Slurred Speech Research Articles

Lingual mucosal graft substitution urethroplasty for anterior urethral strictures: our technique of graft harvesting

To describe our technique of lingual mucosal graft harvesting for substitution urethroplasty and the complications encountered at the donor site. Twenty-eight patients who underwent lingual mucosal graft urethroplasty between May 2006 and March 2007 were included in this study. The site of the graft harvest is the lateral mucosal lining of the tongue. Graft harvesting is started from the posterior landmark of the graft on the left side of the tongue. It is continued across the tip of the tongue to the other side if lengthier graft is required. The graft harvesting site is simultaneously closed with continuous running suture using 4-0 polyglactin suture to achieve immediate and good homeostasis. Mean duration of follow up was 4.2 months. Average length of harvested graft was 6.5 cm. Mean harvesting time was 18 min. At the first postoperative day, 92% patients experienced pain at donor site and 24% had slurring of speech. By third postoperative day, >70% were free of pain and four had slurring of speech. By sixth postoperative day, none of the patient suffered pain. All the patients were able to resume oral fluid within 24 h, eat soft solid diet in 48-72 h and return to normal diet after 4-5 days of surgery. No patient suffered from difficulty in opening the mouth, salivation disturbances, peri-oral numbness or difficulty in protrusion of tongue. Lingual mucosal graft harvesting is a simple procedure, provides lengthy graft and is associated with minimal donor site complications.

Brown-Vialetto-Van Laere syndrome

The Brown-Vialetto-Van Laere syndrome (BVVL) is a rare neurological disorder characterized by progressive pontobulbar palsy associated with sensorineural deafness. Fifty-eight cases have been reported in just over 100 years. The female to male ratio is approximately 3:1. The age of onset of the initial symptom varies from infancy to the third decade. The syndrome most frequently presents with sensorineural deafness, which is usually progressive and severe. Lower cranial nerve involvement and lower and upper motor neuron limb signs are common neurological features. Other features include respiratory compromise (the most frequent non-neurological finding), limb weakness, slurring of speech, facial weakness, and neck and shoulder weakness. Optic atrophy, retinitis pigmentosa, macular hyperpigmentation, autonomic dysfunction, epilepsy may occur. The etiopathogenesis of the condition remains elusive. Approximately 50% of cases are familial, of which autosomal recessive is suggested. The remaining cases are sporadic. The diagnosis is usually based on the clinical presentation. Investigations (neurophysiological studies, magnetic resonance imaging of the brain, muscle biopsy, cerebrospinal fluid examination) are done to exclude other causes or to confirm the clinical findings. The differential diagnoses include the Fazio-Londe syndrome, amyotrophic lateral sclerosis, Nathalie syndrome, Boltshauser syndrome and Madras motor neuron disease. Treatment with steroids or intravenous immunoglobulin may result in temporary stabilization of the syndrome. However, the mainstays of management are supportive and symptomatic treatment, in particular assisted ventilation and maintenance of nutrition via gastrostomy. The clinical course of BVVL is variable and includes gradual deterioration (almost half of cases), gradual deterioration with stable periods in between (a third of cases) and deterioration with abrupt periods of worsening (just under a fifth of cases). After the initial presentation, one third of patients survive for ten years or longer.

ORAL COMPLICATIONS AFTER LINGUAL MUCOSAL GRAFT HARVEST FOR URETHROPLASTY

The aim of this study was to assess the complications at donor site after lingual mucosal graft harvesting for urethroplasty. From March 2006 to December 2006, 30 patients of anterior urethral stricture underwent lingual mucosal graft urethroplasty. The site of the harvest graft was lateral mucosal lining of the tongue. Donor site complications, that is, pain, slurring of speech, pain during speech, salivatory changes and difficulty in protrusion of tongue were noted. The mean (range) age of patients was 36.2 years (22-52 years). The mean (range) stricture length was 8.4 cm (4.8-16 cm) and graft length was 8.5 cm (4.2-16.2 cm). Mean duration of follow up was 3.8 months. At the first postoperative day, 90% of the patients experienced pain at donor site and 20% had slurring of speech. Pain was mild to discomforting in 80% and distressing to horrible in 13% of the patients. By third postoperative day, two-thirds were pain free, one-thirds had mild pain only and none had slurring of speech. By day 6 of surgery, all patients were pain free. Six per cent of the patients reported numbness over ventral aspect of anterior half of tongue, which persisted in the first follow up and subsided by second follow up. There was no bleeding, haematoma or infection at donor site. All patients were able to resume oral fluid within 24 h, eat soft solid diet in 48-72 h and return to normal diet after 4-5 days of surgery. No patient complained of difficulty in opening the mouth, salivation disturbances, perioral numbness or difficulty in protrusion of tongue. No long-term functional or aesthetic complications were reported. Lingual mucosal graft harvesting is feasible, provides a long graft, is easy to carry out and is the least morbid procedure.

