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 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.

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.

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.

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.

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.

Late reversal reaction after 10 years of adequately treated leprosy.

Differentiation between a relapse or late reversal reaction following completion of regular drug therapy in patients with leprosy is often difficult, though it has definite therapeutic implications. The present case documents a late reversal reaction occurring an unusually long time after the completion of multi-drug therapy.

Electro-physiological evaluation of nerves during reactions in leprosy.

Forty-two patients with leprosy (7 with tuberculoid type, 30 borderline, 5 lepromatous) were studied electrophysiologically during reactions. Thirty-three had type I reactions while 9 had type II reactions. Each patient received 60 mg/day prednisolone tapered over a 6-week period. Motor conduction studies were performed on one clinically affected and one unaffected nerve and were repeated 12 weeks after the beginning of steroid therapy. Significant motor conduction abnormalities were observed in 14 affected (33.3%) and 8 unaffected nerves (19.1%). The majority of these nerves were in patients with borderline leprosy having type I reactions. Following steroid therapy, nerve function improved in 14 affected (33.3%) and 20 unaffected nerves (47.66%). However, 5 affected (10.2%) and 12 unaffected nerves (28.5%) showed a worsening of nerve function following steroid therapy. The majority of the nerves that showed improvement with steroid therapy had type I reactions, while those that showed deterioration had type II reactions. Steroids improved nerve function mainly in patients with type I reactions. Their role in patients with type II reactions remains debatable.

Bilateral anterior tibial compartment syndrome in association with hypothyroidism.

Local vascular and traumatic involvement are well-recognized causes of anterior tibial compartment syndrome (ATCS) terminating as myoneural ischaemia. However, in a large number of patients the cause of ATCS remains unidentified. We document the occurrence of bilateral ATCS in a patient of hypothyroidism, to our knowledge a previously unrecorded association.

Disappearing CT lesion in a nonepileptic patient.

Despite its high incidence, the exact cause of disappearing CT lesions in patients with epilepsy is not clear. We document a non-epileptic patient, whose clinical picture simulated idiopathic intracranial hypertension, but CT showed a spontaneously resolving ring enhancing lesion.

Epidemiology of idiopathic intracranial hypertension: a prospective and case-control study.

An epidemiologic survey of idiopathic intracranial hypertension (IIH) in Benghazi, Libya, over a period from September 1982 through August 1989 ascertained 81 patients. The group was comprised of 76 females and 5 males. Ages ranged from 8 to 55 years; the mean +/- S.D. was 28.6 +/- 7.9 for women and 21.0 +/- 14.5 for men. The average crude annual incidence rates for IIH per 100,000 persons were 2.2 for the total and 4.3 for females for all ages (3.2 for the total and 5.9 for the females when adjusted to the 1980 United States population). In females aged 15-44 years, IIH occurred at a rate of 12.0 per 100,000 per year; for those defined as obese, the rate rose to 21.4. Moderate to severe visual loss occurred as a sequelae in 20% of our patients. The extent of visual loss did not correlate with age at diagnosis, duration of symptoms, degree of obesity, use of oral contraceptive pills, cerebrospinal fluid (CSF) opening pressure, steroid treatment, or recurrence. We found no correlation between CSF protein and opening pressure. We conducted a case-control study on 40 consecutive female incident IIH patients and 80 age-matched female control subjects. Obesity and recent weight gain occurred more frequently in patients. More patients were married and more had irregular menses. The incidence rate for IIH described in our study is three to four times higher than that reported from the United States.