Introduction.

Paraneoplastic syndromes represent rare symptom complexes resulting from the ability of tumour cells to disrupt the homeostatic processes of various bodily systems. Here we present two cases to demonstrate how such tumours may evade detection even after extensive investigation and how even relatively benign tumours can produce severe neurological symptoms. Case 1. A 69-year-old female was admitted with a subacute onset of dysarthria, ataxia, and cerebellar signs. Workup revealed a relatively benign Non-Hodgkin's Lymphoma. Case 2. A 64-year-old female was admitted with acute leg weakness, which progressed to quadriplegia and was eventually fatal over the ensuing months. Her Ca-125 was elevated, though three different CT views of her pelvis and surgical exploration failed to demonstrate any malignancy. Discussion. These cases highlight how even relatively benign or very small tumours may result in severe neurological symptoms. Suspecting and investigating paraneoplastic syndromes (PNSs) are crucial as up to 80% of patients present with PNS before there is any other indication of malignancy. A PET scan and regular surveillance may reveal occult malignancies better than CT or MRI. Neuromodulatory therapies and treatment of the underlying malignancy remain the best management options in these patients.

We report a case of atrial fibrillation in a patient in whom a mobile thrombus in the left atrial appendage increased in size after low-dose dabigatran therapy. A 74-year-old man was admitted to our hospital because of sudden onset of right hemiplasia and dysarthria. On admission, his National Institutes of Health Stroke Scale score was three. Axial diffusion-weighted magnetic resonance images and magnetic resonance angiography images showed hyperintense signals in the left front-parietal cerebral cortex without any intracranial stenotic lesions, and acute cardioembolic stroke associated with nonvalvular atrial fibrillation was diagnosed. Transesophageal echocardiography revealed a mobile thrombosis (1.0 × 2.2 cm) in the left atrial appendage, and dabigatran therapy (110 mg b.i.d.) was initiated to prevent stroke recurrence. Transesophageal echocardiography performed 6 days later revealed that the size of the thrombus had increased to 1.5 × 3.0 cm. Medication was changed to warfarin, and the thrombosis subsequently decreased in size. The patient did not have a recurrent stroke and was discharged with a National Institutes of Health Stroke Scale score of zero. This case demonstrates that low-dose dabigatran may not be effective in reducing the size of a thrombus.

INTRODUCTION:

Tongue protrusion dystonia can cause difficulty with speech, mastication, breathing, and swallowing. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a widespread therapeutic alternative for treating medically refractory dystonia. To our knowledge, detailed reports regarding DBS for tongue protrusion dystonia are rare. In this report, we describe two patients with "sticking out" tongue protrusion who had undergone bilateral GPi DBS.

METHODS:

Operations were performed with surface electromyographic (EMG) monitoring, microelectrode recording, and macrostimulation to identify the point at which tongue kinetic cells respond most effectively. The most effective location for active contacts was identified according to burst EMG response in the posteroventral GPi.

RESULTS:

Two years after DBS, total Burke, Fahn, and Marsden Dystonia Rating Scale scores of two patients were improved from 12.5 to 1 (92.0%) and from 13 to 1 (92.3%), respectively. One 58-year-old woman who lost 7 kg weight from not eating well improved enough to eat solid food and became free from choking. Another 54-year-old woman who had dysarthria and mumbled could speak more fluently and would not have complained difficulty in reading any more.

CONCLUSION:

Stimulation on posteroventral GPi for patients with idiopathic "sticking-out" tongue movement changes EMG pattern in orofacial muscles. This fact supports a reason for modulation of unknown circuit connecting tongue-specific area in motor cortex, and basal ganglia.