Successful amelioration of oxaliplatin-induced hyperexcitability syndrome with the antiepileptic pregabalin in a patient with pancreatic cancer

Oxaliplatin, a platinum derivative used in the treatment of gastrointestinal cancers, has been associated with sensory neuropathies and, more infrequently, a neuromyotonia-like hyperexcitability syndrome. We present a case of hyperexcitability syndrome that developed during the treatment of pancreatic cancer with oxaliplatin and gemcitabine (GEMOX) that was successfully treated with pregabalin. A 54-year-old woman was undergoing chemotherapy with gemcitabine and oxaliplatin (GEMOX) for stage II-B pancreatic adenocarcinoma. On the third day of her fourth cycle, she presented with twitching of eyelids and tremors of hands. This twitching started bilaterally on the eyelids, followed by teeth jittering, hand shaking, and slurring of speech. A thorough neurological exam revealed no abnormalities except increased tone of both hands-she had difficulty opening her hand after closing it for a hand-grip. She was given a dose of 1 g of IV magnesium sulfate and 1 g of IV calcium gluconate, and 50 mg of IV diphenhydramine. In addition to reassurance, pregabalin was prescribed for these myotonic symptoms at a dosage of 50 mg by mouth three times daily. Improvement occurred in these symptoms within 12 h and she was almost asymptomatic within 72 h. Oxaliplatin causes a unique spectrum of acute neurological toxicities that have not been observed in patients receiving either cisplatin or carboplatin. Clinically, sensory alterations are most prominent, particularly cold-induced and perioral paresthesias. Other symptoms, such as cramps, jaw stiffness, voice changes, ptosis, and visual field changes suggest that motor nerves or muscles may also be involved (hyperexcitability). Hyperexcitability syndrome, distinct from cold-induced paresthesias and sensory neuropathy, is a rare complication of oxaliplatin chemotherapy; and up to date no pharmacotherapy has been successful in treating these symptoms. This is the first report of the successful amelioration of this syndrome with the antiepileptic pregabalin.

Slowly progressive hemiparesis

A 44-year-old Chinese man was admitted with right-sided weakness of 2 days’ duration. He had noted “absent-mindedness” for one month, developing word-finding difficulties, right-sided clumsiness and slurring of speech over the next 2 weeks, culminating in hemiparesis which resulted in gait difficulties and facial asymmetry. He had poor appetite and had lost 3 kg in weight over 1 month, but had neither change of bowel habits nor fever. Clinical examination was significant for right hemiparesis of MRC grade 4/5 and upper motor neuron palsy of the right facial nerve.

