ABSTRACT
Celiac disease can be associated with a wide spectrum of neurological and psychiatric symptoms (cerebellar ataxia, neuromuscular manifestations, epilepsy, dementia), even in the absence of malabsorption or gastrointestinal symptoms. The case of a 72-year-old man with a rapidly progressive, lethal encephalopathy secondary to celiac disease is reported, together with a review of documented neurological symptoms in celiac disease. The aetiology of these neurological symptoms is unknown, although immunological mechanisms are suspected.
Subject(s)
Antibodies/analysis , Brain Diseases/etiology , Celiac Disease/complications , Gliadin/immunology , Mental Disorders/etiology , Neuromuscular Diseases/etiology , Aged , Brain Diseases/diagnosis , Brain Diseases/immunology , Celiac Disease/diagnosis , Celiac Disease/immunology , Cerebellar Ataxia/diagnosis , Cerebellar Ataxia/etiology , Cerebellar Ataxia/immunology , Diagnosis, Differential , Gait , Humans , Male , Mental Disorders/diagnosis , Mental Disorders/immunology , Neuromuscular Diseases/diagnosis , Neuromuscular Diseases/immunologyABSTRACT
We described clinical manifestations, outcomes, prognostic indicators and clinico-epidemiological subgroups for 53 adult patients with Guillain-Barré syndrome (GBS) in Sweden during the period 1996-97. These patients were identified from a population of 2.8 million inhabitants and prospectively followed up for one year by a network of neurologists. An additional 10 cases, of whom five were adults who had not been prospectively followed up, were not included in the analyses. At 6 months after onset 80% of the patients could walk without aid, while at 1 year 46% were fully recovered, 42% had mild residual signs or symptoms, 4% had moderate and 6% severe disabilities, and 2% had died. Intravenous human immunoglobulin or plasmapheresis were used in 72% of the patients. The sum of the Medical Research Council (MRC) score at nadir was found as the only significant predictor for residual signs at 1 year in a multivariate model. Three subgroups, with different clinico-epidemiological characteristics, were identified by using cluster analysis. In conclusion, GBS in Sweden is frequently preceded by a respiratory infection, is often treated with immunomodulatory therapies, and exhibits a high recovery rate and a low fatality rate.
Subject(s)
Guillain-Barre Syndrome/epidemiology , Guillain-Barre Syndrome/physiopathology , Adult , Cluster Analysis , Female , Follow-Up Studies , Guillain-Barre Syndrome/rehabilitation , Humans , Incidence , Male , Middle Aged , Prognosis , Sweden/epidemiology , Time Factors , WalkingABSTRACT
Peduncular hallucinosis is usually caused by infarction of the mesencephalic portion of the brainstem, and is characterised by acute onset of vivid, colorful hallucinations often consisting of moving patterns, people or animals, which the patient is aware are unreal. Charles Bonnet's syndrome is a similar condition, caused by impaired version. Both syndromes may be explained by visual release phenomena caused by lesions in different parts of the visual system. The article consists in two case reports of peduncular hallocinosis and a review of partinent literature.
Subject(s)
Brain Ischemia/psychology , Cerebral Infarction/psychology , Hallucinations , Mesencephalon/pathology , Aged , Brain Ischemia/complications , Brain Ischemia/drug therapy , Cerebral Infarction/complications , Cerebral Infarction/drug therapy , Diagnosis, Differential , Female , Hallucinations/diagnosis , Hallucinations/drug therapy , Hallucinations/etiology , Humans , Mesencephalon/blood supplyABSTRACT
Three cases of hereditary neuropathy with liability to pressure palsies (HNPP), characterised by the typical deletion in chromosome 17p11 2-12, are described in the article. The clinical manifestations were pain-free brachial plexus palsies in two patients (belonging to the same family), and multiple episodes of numbness and weakness in various nerves in the third patient. The clinical, electrophysiological, histological and genetic findings are reviewed.
Subject(s)
Paresis/genetics , Peripheral Nervous System Diseases/genetics , Adult , Chromosome Deletion , Chromosomes, Human, Pair 17 , Diagnosis, Differential , Female , Humans , Male , Middle Aged , Paresis/diagnosis , Paresis/pathology , Peripheral Nervous System Diseases/diagnosis , Peripheral Nervous System Diseases/pathology , Pressure , RecurrenceSubject(s)
Demyelinating Diseases/diagnosis , Motor Neuron Disease/diagnosis , Adult , Demyelinating Diseases/physiopathology , Demyelinating Diseases/therapy , Diagnosis, Differential , Humans , Immunoglobulins, Intravenous/therapeutic use , Male , Motor Neuron Disease/physiopathology , Neural ConductionABSTRACT
Multiple sclerosis (MS) is traditionally a clinical diagnosis based on relapsing and remitting multifocal CNS symptoms. In recent years new diagnostic tools such as cerebrospinal fluid analysis, magnetic resonance imaging and evoked potential testing have been developed. This article reviews diagnostic difficulties and discusses other medical conditions that can be mistaken for MS due to similar clinical, laboratory or radiological findings.
