Adverse medical complications: an under-reported contributory cause of death in New York City.

OBJECTIVES: The current death certification system in the USA fails to accurately track deaths due to adverse medical events. The aim of this study was to demonstrate the under-reporting of deaths due to adverse medical events due to limitations in the current death certification/reporting system, and the benefits of using the term 'therapeutic complication' as the manner of death. STUDY DESIGN: Retrospective review and comparison of death certificates and vital statistical coding. METHODS: The manner of death is certified as a therapeutic complication when death is caused by predictable complications of appropriate therapy, and would not have occurred but for the medical intervention. Based on medical examiner records, complications that caused or contributed to deaths over a five-year period were examined retrospectively. These fatalities were compared with deaths coded as medical and surgical complications by the New York City Bureau of Vital Statistics. RESULTS: The Medical Examiner's Office certified 2471 deaths as therapeutic complications and 312 deaths as accidents occurring in healthcare facilities. In contrast, the New York City Bureau of Vital Statistics reported 188 deaths due to complications of medical and surgical care. CONCLUSIONS: Use of the term 'therapeutic complication' as the manner of death identified nearly 14 times more deaths than were reported by the New York City Bureau of Vital Statistics. If these therapeutic complications and medical accidents were considered as a 'disease', they would rank as the 10th leading cause of death in New York City, surpassing homicides and suicides in some years. Nationwide policy shifts that use the term 'therapeutic complication' would improve the capture and reporting of these deaths, thus allowing better identification of fatal adverse medical events in order to focus on and assess preventative strategies.

Multisystem involvement of the central nervous system in Strümpell's disease. A neurophysiological and neuropsychological study.

The multisystem involvement of the central nervous system (CNS) in familial spastic paraplegia (FSP) has not been fully investigated by means of complete neurophysiological and neuropsychological examinations. The classification which distinguishes pure and complicated forms of FSP, is based on clinical features and does not take into account the possibility that clinically silent lesions of the CNS can be identified by means of adequate tests. The study was intended to assess the subclinical and multisystem involvement of the CNS in a group of 11 patients affected by FSP, clinically distinguished in 7 pure forms and 4 complicated forms. Neurophysiological tests included saccadic eye movements analysis, visual and auditory brain stem evoked responses. Neuropsychological examination was devised by means of a special purpose mental deterioration battery. Our results, showing a high incidence of multisystemic subclinical involvement of the CNS, confirm and extend the concept that FSP is a multisystemic degenerative disease of the CNS, and that the existence of "pure" forms should be reconsidered.

Intranasal versus intravenous neostigmine in myasthenia gravis: assessment by computer analysis of saccadic eye movements.

Intranasal (i.n.) neostigmine has been developed to obtain a quicker onset of action and a more adaptable dosage regimen than oral neostigmine. The effect of this neostigmine formulation in myasthenia gravis (MG) was studied by means of computer analysis of saccadic eye movements (SEMs). Six MG patients were selected on the basis of a positive effect of Tensilon on hypometria of the first saccade. The effect of the i.v. formulation (0.5 mg) was compared to 1, 2, 3, and 4 puffs of i.n. neostigmine (one puff = 4.6 mg). The drug effect on SEMs was monitored at intervals up to 2 h. Administration of i.v. neostigmine produced a marked effect immediately after the injection and the benefit lasted over 1 h. Following administration of i.n. neostigmine a marked effect was found for two, three, and four puffs. The drug effect was evident within 3 min, peaked at 18-33 min, and lasted over 2 h. Our data indicate the efficacy of the new formulation of neostigmine in MG as measured by means of quantitative analysis of SEMs.

Hydromyelia secondary to spinal epidural abscess. A case report.