As Wilson's disease is both preventable and treatable, the diagnosis must not be missed. Despite this, it is usually misdiagnosed. Misdiagnosis and delay in treatment are clinically relevant because if left untreated, Wilson's disease progresses to hepatic failure or severe neurologic disability, and death. Those adequately treated have a normal life span. Wilson's disease is an autosomal recessive disease caused by mutations in the ATP7B gene. Mutations in ATP7B result in abnormal copper metabolism and subsequent toxic accumulation of copper. The clinical manifestations of neurologic Wilson's disease include variable combinations of dysarthria, dystonia, tremor, parkinsonism, ataxia, and choreoathetosis. Once the possibility of Wilson's disease is considered, diagnosis is straight forward. Currently available treatments, including zinc acetate and trientine, are generally well tolerated and effective.

Legionnaires' disease is frequently complicated by extrapulmonary symptoms such as neurological abnormalities. Cerebellar disorders are rare, but can be long-lasting and disabling.A 57-year-old male developed severe cerebellar disorders such as dysarthria and ataxia after being hospitalized for pneumonia. Despite an extensive diagnostic work-up, no explanation for the cerebellar symptoms could be found. The patient was transferred to a rehabilitation facility. Repeated serological tests revealed infection with Legionella, which was most probably the cause of the cerebellar dysfunction.Cerebellar disorders are a rare complication of Legionnaires' disease. The pathogenesis is unknown. There is no diagnostic test that proves or excludes cerebellar involvement in an infection caused by Legionella. The treatment is supportive and recovery can take months or even years.

Acute ischemic stroke is a potentially catastrophic medical emergency. Recently, successful reversal of the neurologic deficits associated with major ischemic strokes has been accomplished in selected patients through the use of intravenous tissue plasminogen activator (tPA), an agent that can accomplish thrombolysis of arterial clots if given within the first few hours after the onset of stroke. Because tPA works by thrombolysis of fresh clots, a potential exists for catastrophic hemorrhage if given to acute postoperative patients. Therefore, the use of tPA has never been studied in postoperative patients, and the safety of the drug in postoperative patients is unknown.The author describes a patient who had an acute ischemic stroke 2 days after total hip arthroplasty who was successfully treated with tPA without major complications. The patient was 51 years old and developed progressive facial droop, right arm paralysis, and dysarthria 2 days after elective hip arthroplasty. Imaging confirmed occlusion of the left middle cerebral artery. Neurologic recovery was believed to be unlikely without tPA. After tPA administration, the patient had full neurologic recovery within minutes but did develop a large (nondraining) hematoma and severe ecchymosis at the surgical site; a drop in hematocrit required 3 units of packed red blood cell transfusion. The wound did not develop skin necrosis, infection, or compartment syndrome, and the hematoma resolved within several weeks without the need for surgical intervention.The author describes the patient's specific circumstances, the decision-making process behind the use of tPA, and the need for contingency plans in the event that severe uncontrolled hemorrhage occurs. This information may be useful if other surgeons are faced with the dilemma of a major stroke in acute postoperative patients.

INTRODUCTION:

The normal startle response is a form of physiological myoclonus. Its anatomic origin is probably the brain stem. Pathologic startles are defined as reproducible exaggerated startle responses to trivial and not surprising stimuli. Symptomatic forms of an exaggerated startle response can be due to a variety of brain stem disorders. We have, however, found scant data about an exaggerated startle reflex induced by Lyme neuroborreliosis. We therefore report the case of a patient with this unusual presentation.

CASE PRESENTATION:

A 69-year old Caucasian man presented with a two-week history of a pronounced startle myoclonus, as well as a four-week history of double vision, gait disturbance and severe lancinating pain in his upper thoracic region. Neurological examination showed an excessive startle reaction of his upper trunk evoked by visual and tactile stimulation, a positive sign of Lhermitte, mild right-sided palsy of his sixth and seventh cranial nerve, moderate dysarthria, very brisk deep tendon reflexes, pallhypesthesia of his legs, and an atactic gait disturbance. A diagnosis of a Lyme neuroborreliosis was confirmed by cerebrospinal fluid examination. Under intravenous treatment with ceftriaxone, our patient improved considerably with complete remission in a follow-up at two months.

