attributed to “mechanical pressure to an already sensitive carotid sinus from thrombus secondary to a dissection of the internal carotid.” Sarikaya et al2 argued that disrupted descending autonomic pathways from the infarcted insular cortex were responsible. Right-sided insular lesions are known to cause bradyarrhythmias.3 We present a case of bilateral carotid artery dissection in which dysautonomia occurred in the absence of cortical damage. A 47-year-old right-handed woman with rheumatoid arthritis experienced symptoms of an apparent upper respiratory infection with a persistent cough after the syndrome resolved. After …

Persistent, severe hypoglycemia-induced organic brain syndrome with neurological sequelae: a case report

Hypoglycemia is a biochemical abnormality and often the rate-limiting step in the treatment of both type 1 and type 2 diabetes mellitus. Left uncorrected and prolonged, hypoglycemia can result in neuronal dysfunction and death, with deficits ranging from measurable cognitive impairments to aberrant behavior, seizures and coma. In this case report, hypoglycemia resulted in severe and persistent neurological (slurred speech and gait abnormalities), cognitive (inattention, disorientation and memory deficits) and behavioral manifestations (verbal hostility and irritability). It highlights the potentially severe neuropsychiatric sequelae following hypoglycemia and is timely for clinicians to be reminded that hypoglycemia prevention needs to be more of a focus of diabetes care in general.