Malignant hyperthermia 2020: Guideline from the Association of Anaesthetists.

Malignant hyperthermia is defined in the International Classification of Diseases as a progressive life-threatening hyperthermic reaction occurring during general anaesthesia. Malignant hyperthermia has an underlying genetic basis, and genetically susceptible individuals are at risk of developing malignant hyperthermia if they are exposed to any of the potent inhalational anaesthetics or suxamethonium. It can also be described as a malignant hypermetabolic syndrome. There are no specific clinical features of malignant hyperthermia and the condition may prove fatal unless it is recognised in its early stages and treatment is promptly and aggressively implemented. The Association of Anaesthetists has previously produced crisis management guidelines intended to be displayed in all anaesthetic rooms as an aide memoire should a malignant hyperthermia reaction occur. The last iteration was produced in 2011 and since then there have been some developments requiring an update. In these guidelines we will provide background information that has been used in updating the crisis management recommendations but will also provide more detailed guidance on the clinical diagnosis of malignant hyperthermia. The scope of these guidelines is extended to include practical guidance for anaesthetists dealing with a case of suspected malignant hyperthermia once the acute reaction has been reversed. This includes information on care and monitoring during and after the event; appropriate equipment and resuscitative measures within the operating theatre and ICU; the importance of communication and teamwork; guidance on counselling of the patient and their family; and how to make a referral of the patient for confirmation of the diagnosis. We also review which patients presenting for surgery may be at increased risk of developing malignant hyperthermia under anaesthesia and what precautions should be taken during the peri-operative management of the patients.

Electrophysiological Study of Nerves in Type-II Reaction in Leprosy.

BACKGROUND: Leprosy is a chronic granulomatous infection primarily affecting the peripheral nervous system, skin and reticuloendothelial system. Cutaneous nerves are severely affected in lepra reaction and this leads to morbidity. OBJECTIVE: To study electrophysiological pattern of different nerves involved in Type-II reactions in leprosy. METHOD: The present study was undertaken in 21 leprosy patients with Type-II reactions attending in and out-patient department of Dermatology & Venereology, B.R.D. Medical College, Gorakhpur from July 2005 to October 2006. This was a prospective case control study in which 20 healthy, age and sex matched people with no evidence of any disease (particularly nerve involvement) were included. LIMITATION: Lesser number of cases were studied. RESULT: The proximal motor conduction latency was significantly prolonged in both ulnar and common peroneal nerve and proximal motor conduction velocity was also significantly reduced. On examining the values beyond 2S.D. of the control value, distal latency was not affected and only proximal conduction was affected in ulnar nerve. CONCLUSION: In Type II lepra reaction the motor conduction abnormalities are not prominent. Abnormalities are relatively more marked in the proximal segment.

Japanese encephalitis (JE) part II: 14 years' follow-up of survivors.

Japanese encephalitis, the commonest Arbovirus encephalitis, has been endemic in many parts of Asia, the Pacific Islands, and India; also, there have been many epidemics. Most of the post JE cases have been associated with neurological and neuropsychiatric deficits but have not been properly classified and followed. Practically all the previous studies were in children or young adults. The aim of this study, involving only adult cases, the largest ever being reported, has been to follow the 688/1,199 survivors of JE patients out of 1,282 of acute cases admitted during four epidemics for a period of 14 years after properly classifying the sequelae. This prospective study was conducted in B.R.D. Medical College Gorakhpur (India), involving 665/688 post JE cases with neuropsychiatric deficits from four epidemics of 1978, 1980, 1988 and 1989 which were properly classified in nine groups. While the first epidemic of 1978 was being studied, more disastrous episodes flared up and the patients were subsequently added. Hence, the total duration of this prospective study was from November 1978 to December 2003. There were 14 defaulted initially from 688 followed (23/688 without sequelae and 665/688 with neuropsychiatric deficits), and later 130 were lost from time to time at various stages of follow up. Four out of 23/688 discharged without any deficit had to be readmitted for bizarre movements, assaultative behaviour and euphoria without fever and altered sensorium. All of them improved by symptomatic treatment. Progressive improvement occurred in all the parameters consisting of psychological disturbances, higher cerebral dysfunction, speech disorders (dysphonia, dysarthria, dysphasias, apraxia and agnosia), extra pyramidal, pyramidal features, and hypothalamic disturbances, cranial nerves including pupils and fundi and seizures. Maximum cases improved between 6 months (55%) to 1 year (78%). Only some features improved between 5 to 14 years. Four patients of hemiplegia remained bed ridden. Some non disabling features like dysarthria and corticospinal features without paralysis persisted in 5% (95% improved) and 74% (26% improved) respectively. One patient with bizarre movement and nine with marked tremors could not regain normalcy. A large number of patients of JE are left with several minor or gross residual neuropsychiatric and neurological features after the acute phase. In this series also the discharged patients with neurological deficits who were quite disabled initially and needed constant care by family members and also those who required some help intermittently improved with passage of time and eventually returned to normal life. Some of them were left with non-disabling residual neurological signs even after 14 years. Fourteen of 544 (3%) could not return to their livelihood.