Quetiapine-Induced Hypersalivation

Sir: Quetiapine fumarate is a dibenzothiazepine-derivative antipsychotic with combined antagonism at dopamine (D1, D2) and serotonin type 1 and type 2 (5-HT1A, 5-HT2A, 5-HT2C) receptors. Additionally, quetiapine antagonizes α1- and α2-adrenergic and histamine H1 receptors. Protean binding at the above receptors explains the common side effects, which include hypersomnolence, hypotension, headache, weight gain, and gastrointestinal disturbance. However, to our knowledge, there have been no case reports of quetiapine-induced hypersalivation. We report the case of a patient with schizoaffective disorder taking low-dose quetiapine who developed hypersalivation, which remitted after discontinuation of the drug. We suggest that quetiapine antagonism at the α2 receptor contributes to the phenomenon of hypersalivation. Case report. Ms. A, a 36-year-old woman of Korean descent with a 16-year history of schizoaffective disorder, was admitted to the adult psychiatric medical care unit with a relapse of mania with psychosis. The patient's symptoms included elated mood, grandiose delusions of being a Power Ranger, disorganized behavior, and decreased need for sleep. The patient had been previously stabilized on clozapine with the only noted side effect being increased sedation. During this hospitalization, the patient refused administration of clozapine and instead was started on risperidone. Within a few days of commencement, the patient developed extrapyramidal symptoms with rigidity, dystonia, and slurring of speech, which prompted discontinuation of the risperidone. After a drug-free period of 3 days, the patient was started on quetiapine 25 mg b.i.d., and within a day, she developed increased salivation. When the dosage was increased to 50 mg b.i.d., the salivation worsened; the patient was drooling most of the day, and all of her clothing was wet. Benztropine (1 mg p.o. b.i.d.) and atropine eye drops (0.1%) sublingually did not reduce the hypersalivation. The quetiapine was stopped, and 1 day later, the hypersalivation resolved. Based on the temporal relationship between the administration and discontinuation of quetiapine and the onset and cessation of the patient's hypersalivation, it is possible that the hypersalivation was induced by the quetiapine. Hypersalivation is a common and distressing side effect seen with many psychotropic medications. However, no previous reports have associated increased salivation with the use of quetiapine. We attempt to explain the mechanism behind this event using the drug's known receptor affinities and the resultant binding effects observed in a closely related antipsychotic agent. Among the antipsychotics, clozapine is well recognized for inducing increased, voluminous salivation early in the course of treatment in up to 54% of patients.1 Clozapine is structurally similar to quetiapine and possesses a comparably broad receptor profile, which includes antagonistic activity at dopamine (D1, D2, D3, D4) receptors, adrenergic blockade activity at the α1 and α2 receptors, and antagonism at serotonin 5-HT2 receptors and histamine H1 receptors. However, unlike quetiapine, clozapine possesses both agonistic and antagonistic properties at muscarinic receptors. Specifically, clozapine has antagonist activity at M1, M2, M3, and M5 receptors, but exhibits agonist effects on the M4 receptor. One proposed mechanism for clozapine-induced hypersalivation focuses on its antagonism of α2-adrenergic receptors.2 Through blockade of sympathetic stimulation, parasympathetic stimulation is left unopposed to cause high salivary flow rates. Direct parasympathetic stimulation through its agonist effects at the M4 receptor has also been suggested to explain clozapine-induced hypersalivation.3 Quetiapine and clozapine both share blocking affinity at α2-adrenergic receptors, whereas only clozapine has agonist properties at the M4 receptor. We thus propose that α2-adrenergic blockade is the mechanism for quetiapine-induced hypersalivation. Further study is needed to draw definite conclusions regarding this proposed relationship.

Malignant external otitis with extensive cranial neuropathy but no facial paralysis

