1/29. Hypertonia, hyperreflexia, and excessive startle response in a neonate.Following an uneventful gestation, a newborn girl presented with hypertonia, hyperreflexia, tremor, and excessive startle response. nose tap elicited a dramatic head recoil. Her mother had similar symptoms beginning as a child that improved but persisted into adulthood. In addition, several members of mother's family died unexpectedly in infancy. Hypertonia in the newborn period indicates central nervous system dysfunction of several possible causes, most of which are associated with severe cognitive deficits and limited neurological development.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
2/29. Hyperreflexia in a patient with motor axonal guillain-barre syndrome.We report a patient who presented after an episode of diarrhoea with ascending, symmetrical weakness without sensory loss, and without sphincter or other autonomic dysfunction. On clinical examination there were no cranial nerve deficits. Hyperreflexia of tendon jerks without other upper motor neurone signs was found. Electrophysiological examination demonstrated acute distal symmetrical motor axonal polyneuropathy. No electrophysiological signs of peripheral nerve demyelination or central nervous system involvement were found. Albuminocytologic dissociation was present in the cerebrospinal fluid. Stool culture and serological tests were inconclusive. Our patient's clinical picture was, apart from hyperreflexia of tendon jerks throughout the disease, characteristic of guillain-barre syndrome. This is the first such patient reported in europe. The aetiology remained unclear. We suggest that selective axonal motor fibre affection, with possible mild pyramidal involvement, caused tendon jerk hyperreflexia.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
3/29. central nervous system involvement following type I aviator's bends complicated by complacency.A false sense of security surrounds the possibility of post-flight complications resulting from "aviator's bends." The accepted clinical clue that a patient is at risk for serious complications is the presence of some form of dysbarism at altitude. This principle has been inappropriately extended to imply that serious post-flight complications of the evolved gas syndrome only follow serious in-flight symptoms. This paper, in addition to reporting the occurrence of post-flight neurologic signs in a patient after Type I pain-only bends during an altitude chamber flight, also identifies a broader subtle complacency in the professional community that routinely deals with hypobarics.- - - - - - - - - - ranking = 4keywords = nervous system (Clic here for more details about this article) |
4/29. Sensory neuropathy in autosomal recessive juvenile parkinsonism (PARK2).Autosomal recessive juvenile parkinsonism (ARJP/PARK2) is a distinct clinical and genetic entity characterized by early-onset levodopa-responsive parkinsonism, foot dystonia, sleep benefit, and hyperactive tendon reflexes. We report a patient with genetically confirmed ARJP, who showed mild sensory disturbance and diminished deep tendon reflexes in the advanced stage. Nerve conduction studies revealed a sensory dominant neuropathy, which has not been described in ARJP. We suggest that peripheral neuropathy may occur in patients with advanced ARJP due to the loss of parkin protein function, although the function of parkin in the peripheral nervous system remains to be clarified.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
5/29. A case of miller fisher syndrome: atypical findings and therapeutic considerations.The miller fisher syndrome (MFS) is generally considered to be a disease of the peripheral nervous system. In some cases contemporary involvement of the central nervous system has been described (CNS). We report a case in which it was possible to prove involvement of cranial nerves VII, VIII, IX and X and to exclude CNS involvement. We discuss the possible role of early plasmapheresis treatment on disease evolution.- - - - - - - - - - ranking = 2keywords = nervous system (Clic here for more details about this article) |
6/29. Non-MS recurrent demyelinating diseases.The introduction of MRI has shown that the acute, recurrent (R), and multiphasic (M) forms of disseminated encephalomyelitis (DEM) are more common than suspected in adults, and that their MR images are sufficiently characteristic in most instances to make differentiation from multiple sclerosis (MS) possible. In addition, a number of clinical features of DEM are rarely seen in MS: fever, malaise, nausea, vomiting, positional vertigo, convulsions, aphasia, meningism, bilateral optic neuritis, and CSF leukocytosis and elevated protein. CSF oligoclonal bands are usually absent. It is remarkable that confusion between R- and MDEM and MS persists despite the numerous published reports on recurrent DEM dating back 70 years, many illustrating the characteristic MRIs. There are many case reports of DEM erroneously diagnosed as MS, Schilder's, Marburg's, Devic's, and Balo's disease, and, in particular brain tumors. It is probable that acute DEM is occasionally mistaken for a clinically isolated symptom of MS. Possible mechanisms for recurrence include localization at the site of a previous injury to the nervous system, or by the phenomenon of molecular mimicry. The importance of differentiating R- and MDEM from MS is greater today due to the recommendation that immunodulatory treatment be initiated in patients with a clinically isolated syndrome, or when the occurrence of a second clinical episode establishes the diagnosis of MS.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