Spinal epidural abscess (SEA) is a rare condition that can be fatal if untreated. Risk factors are immunocompromised states as well as spinal procedures including epidural anesthesia and spinal surgery. The signs and symptoms of SEA are nonspecific and can range from low back pain to sepsis. The treatment of choice is surgical decompression followed by four to six weeks of antibiotic therapy. The most common causative organism in SEA is staphylococcus aureus and spread is usually haematogenous or contiguous from psoas, paraspinal or retropharyngeal abscesses. The exact mechanism by which an epidural abscess causes spinal cord damage is unclear. In fact, the damage is often out of proportion to the degree of compression demonstrated radiologically. There is only a report of a patient with syrinx formation secondary to epidural abscess. We describe the case of a 48-year-old woman with a two-week history of thoracic back pain and evidence of dorsal SEA probably from contiguous psoas abscess. Neurological examination revealed flaccid paraplegia and loss of sphincter control. A spinal MRI scan with Gd-enhancement revealed focal high intensity signal in the T2-weighted and FLAIR images at the level of the vertebral bodies in segments D3-D11. The patient was treated with posterior decompression and drainage of the SEA, but with a poor outcome. Six weeks after the onset of symptoms, an MRI scan showed a newly-formed hydromyelia formation from D4 to D8. The case reported is the second to describe hydromyelia formation secondary to epidural abscess and a poor outcome, experiencing partial improvement without recovery. For this reason, we confirmed that the essential problem of SEA lies in the need for early diagnosis, because the early signs and symptoms may be vague and the "classic" triad of back pain, fever and variable neurological deficits occur in only 13% of patients by the time of diagnosis. Only timely treatment will avoid or reduce permanent neurological deficits before massive neurological symptoms occur. The clear message is that a high index of suspicion and modern imaging techniques are essential.

Neuroradiological and neuropsychological study of a callosal infarct. A case report.

Corpus callosum infarcts are rare, constituing 0.6% of cerebral infarcts, most likely due to a rich blood supply from three main arterial systems. These infarcts may present with slowly evolving and non-localizing neurologic signs and symptoms that suggest the diagnosis of neoplasm rather than infarct. In addition, they may exhibit radiologic features more often associated with neoplasm, such as mass-like enhancement or extension across the midline. We describe a patient with corpus callosum infarct presenting with a non specific ataxia-like gait disorder, speech difficulty and left-side weakness. MR imaging disclosed an ischemic area in the body and splenium of the corpus callosum with extension to the right semiovalis centre. Neuropsychological study revealed constructive apraxia, attention reduction, motor programming and inhibiting control involvement. The atypical clinical presentation suggested involvement of the posterior circulation, while apraxia and attention impairment were in accordance with lesion localization. In-depth neuropsychological study is essential in patients with suspected corpus callosum involvement because the neurological signs and symptoms are confounding. The infarct localization in the body and semiovalis centre is an interesting neuroradiological feature.

Distal anterior cerebral artery aneurysms: endovascular or surgical treatment? A case report.

Cerebral aneurysms are occasionally associated with anomalies of the cerebral arteries. Most reports on anomalies of the anterior cerebral artery have been concerned with hypoplasia, fenestration and the infra-optic course of the A1, variant A1 perforators or Heubner's artery, multi-channeled anterior communicating artery, and azygos anterior cerebral artery. Distal anterior cerebral artery (ACA) aneurysms are known to have a poor clinical course and prognosis compared to other supratentorial aneurysms. The presence of the unpaired, distal, postcommunicating (A2) segment of the ACA is very rare in adults. We describe a patient with a ruptured aneurysm arising from the proximal end of the azygos ACA, first surgically treated with clipping and then with endovascular coiling. A 37-year-old woman at 34 weeks' gestation was transferred to our emergency room with sudden onset of severe headache and vomiting. Computed tomography (CT) revealed subarachnoid hemorrhage in the basal cisterna and the sylvian and interhemispheric fissures. Cerebral angiography showed an azygos ACA, a saccular aneurysm at the junction of the azygos ACA and the right A1 segment. A right fronto-temporal craniotomy was performed in the day of admission, and the neck of the aneurysm was clipped. One year later, an angiographic control examination revealed a regrowth of the aneurysm. The patient underwent endovascular treatment with coiling. Aneurysms of the azygos ACA are rare and their pathogenesis and course are still a matter of discussion. Developmental abnormalities or dynamic vessel wall stresses can explain the high incidence of aneurysms in these cases. The association of a rare anatomical variant with an aneurysm in the same location may suggest an embryogenesis alteration in the Willis circulation.