Sirs: Malignant or necrotizing external otitis (MEO) is a lifethreatening Pseudomonas aeruginosa infection of the external auditory canal leading to skull base osteomyelitis and soft tissue inflammation of the infratemporal fossa. Although MEO primarily belongs to the domain of the earsnose-throat specialist, it represents a true neurological emergency if cranial nerves are affected. The disease is characterised by the following triad: (A) An elderly diabetic or otherwise immunocompromised patient presents with (B) persistent headache, otalgia and auditory discharge after water irrigation for cerumen impaction, (C) complicated by multiple cranial nerve palsies. Due to its close proximity to the external acoustic meatus and the stylomastoid foramen, the facial nerve is the first and foremost involved cranial nerve [1]. However, here we report to our knowledge the first case of a patient with MEO suffering from extensive cranial neuropathy without facial paralysis. In November 2005 a 68-year old man with poorly regulated diabetes mellitus and hypertension consulted an otolaryngologist because of left ear deafness. After irrigation for cerumen impaction, hearing returned to normal. A week later he developed constant left-sided headache and painful drainage of a yellowish smelly fluid from the left ear. Otalgia and otorrhoea resolved two weeks later after repeated irrigations and the prescription of topical hydrocortisone cream. However, headache persisted. The pain extended to the left temporo-mandibular joint and mastoid. In December the patient noticed hypestesia on the left side of the face. In January 2006 he was operated for an abscess of the left external auditory canal. In February he developed slurred speech and hoarseness, in March difficulties with swallowing. He lost six kg in three weeks and complained of constant headache. He was now referred by his otolaryngologist to a university neurological department. On admission he was alert and orientated without fever or neck stiffness. Neurological examination revealed a sensory deficit in the maxillary and mandibular nerve distribution, mild conductive hearing impairment, deviation in the Unterberger’s stepping test, a positive curtain sign with elicitation of the gag reflex, dysphagia, hoarseness and tongue deviation. All symptoms were confined to the left side. Of note, there was no facial paresis. A CT and MRI scan of the head revealed bone erosion of the left occipital condyle and mastoid with extensive soft tissue inflammation around the skull base (Figs. 1, 2); however, the facial nerve remained unaffected (Fig. 1). A diagnosis of MEO with involvement of multiple cranial nerves was made and the patient received ciprofloxacin 750 mg twice daily. Two days later the headache had vanished. After three more days dysphagia and hoarseness had resolved. Culture from a mastoid biopsy revealed Pseudomonas aeruginosa. The patient received eight weeks of antibiotic treatment and made an uneventful recovery. Although MEO affects mostly elderly male diabetics, due to increased frequency of immunosuppression MEO is now seen in patients with AIDS or malignancies as well. When left untreated, MEO has a mortality of more than 50% [1], but since the introduction of ciprofloxacin and other oral antipseudomonal agents in the 1990ies, survival rates are much better. Long-term systemic therapy (>6 weeks) is mandatory and excellently tolerated [2]. Local application of aluminium acetate, known as Burow’s solution [3], might be used additionally. Moreover, hyperbaric therapy has been widely advocated [4, 5], but a recent Cochrane Review concluded that there is so far no evidence from randomized controlled trials for such an approach [6]. Due to undifferentiated ciprofloxacin use for simple external otitis, aberrant and recurrent forms of MEO and even quinolone-resistance are increasingly reported. Sinus and jugular vein thrombosis, meningitis, trismus, facial nerve palsy and other cranial neuropathies, ophtalmoplegia and skull base osteomyelitis often necessitate surgical debridement. Even with aggressive surgical and medical treatment mortality of protracted MEO is high [7]. Thus, early diagnosis of MEO is essential. CT, MRI and SPECT bone imaging provide the anatomic D. Kondziella, MD, DMSC (&) I. Skagervik, MD Dept. of Neurology Goteborg University Hospital Sahlgrenska 41345 Goteborg, Sweden Tel.: +46-730791599 Fax: +46-31826227 E-Mail: daniel_kondziella@yahoo.com LETTER TO THE EDITORS J Neurol (2007) DOI 10.1007/s00415-006-0516-1

Priapism in the Oncology Setting

N.L. is a 20-year-old Caucasian man with no significant medical history. He presented to the hospital with a two-day history of feeling ill with intermittent nausea, vomiting, blurred vision, and headache. He also noted slurred speech and left-upper-quadrant fullness for almost a month.

What's Your Diagnosis?

<h4>The Case</h4> <p>A 15-year-old girl was depressed. A physical examination detected splenomegaly, and a blood test revealed leukopenia and thrombocytopenia. Splenectomy and open liver biopsy were performed, revealing a hepatic cirrhosis. At 16, she became uncoordinated, developed slurred speech, and had micrographia, rigidity of the upper limbs, and an expressionless face. After several misdiagnoses, a physician suggested an ophthalmologic examination to confirm his suspected diagnosis. On ocular examination, her visual acuity was 20/20 in both eyes. Anterior segment examination revealed a perilimbal corneal golden-brown discoloration in Descemet’s membrane (Figure). The lenses, extraocular movements, and fundi were normal. Following correct diagnosis and initiation of medical treatment, her signs and symptoms resolved and the corneal abnormality decreased significantly.</p>

Ophthalmoplegia and Slurred Speech in an Intravenous Drug User

A 35-year-old heroin user presented with acute, progressive diplopia and slurred speech. Krishna and colleagues discuss the diagnosis and management of this patient.

Glutamate uptake block triggers deadly rhythmic bursting of neonatal rat hypoglossal motoneurons