CONCLUSIONS:

This case illustrates the chameleon role that neuroborreliosis likes to play: although the wide spectrum of different symptoms that neuroborreliosis can present with has been described, to the best of our knowledge this is the first case report about a symptomatic form of a pathologic startle response as the predominating sign of Lyme neuroborreliosis.

A 41-year-old male with a history of human immunodeficiency virus (HIV) infection developed motor aphasia, dysarthria, and right hemiparesis. A magnetic resonance imaging scan of the brain revealed a cerebral infarction in the territory of the left middle cerebral artery. The laboratory data showed decreased levels of protein S and protein C. Transesophageal contrast-enhanced echocardiography revealed a patent foramen ovale (PFO). Prothrombotic states, such as protein S and C deficiency, have been reported in HIV-infected patients. In addition, previous studies have reported prothrombotic states to be risk factors for PFO-related cerebral infarction. An association between combined protein S and C deficiency caused by HIV infection and PFO-related cerebral infarction was suggested in our patient.

BACKGROUND:

Communication is frequently impaired in young people (YP) with bilateral cerebral palsy (CP). Important factors include motoric speech problems (dysarthria) and intellectual disability. Augmentative and Alternative Communication (AAC) techniques are often employed. The aim was to describe the speech problems in bilateral CP, factors associated with speech problems, current AAC provision and use, and to explore the views of both the parent/carer and young person about communication.

METHODS:

A total population of children with bilateral CP (n = 346) from four consecutive years of births (1989-1992 inclusive) with onset of CP before 15 months were reassessed at age 16-18 years. Motor skills and speech were directly assessed and both parent/carer and the young person asked about communication and satisfaction with it.

RESULTS:

Sixty had died, eight had other conditions, 243 consented and speech was assessed in 224 of whom 141 (63%) had impaired speech. Fifty-two (23% of total YP) were mainly intelligible to unfamiliar people, 22 (10%) were mostly unintelligible to unfamiliar people, 67 (30%) were mostly or wholly unintelligible even to familiar adults. However, 89% of parent/carers said that they could communicate 1:1 with their young person. Of the 128 YP who could independently complete the questions, 107 (83.6%) were happy with their communication, nine (7%) neither happy nor unhappy and 12 (9.4%) unhappy. A total of 72 of 224 (32%) were provided with one or more types of AAC but in a significant number (75% of 52 recorded) AAC was not used at home, only in school. Factors associated with speech impairment were severity of physical impairment, as measured by Gross Motor Function Scale level and manipulation in the best hand, intellectual disability and current epilepsy.

CONCLUSIONS:

In a population representative group of YP, aged 16-18 years, with bilateral CP, 63% had impaired speech of varying severity, most had been provided with AAC but few used it at home for communication.

Dysarthria is a group of speech disorders resulting from neurological disturbances in central or peripheral systems. There are six single types of dysarthria and all present with deviations at both segmental and suprasegmental level. However, it is unclear what matters more to the listener: the deficits at the segmental or suprasegmental level. In this study, reading samples were collected from subjects with any of the three types of dysarthria: scanning speech of ataxic dysarthria, spastic dysarthria, and hypokinetic dysarthria. All had slow speaking rate, monopitch, and monoloudness. Acoustic analyses were used to examine changes at both segmental and suprasegmental level. At the segmental level, parameters obtained include word and syllable per minute, vowel F1 and F2, syllable, word, sentence, and pause duration, mean F0 and vF0 at sentence and paragraph level. Peak F0 and vowel duration of stressed and unstressed vowels were also obtained. Perception experiment was conducted. Pitch contours were extracted and tested separately from those un-manipulated stimuli. Listeners made forced choice for rate and speech naturalness for the former and for overall speech intelligibility, speech rate, and speech naturalness for the latter. Effective size was used to determine the contributions of parameters at the segmental and suprasegmental level.