Subject(s)
Central Nervous System Diseases/diagnosis , Multiple Sclerosis/diagnosis , Collagen Diseases/diagnosis , Diagnosis, Differential , Diagnostic Errors , Evoked Potentials, Somatosensory , Humans , Magnetic Resonance Imaging , Multiple Sclerosis/cerebrospinal fluidSubject(s)
Leg , Paralysis/etiology , Puerperal Disorders/etiology , Adult , Female , Humans , Lumbosacral Plexus/physiopathology , Obstetric Labor Complications/diagnosis , Obstetric Labor Complications/physiopathology , Paralysis/diagnosis , Paralysis/physiopathology , Paresthesia/diagnosis , Paresthesia/etiology , Paresthesia/physiopathology , Pregnancy , Prognosis , Puerperal Disorders/diagnosis , Puerperal Disorders/physiopathologyABSTRACT
Acute postasthmatic amyotrophy is a rare condition, previously reported in only 26 cases. It is characterized by a sudden onset of a flaccid paralysis of an arm or a leg with completely preserved sensibility, about 1 week after an asthmatic attack. The cause is probably due to a lesion of the anterior horn of the spinal cord, but evidence indicates a more widespread pathological process. The etiology is unknown, but infectious or immunological mechanisms are likely.
Subject(s)
Asthma/complications , Muscle Hypotonia , Paralysis/etiology , Paralysis/physiopathology , Action Potentials , Child , Electromyography , Evoked Potentials, Somatosensory , Female , Humans , Magnetic Resonance Imaging , Male , Nervous System/physiopathology , Paralysis/diagnosis , Poliomyelitis/physiopathology , Sensation/physiology , Spinal Cord/pathology , SyndromeABSTRACT
A 52 year old woman, previously healthy apart from a mild chronic persisting hepatitis, developed a sensory ataxia one week after an acute febrile illness. Clinically she had a severe impairment of proprioception and kinesthesia in the arms. Neurophysiological examinations demonstrated absent sensory potentials in the hands. No malignancy was found, and the patient had no sicca symptoms. Some tests for Sjögren's syndrome were positive, but not conclusive. A slight improvement followed after the acute phase. The diagnosis is compatible with an idiopathic subacute sensory neuronopathy.
Subject(s)
Central Nervous System Diseases/diagnosis , Sensation Disorders/diagnosis , Acute Disease , Ataxia/diagnosis , Diagnosis, Differential , Female , Humans , Middle Aged , Polyradiculoneuropathy/diagnosis , Sjogren's Syndrome/diagnosisABSTRACT
Assuming the presence of glutamate-induced neurotoxicity in amyotrophic lateral sclerosis 14 patients were treated with dextromethorphan, an N-methyl-D-aspartate receptor antagonist. The patients were treated with 150 mg dextromethorphan or placebo daily for 12 weeks in a double-blind crossover trial, with a wash out period of 4 weeks between the two treatment periods. Thereafter the surviving patients were treated with 300 mg dextromethorphan daily for up to 6 months in an open trial. No positive effects on clinical or neurophysiological parameters (relative number of axons, and compound muscle action potentials in the abductor digiti minimi muscle) were observed either in the double-blind trial or in the open trial.
Subject(s)
Amyotrophic Lateral Sclerosis/drug therapy , Dextromethorphan/therapeutic use , Aged , Double-Blind Method , Electromyography/drug effects , Female , Humans , Male , Middle Aged , Neurologic Examination/drug effects , Pilot Projects , Receptors, N-Methyl-D-Aspartate/antagonists & inhibitorsSubject(s)
Cerebrovascular Disorders/etiology , Eye Diseases/etiology , Pseudoxanthoma Elasticum/complications , Vision Disorders/etiology , Adult , Cerebrovascular Disorders/diagnosis , Diagnosis, Differential , Eye Diseases/diagnosis , Eye Diseases/pathology , Fluorescein Angiography , Humans , Male , Pseudoxanthoma Elasticum/diagnosis , Pseudoxanthoma Elasticum/pathology , Skin/pathology , Vision Disorders/diagnosisABSTRACT
The clinical distinction between cardiovascular and epileptic causes of loss of consciousness is sometimes difficult, but becomes more complicated when a primary epileptic seizure secondarily causes a cardiac arrest. This has been reported previously in only eight patients. We report an additional patient with cardiac arrest during a mesiobasal left temporal lobe seizure discharge.