In the brain the extracellular concentration of glutamate is controlled by glial transporters that restrict the neurotransmitter action to synaptic sites and avoid excitotoxicity. Impaired transport of glutamate occurs in many cases of amyotrophic lateral sclerosis, a devastating motoneuron disease. Motoneurons of the brainstem nucleus hypoglossus are among the most vulnerable, giving early symptoms like slurred speech and dysphagia. However, the direct consequences of extracellular glutamate build-up, due to uptake block, on synaptic transmission and survival of hypoglossal motoneurons remain unclear and have been studied using the neonatal rat brainstem slice preparation as a model. Patch clamp recording from hypoglossal motoneurons showed that, in about one-third of these cells, inhibition of glutamate transport with the selective blocker dl-threo-beta-benzyloxyaspartate (TBOA; 50 mum) unexpectedly led to the emergence of rhythmic bursting consisting of inward currents of long duration with superimposed fast oscillations and synaptic events. Synaptic inhibition block facilitated bursting. Bursts had a reversal potential near 0 mV, and were blocked by tetrodotoxin, the gap junction blocker carbenoxolone, or antagonists of AMPA, NMDA or mGluR1 glutamate receptors. Intracellular Ca(2+) imaging showed bursts as synchronous discharges among motoneurons. Synergy of activation of distinct classes of glutamate receptor plus gap junctions were therefore essential for bursting. Ablating the lateral reticular formation preserved bursting, suggesting independence from propagated network activity within the brainstem. TBOA significantly increased the number of dead motoneurons, an effect prevented by the same agents that suppressed bursting. Bursting thus represents a novel hallmark of motoneuron dysfunction triggered by glutamate uptake block.

Secondary Mania in Older Adults

Although mania is commonly associated with bipolar disorder, it can have many etiologies (1). Thus, “primary mania” results from bipolar disorder, whereas “secondary mania” results from pharmacological, metabolic, or neurologic causes (1, 2). Older adults are at risk for secondary mania because of increased medical comorbidities and neurological changes. In one retrospective study of 50 patients with mania who were older than 65 years, it was the first manic episode for 28% of the patients and 71% had a comorbid neurological disorder (3). The etiology of mania is important because although acute symptomatic treatment of both primary and secondary mania may be similar, appropriate treatment of secondary mania includes addressing the cause (1). We present here two case histories of secondary mania in older adults, discuss their presentations and differential diagnosis in turn, and discuss treatment.

Neurocardiogenic Syncope and Related Disorders of Orthostatic Intolerance

Received September 14, 2004; revision received January 31, 2005; accepted March 9, 2005. “We shall not cease from exploration and the end of all our exploring will be to arrive where we started and know the place for the first time” — —T.S. Eliot, Four Quartets Syncope, defined as transient loss of consciousness and postural tone with spontaneous recovery, has both challenged and perplexed physicians since the dawn of recorded time. The earliest written accounts come from Hippocrates, and the word syncope itself is derived from an old Greek term meaning “to cut short” or “interrupt.” Recurrent episodes of syncope may result from a large number of different disorders, all of which cause a transitory reduction in cerebral blood flow sufficient to disturb the normal functions of the brain. Over the last 2 decades, considerable attention has been given to types of syncope that occur due to a centrally mediated (or “reflex”) fall in systemic blood pressure, a condition that has been referred to as vasovagal (and later neurocardiogenic) syncope. However, research into the nature of this disorder revealed that it is but one aspect of a broad and varied group of disturbances in the normal functioning of the autonomic nervous system (ANS), each of which may result in orthostatic intolerance, hypotension, and ultimately syncope. Continued investigations into the nature of these similar yet different disorders has led to the development of a system of classification that attempts to more accurately reflect our understanding of these conditions and their interrelationships.1 The present system of classification has proven both functional and clinically relevant and includes a group of disorders that most investigators have thought to be principally autonomic in nature. Because both the cardiologist and the cardiac electrophysiologist frequently are expected to both diagnose and treat these conditions, the following …

Spontaneous Intracerebral Hemorrhage Related to Methamphetamine Abuse

A healthy 31-year-old male abstinent from drug abuse during his recent incarceration developed slurred speech, a severe headache, and left-sided hemiparesis prior to his eventual death 9.5 hours after inhalation of methamphetamine. On postmortem examination, inspection of the brain revealed bilateral subarachnoid hemorrhage, with a prominent intralobar hemorrhage centered within the right frontal cerebral hemisphere. No evidence of vasculitis, infarction, intraventricular hemorrhage, or ruptured aneurysm could be observed. While this is not the first report of a methamphetamine-related stroke, this report describes the autopsy findings of an intracerebral hemorrhage secondary to methamphetamine abuse on autopsy and compares the findings and antemortem history to previously reported methamphetamine cerebral vascular deaths.

Personal history: cross-coverage.