Japanese encephalitis (JE). Part I: clinical profile of 1,282 adult acute cases of four epidemics.

Japanese encephalitis (JE) is numerically the most important global cause of encephalitis and so far confirmed to have caused major epidemics in India. Most of the reported studies have been in children. This largest study involving only adults, belonging to four epidemics, is being reported from Gorakhpur. The aim of this study is to detail the acute clinical profile (not viral) outcome and to classify the sequelae at discharge. This prospective study involved 1,282 adult patients initially diagnosed as JE admitted during the epidemics of 1978, 1980, 1988, and 1989, on identical clinical presentation and CSF examination. In the meantime, the diagnosis of JE was confirmed by serological and/or virological studies in only a representative number of samples (649 of 1,282 cases). Eighty-three left against medical advice (LAMA) at various stages, so 1,199 of 1,282 were available for the study. Peak incidence of [1,061 of 1,282 (83%)] of clinically suspected cases was from September 15 to November 2. Serum IgM and IgG were positive in high titers in 50.87% (330 of 649) and IgM positive in CSF in 88.75% (109 of 123) of the cases. JE virus could be isolated from CSF and brain tissue in 5 of 5 and 4 of 5 samples, respectively. Altered sensorium (AS) in (96%), convulsions (86%), and headache (85%) were the main symptoms for hospitalization by the third day of the onset. Other neurological features included hyperkinetic movements in 593 of 1,282 (46%)-choreoathetoid in 490 (83%) and bizarre, ill-defined in 103 (17%). The features of brain stem involvement consisted of opsoclonus (20%), gaze palsies (16%), and pupillary changes (48%) with waxing and waning character. Cerebellar signs were distinctly absent. Dystonia and decerebrate rigidity was observed in 43 and 6%, respectively, paralytic features in 17% and seizures in 30%. Many non-neurological features of prognostic importance included abnormal breathing patterns (ABP) (45%), pulmonary edema (PO) (33%), and upper gastrointestinal hemorrhage (UGIH) (16%). Injection dexamethasone was used in 1978 in all 208 cases, including 21 of PO. Patients were later randomized alternately in dexa and non-dexa groups. Forty-six cases of PO from the non-dexa group were transferred to the dexa group as an ultimate life-saving measure. Thus, it was administered in 737 of 1,199 patients including 529 patients from the later epidemics in doses of 4 mg IV every 8 h for 7 days. Of 1,199, 462 did not receive it. There was no significant difference in mortality (p > 0.05) between the dexa (42.47%) and the non-dexa group (42.86%). All PO cases expired; so after the exclusion of the PO cases from dexa group, the difference of 6.14% (42.86 and 36.72) became significant (p < 0.01) (511 of 1,199 (43%) expired, [320 of 511 (63%) died within 3 days of hospitalization]). Out of a total of 1,199 patients treated, 688 (57%) were discharged; 23 of 688 (3%) without any sequelae and 665 of 688 (97%) with neuropsychiatric deficits classified into nine groups. During the four epidemics, the diagnosis of JE was basically on identical clinical presentation of acute encephalitic syndrome (AES) consisting of (1) abrupt onset of fever, headache, and AS, (2) dystonias and various movement disorders, (3) opsoclonus and gaze palsies, (4) CSF findings, and (5) the presence of residual neuropsychiatric and neurological features in the survivors.