Does abnormal neuronal excitability exist in myotonic dystrophy? I. Effects of the antiarrhythmic drug hydroquinidine on slow saccadic eye movements.

The abnormal neuronal excitability hypothesized in myotonic dystrophy (MD) might contribute to psychomotor and behavioral disturbances of MD patients. To gain new insights into the pathophysiology of MD, we determined whether the antiarrhythmic drug hydroquinidine would ameliorate slow saccadic eye movements (SEMs), apathy and hypersomnia in MD patients. SEMs were selected as simple modality for psychomotor investigation. The study was conducted in a randomized, placebo-controlled, double-blind, crossover manner. Ten ambulatory patients without contraindications to hydroquinidine administration were enrolled. Hydroquinidine (450 mg/day) or placebo was given orally for 6 weeks with a washout period of 6 weeks between treatments. SEMs were recorded by electrooculography and analyzed by a computer system. Two patients withdrew in the first week of active treatment because of nausea and epigastralgia. Hydroquinidine significantly increased the normalized peak saccadic velocity and shortened the saccadic reaction time compared to placebo. The drug's effects on apathy and hypersomnia are presented in a companion paper.

Does abnormal neuronal excitability exist in myotonic dystrophy? II. Effects of the antiarrhythmic drug hydroquinidine on apathy and hypersomnia.

An abnormal neuronal excitability in myotonic dystrophy (MD) might contribute to psychomotor and behavioral disturbances of MD patients. To gain new insights into the pathophysiology of MD, we determined whether the antiarrhythmic drug hydroquinidine could ameliorate apathy and hypersomnia besides slow saccadic eye movements in these patients. The study was conducted in a randomized, placebo-controlled, double-blind, crossover manner. Ten ambulatory patients without contraindications to hydroquinidine administration were enrolled. Hydroquinidine (450 mg/day) or placebo was given orally for 6 weeks with a washout period of 6 weeks between treatments. Apathy was evaluated by means of the apathy evaluation scale (AES) and hypersomnia by a sleep diary. Two patients withdrew in the first week of active treatment because of nausea and epigastralgia. The drug significantly reduced AES scores and daily sleep time compared to placebo. Thus, hydroquinidine can ameliorate apathy and hypersomnia in MD. However, the possibility of proarrhythmia and the high frequency of cardiac disturbances in MD seriously limit the therapeutic perspective. The effects on eye movements are presented in a companion paper.

Relative sparing of extraocular muscles in myotonic dystrophy: an electrooculographic study.

We studied 40 patients with myotonic dystrophy (MD) to investigate whether saccadic eye movement (SEM) abnormalities have a central or peripheral origin. SEMs were recorded by electrooculography and analyzed by a computer system. Six patients were followed up to 2-7 years. Slow SEMs were present in 70% of patients, while saccadic latency and accuracy were within normal ranges. Peak saccadic velocity (PSV) did not correlate with disease duration and muscular disability, and showed a significant reduction only in 1 patient during the follow-up. Muscular disability correlated significantly with age and disease duration and worsened in 4 patients over time. The doll's head maneuver elicited vestibular compensatory eye movements with high velocities. These findings suggest that the extraocular muscles are at least partially spared in MD and that supranuclear structures, most likely the burst cells in brainstem reticular formation, may contribute to the slowing of SEMs.

Saccadic eye movements analysis in the early diagnosis of myasthenia gravis.

Saccadic eye movements were examined by electro-oculography in 21 patients with suspected myasthenia gravis. The presence of dissociated nystagmus and quiver eye movements was also assessed. The aim of the study was to assess the diagnostic value of saccadic abnormalities in the early stage of the disease. Pathological oculographic findings consisted of intra and post saccadic disorders and intersaccadic variability. A fatigue test was useful in detecting latent disorders in most patients. A Tensilon test was positive in 11 out of 16 patients with oculographic abnormalities. Diagnostic problems, apparently paradoxical findings and differential involvement of extraocular muscle fibers are discussed.