So live, that when thy summons comes to join The innumerable caravan, which moves To that mysterious realm, where each shall take His chamber in the silent halls of death, Thou go not, like the quarry-slave at night, Scourged to his dungeon, but, sustained and soothed By an unfaltering trust, approach thy grave, Like one who wraps the drapery of his couch About him, and lies down to pleasant dreams. — —William Cullen Bryant, “Thanatopsis” I first met “Jane” when she “finally” agreed to accept a nasogastric tube at the tail end of my co-intern’s postcall day. This was just a few months into the year, before I knew to use scopolamine to quiet a death rattle, before I knew on a fundamental level that you don’t put a percutaneous gastrostomy tube in someone with days to live. Steve signed out at 7 pm after tracking me down in the nurses’ station. “I just can’t put in an NG tube now.” He dropped heavily into one of the swivel chairs, grimacing at the nighttime skyline visible from the fifth floor windows. “I’m beat. Do you mind?” I said I didn’t. “What’s it for?” “It’s freakish. She’s 45, been getting weaker, falling, slurred speech for a year , finally comes in to see a doctor 5 days ago and guess what, she has end-stage ALS. She’s in for a PEG and needs decompression with an NG tube overnight. She can barely manage any secretions. Oh, and she’s full code.” “Full?” I asked skeptically. “Why?” “Can’t let go,” Steve said. “Family heavily involved. I really have to go see my family for once. Thanks for doing the NG tube.” He rose and trudged off the ward. I went to see her shortly after. Her mother, brother, and sister sat on her bed …

Case 1: The girl who couldn't sit still

A seven-year-old Caucasian girl was referred to the hospital from the community with concerns about abnormal movements of her upper and lower extremities and slurring of speech. Before this, she had a two-week history of low grade fever, difficulty with feeding herself, deterioration in handwriting and trouble concentrating in school. She was seen by her family doctor at the onset and was believed to have had a viral infection. There was no history of a preceding sore throat or any concurrent symptoms such as headache, vomiting or fluctuating level of consciousness. Her past medical history was unremarkable. She was not taking any medications and there was nothing to suggest any intentional or accidental drug or poison ingestion. On examination, she was afebrile with stable vital signs. She was alert and cooperative but would not speak to hospital staff. She displayed facial grimacing, thrusting of the tongue and bilateral writhing nonpurposeful movements of her arms and legs, as well as the ‘milk maid’ sign when asked to squeeze the examiner’s fingers. She had a mild fleeting erythematous macular rash over her forearms. Cardiac auscultation revealed normal heart sounds, with no audible murmur or rub. The remainder of her examination was normal. Laboratory investigations showed a white blood cell count of 11.1×109/L (polymorphs 4.8×109/L, bands 1.1×109/L, lymphocytes 4.0×109/L), hemoglobin of 128 g/L, and platelets of 536×109/L. Her erythrocyte sedimentation rate was elevated at 30 mm/h (normal <10 mm/h). Throat swab, cerebrospinal fluid culture, blood and urine toxicology screens and a head CT scan were normal. Antinuclear antibody, anti-double stranded DNA antibody, and 24 h urine copper level and serum ceruloplasmin were normal. Anticardiolipin antibody and the lupus anticoagulant screen were both negative. A further laboratory test confirmed the diagnosis.

Erdheim-Chester Disease: Case Report and Review of Associated Urological, Radiological and Histological Features

A 48-year-old man presented with progressive gait ataxia, slurring of speech and bilateral leg weakness 6 months in duration. Neurological evaluation included computerized tomography (CT) of the head, magnetic resonance imaging and myelography, all of which were negative for neuromuscular deficiency. Positron emission tomography showed increased uptake in the radii and femora bilaterally. Presumptive diagnosis was Paget’s disease, and the symptoms stabilized for several months. However, the patient subsequently presented with bilateral flank pain and an increased serum creatinine of 4.5 g./ml. (normal 0.6 to 1.2 mg./dl.). CT of the abdomen demonstrated bilateral hydronephrosis and perinephric fat infiltration (fig. 1, A). Indwelling ureteral stents were placed and a diagnosis of Erdheim-Chester disease was considered. Plain x-rays of the extremities revealed cortical and medullary sclerosis of the distal radii and femora-findings classic for Erdheim-Chester disease (fig. 1, B). Laparoscopic retroperitoneal biopsy of the perinephric tissue showed nonLangerhans type histiocytosis (fig. 2). The patient is currently being treated with oral 6-mercaptopurine. He continues to require an indwelling ureteral stent.