Scorpion bite and multiple cerebral infarcts.

Multiple cerebral infarcts, bilateral optic neuropathy with limb ischemia, following scorpion bite is documented. Vasospasm and autonomic storm due to envenomation is a plausible explanation for this symptom complex.

The BMP family member Gdf7 is required for seminal vesicle growth, branching morphogenesis, and cytodifferentiation.

Epithelial-mesenchymal interactions play an important role in the development of many different organs and tissues. The secretory glands of the male reproductive system, including the prostate and seminal vesicles, are derived from epithelial precursors. Signals from the underlying mesenchyme are required for normal growth, branching, and differentiation of the seminal vesicle epithelium. Here, we show that a member of the BMP family, Gdf7, is required for normal seminal vesicle development. Expression and tissue recombination experiments suggest that Gdf7 is a mesenchymal signal that acts in a paracrine fashion to control the differentiation of the seminal vesicle epithelium.

Sign language tics in a prelingually deaf man.

Gilles de la Tourette syndrome is characterized by vocal and motor tics starting in childhood. Vocal tics may be either noises or words, and the vocal language tics may consist of obscenities (coprolalia) and repetitions of speech that has been heard (echolalia). We describe a prelingually deaf man who has the full array of tics seen in Gilles de la Tourette syndrome, but in whom vocal language tics are replaced by equivalent sign language tics. This is, to our knowledge, the first report of sign language tics in a person with prelingual deafness. The implications of this phenomenon for the separation between language and ideas in tics and the equivalence of sign language to spoken language is discussed.

Heterosexual couples confronting the challenges of HIV infection.

Couples confronted with HIV infection face significant challenges. Little is known about the impact of HIV on heterosexual couples who account for the vast majority of cases worldwide and an increasing proportion of cases in the USA, especially among women. In this study, analysis of data collected on HIV-discordant couples participating in a ten-week support group revealed four major groups of issues: (1) dealing with the emotional and sexual impact on the relationship; (2) confronting reproductive decisions; (3) planning for the future of children and the surviving partner; and (4) disclosure of the HIV infection to friends and family. These findings have implications for the design of interventions to enhance adaptation to HIV for discordant couples.

Reproductive behavior in HIV-discordant heterosexual couples: implications for counseling.

The development of effective behavioral interventions is critical to controlling the HIV epidemic. Although heterosexual transmission accounts for a growing proportion of new infections in the United States, little is known about factors that influence sexual behavior in HIV-discordant heterosexual couples. The objective of this study was to examine the reproductive behaviors of HIV-discordant heterosexual couples. Data were obtained on 71 discordant couples enrolled in a study of HIV heterosexual transmission. Results showed that women in such couples have pregnancy rates similar to those of women of reproductive age in the general population. One seroconversion occurred as a result of pregnancy attempt. These results suggest the need for educational efforts directed at HIV-discordant heterosexual couples.

Peripheral nerve abscess in leprosy: report of twenty cases.

During the year 1994-1995, 20 of the 67 leprosy patients attending the dermatology department with any kind of nerve involvement were found to be having nerve abscess. These abscesses occurred in all types of leprosy (except the Indeterminate) and a variety of nerve trunks and cutaneous nerves. In none of the instances the abscess was associated with reaction. All the patients were surgically treated, without any steroid therapy. All cases showed significant improvement whenever there was nerve function deficit. Similarly, pain was relieved in all cases, when it was present.

Non-traumatic coma--profile and prognosis.

Two hundred consecutive patients of non-traumatic coma, were investigated to establish its aetiology. Neurologic profile of these patients included assessment of Glasgow Coma Scale (GCS) score and evaluation of brainstem reflexes. 102 patients died and only 54 patients could make good recovery. Cerebrovascular diseases (33%), CNS infections (21%), and hepatic encephalopathy (18%) were the frequent causes of non-traumatic coma, with the first two carrying relatively poor prognosis. Poor outcome was also associated with low GCS score and absence of brainstem reflexes specially absent pupillary, oculocephalic and oculovestibular responses and decerebrate posture.