7/29. Ataxic polyneuropathy and anti-Pr2 IgM kappa M proteinemia.A case of ataxic neuropathy associated with IgM kappa M proteinemia is reported. Double filtration plasmapheresis effectively treated the neuropathy. The IgM kappa antibody had anti-Pr2 cold agglutinin activity. We demonstrated reactivities of the IgM kappa antibody to sialosyl paragloboside, sialosyl lactosaminyl paragloboside, GT1b, GD1a, GD1b, GM3 and GD3 on high-performance thin-layer chromatography immunostaining and enzyme-linked immunosorbent assay, which is previously unreported antigenic specificity of the M proteins in cases of paraproteinemic neuropathy. IgM M protein with anti-Pr2 cold agglutinin activity may play a pathogenetic role in peripheral nerve demyelination, because the target antigens of the M protein are present in myelin and possibly in endothelial cells of the peripheral nervous system.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
8/29. Obstetrical anaesthesia and analgesia in chronic spinal cord-injured women.Improved acute and rehabilitative care and emphasis on integrating patients into society after spinal cord injury is likely to result in increasing numbers of cord-injured women presenting for obstetrical care. Anaesthetists providing care to these women should be familiar with the complications resulting from chronic cord injury and aware that many may be aggravated by the physiological changes of normal pregnancy. These complications include reduced respiratory volumes and reserve, decreased blood pressure and an increased incidence of thromboembolic phenomena, anaemia and recurrent urinary tract infections. patients with cord lesions above the T5 spinal level are at risk for the life-threatening complication of autonomic hyperreflexia (AH) which results from the loss of central regulation of the sympathetic nervous system below the level of the lesion. Sympathetic hyperactivity and hypertension result in response to noxious stimuli entering the cord below the level of the lesion. Labour appears to be a particularly noxious stimulus and patients with injuries above T5 are at risk for AH during labour even if they have not had previous AH episodes. morbidity is related to the degree of hypertension and intracranial haemorrhage has been reported during labour and attributed to AH. We report our experience in providing care to three parturients with spinal cord injuries. Two patients had high cervical lesions, one of whom experienced AH during labour and was treated with an epidural block. The second was at risk for AH having had episodes in the past and received an epidural block to provide prophylaxis for AH. In both cases epidural blockade provided effective treatment and prophylaxis for AH.(ABSTRACT TRUNCATED AT 250 WORDS)- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
9/29. Hyperekplexia and sudden neonatal death.Fifteen patients with hyperekplexia were identified in 3 families; diagnostic clinical characteristics were defined which allowed for early recognition and treatment. During the first 24 hours of life, spontaneous apnea and sluggish feeding effort were observed. After the first 24 hours, surviving infants exhibited the hyperekplexic startle response to nose tapping. This startle response is characterized by sudden muscular rigidity, feeding-induced oropharyngeal incoordination, and poor air exchange often with apnea, persisting with repetitive nose tapping. Untreated infants experienced recurring apnea until 1 year of age. Three of 15 patients died unexpectedly during the neonatal period. patients treated with clonazepam (0.1-0.2 mg/kg/day) had no serious apneic episodes and startle reflexes were diminished. The pathophysiologic mechanism for hyperekplexia remains obscure. Electroencephalographic studies were consistently normal. The response to and tolerance of benzodiazepines are striking in newborns and infants and suggest an aberrant central nervous system reflex as the etiology; therefore, hyperekplexia should be considered in the evaluation of neonates and infants with apnea, aspiration pneumonia, episodic muscular rigidity, hyperexcitability, and near-miss sudden infant death syndrome. The need for immediate monitoring of at-risk infants, observation for signs of hyperekplexia, and initiation of clonazepam in these patients are emphasized. Hyperekplexic startle response to nose tapping should be included in the routine examination of all newborns.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
10/29. Ross syndrome, an entity included within the spectrum of partial disautonomic syndromes.Ross syndrome is a degenerative peripheral nervous system disorder defined by the following triad: unilateral or bilateral segmental anhidrosis, hyporeflexia of deep tendon reflexes and Adie's tonic pupils. The most disturbing symptom is segmental compensatory hyperhidrosis. It has only occasionally been reported in the dermatological literature. We present a 35-year-old woman with chronic hepatitis c who developed the characteristic triad of Ross syndrome within 1 month. The patient was otherwise healthy except for an aneurysm of the left medium brain artery not responsible for the syndrome.- - - - - - - - - - ranking = 1keywords = nervous system (Clic here for more details about this article) |
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