Knowledge of stroke risk factors and warning signs among Michigan adults.

To assess the knowledge of stroke risk factors and warning signs in a representative statewide sample of Michigan adults. Respondents to the 1999 Michigan Behavioral Risk Factor Survey, a random-digit-dialed statewide survey of >2,500 adults, were asked to report up to three risk factors and warning signs for stroke. Predictors of inadequate knowledge (defined as not reporting any correct responses) of stroke risk factors and warning signs were identified using multiple logistic regression. Eighty percent reported at least one correct risk factor for stroke, and 28% reported three. The most frequently mentioned risk factors were hypertension (32%), smoking (29%), and physical inactivity (26%). Sixty-nine percent reported at least one correct warning sign of stroke, but only 14% reported three. The most frequently mentioned warning signs were sudden weakness or numbness (46%) and sudden slurred speech, disorientation, or difficulty understanding (30%). Predictors for inadequate knowledge of both stroke risk factors and warning signs were similar and included age, race, sex, education, hypertension, and smoking. Knowledge of stroke risk factors and warning signs was moderate at best. One in five respondents was not aware of any stroke risk factors, and almost one in three was not aware of any stroke warning signs. Stroke knowledge was poorest among groups that have the highest risk of stroke.

Fits and strokes

A 41-year-old right-handed engineer was admitted in 1998 with a 10-year history of absence seizures. The seizures were characterised by symptoms of olfactory deja vu. During the seizures witnesses reported that the patient was able to walk and undertake complex tasks. The seizures, of which the patient had no recollection, lasted for a few minutes and were followed by several hours of lethargy. They would occasionally occur following binge drinking. He had a past history of migraine with visual aura. One of the patient’s four siblings one was said to have “multiple sclerosis” but the exact nature of the diagnosis was not clear. His mother had thyroid disease and migraine and his father had diabetes but there was no other family history of note. On examination the patient had vitiligo. His BP was 120/80 mm Hg. He seemed to have brisker tendon reflexes on the left side and foot tapping was marginally diminished on the left. His abstract reasoning was mildly impaired. The remainder of the examination was normal. The following investigations were normal, negative or non-diagnostic: full blood count, liver function, thyroid function, ESR (8 mm/h), syphilis serology, blood lactate, amino acid profile, vitamin B12, folate, lupus anticoagulant, proteins C & S, activated protein C resistance, antibody to thrombin III, autoantibody screen, angiotensin converting enzyme concentrations in serum and cerebrospinal fluid (CSF), visual, brainstem and somatosensory evoked potentials, magnetic resonance imaging (MRI) of the neck vessels, and cardiac echocardiography. An electroencephalogram showed only mild non-specific left temporal disturbances of cerebral activity. CSF examination showed an opening pressure of 16·5 cm of water, glucose 4 mmol/L (peripheral 7 mmol/L), protein 0·96 g/L, lymphocytes 2 10/L, red cells 1 10/L, normal CSF lactate, and no oligoclonal bands. MRI of his head (figure) showed multiple areas of high signal in the white matter bilaterally and one in the pons posteriorly. These areas were confluent periventricularly. 4 months later the patient was readmitted following a sudden onset of unsteadiness of gait, diplopia, nausea, and vertigo. Over the next 48 h he developed slurred speech. On examination he had ataxia with multi-directional and rotatory nystagmus. There was marked cerebellar incoordination in the left arm. A repeat Fits and strokes

Diver with decompression injury, elevation of serum transaminase levels, and rhabdomyolysis

A 43-year-old female recreational scuba diver presented to the emergency department 1 hour after a rapid, uncontrolled ascent. Her presentation included progressing confusion, slow and slurred speech, and complaints of headache and hypesthesia over her forearms and anterior thighs bilaterally. Differential diagnosis included arterial gas embolism and decompression sickness. She underwent recompression therapy with US Navy Table 6 within 120 minutes of her ascent. After recompression therapy, the patient had signs and symptoms consistent with severe rhabdomyolysis, including creatine kinase levels of 36,000 U/L and myoglobinuria. [Shank ES, Muth CM. Diver with decompression injury, elevation of serum transaminase levels, and rhabdomyolysis. Ann Emerg Med. May 2001;37:533-536.]