Sonographic pattern of median nerve enlargement in Hansen's neuropathy.

OBJECTIVES: Median nerve enlargement in leprosy seems to be more proximal than in carpal tunnel syndrome (CTS), but this feature has not been studied systematically. The aim of the study was to compare the sites of median nerve enlargement in patients with leprosy with that of patients with CTS. MATERIALS AND METHODS: Transverse sections of the median nerve were recorded from wrist to the mid-forearm (at distal wrist crease and at 2-cm: M1, 4-cm: M2, 6-cm: M3, 8-cm: M4 and 10-cm: M5, proximal to the distal wrist crease in the forearm) in patients with leprosy, CTS and healthy subjects using high-resolution ultrasound. RESULTS: Twenty-six patients each with leprosy and CTS were compared with healthy controls. Patients with leprosy included 6 (23.1%), 7 (26.9%), 7 (26.9%) and 6 (23.1%) patients with borderline tuberculoid, borderline-borderline, borderline lepromatous and lepromatous leprosy, respectively. Cross-sectional area (CSA) of median nerve was increased in all patients with leprosy as compared to healthy controls at all points of measurement. CSA was higher among patients with leprosy as compared to CTS at all points except at the wrist. In patients with leprosy, the maximal enlargement was noted 2-cm (M1) proximal to the wrist crease with gradual tapering of the CSA proximally (p < .05). In contrast, in patients with CTS the median nerve was maximally enlarged at the distal wrist crease (p<.05). CONCLUSIONS: Median nerve enlargement 2-cm proximal to the distal wrist crease distinguishes leprosy from CTS. This important discriminating sign can be used at point-of-care to identify patients with leprosy.

A rare cause of median neuropathy at the carpal tunnel: Thrombosis of the persistent median artery.

Carpal tunnel syndrome is a common peripheral nerve entrapment neuropathy caused due to compression of the median nerve at the level of the wrist joint. Bifid median nerve associated with a persistent median artery is a rare entity and in itself asymptomatic anatomical variant. However, distension of the persistent median artery due to a thrombus can be symptomatic due to compression on the median nerve and can compromise the blood flow to the palm. We report a case of persistent median artery thrombosis in a young female patient who presented with symptoms of carpal tunnel syndrome diagnosed on the ultrasonography and confirmed on the MRI with subsequent improvement post anticoagulation therapy.

Recurrent tumefactive demyelination: An unusual presentation.

Tumefactive multiple sclerosis (MS) is a rare variant of MS characterized by the presence of large demyelinating plaques of more than 2 cm, identified with magnetic resonance imaging (MRI). Distinguishing tumefactive lesions from other etiologies of intracranial space occupying lesions is necessary to avoid the inadvertent intervention. We had a 14-year-old girl who presented to us with two episodes of subacute hemiparesis over a span of 6 months. Her MRI brain showed large lesions, which were hyperintense on T2-weighted/flair images with incomplete ring enhancement open towards the gray matter in postgadolinium images with minimal surrounding edema and mass effect. We treated her as a case of tumefactive demyelination (TD) with steroids after which patient recovered with minimal deficits. TD occurs more commonly in women and young adults and is reported rarely. TD in a young girl with recurrence in such short span causing bilateral hemiparesis has never been reported.

Carpal tunnel syndrome: Analyzing efficacy and utility of clinical tests and various diagnostic modalities.

BACKGROUND: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy, but not adequately studied in India. OBJECTIVES: To study clinical tests, nerve conduction studies (NCS), ultrasonography (USG), and magnetic resonance imaging (MRI) in diagnosing CTS. MATERIALS AND METHODS: We diagnosed CTS in 54 patients (93 hands) out of 60 screened patients with symptoms compatible with CTS, including 19 control patients (23 hands). We conducted provocative tests and calculated Boston Carpal tunnel Questionnaire (BCTQ) symptom (S) and function (F) scores. NCS positive patients were classified into mild, mild-to-moderate, moderate, severe, and all-CTS groups. Median nerve anteroposterior, transverse, circumference (CIR), and cross-sectional area (CSA) at inlet (I), middle (M), and outlet (O) each was measured by USG in all patients. MRI was done in 26 patients (39 hands). RESULTS: Phalen, hand elevation and pressure provocation tests had higher sensitivity, Tinel's test had higher specificity and tethered median nerve and tourniquet tests had low sensitivity and moderate specificity. USG had low sensitivity but high specificity, and MRI had moderate sensitivity. USG in patients compared to controls was significantly abnormal in CSA-I, CIR-I, and CSA-O. Significant correlation was found between BCTQ-S and NCS and BCTQ-S and CIR-O. CIR-M, CIR-O, CSA-M, and CSA-I had correlation with NCS. MRI was significant in moderate and in moderate + severe groups combined and associated pathologies were detected in 59% patients. CONCLUSION: NCS remain gold standard but USG and MRI help increase sensitivity and detect mass lesions amenable to surgery.

A study of Guillain-Barré syndrome with reference to cranial neuropathy and its prognostic implication.

BACKGROUND: Focused studies on cranial neuropathy in Guillain-Barré syndrome (GBS) and its prognostic implication are not done previously. AIM: To study the clinical profile of GBS patients with special reference to cranial neuropathy and its prognostic implication. MATERIALS AND METHODS: The study included 61 patients with GB syndrome, fulfilling Asbury Cornblath's criteria for GB syndrome. A pre-designed semi-structured questionnaire was used to obtain data regarding demographic profile and clinical profile. All patients underwent detailed neurological examination, investigations including nerve conduction studies and CSF examination and treated according to the severity of the illness. Patients were followed up for 6 months. During analysis two groups were made depending on cranial nerve involvement, and compared with respect to various parameters. RESULTS: Out of 61 patients 38 (62.3%) patients had cranial nerve palsies, in that 25 had multiple cranial nerve palsies, and 13 had single isolated nerve palsy. A majority of 30 (49.2%) had bulbar palsy, 28 (46%) had facial nerve palsy, and all had bilateral involvement except 3 patients who had unilateral palsy. Hypoglossal nerve involvement was seen in six (10%) patients and four (6.5%) patients had ophthalmoplegia. Only one had bilateral vestibulocochlear nerve palsy. On comparing various clinico-electrophysiological parameters among patients of GB syndrome with and without cranial nerve involvement, the presence of respiratory paralysis, IVIg and ventilatory support requirement had significant association with cranial nerve involvement in GBS. CONCLUSION: Our study found a correlation between cranial nerve palsies and severity of the illness. Cranial nerve innervated muscles recover earlier as compared to distal limb muscles. No association was found between outcome at 6 months and cranial nerve involvement.

Guillain-Barre syndrome with posterior reversible encephalopathy syndrome.

Posterior reversible encephalopathy syndrome (PRES) is a clinicoradiologic entity commonly associated with eclampsia, septicemia, chemotherapeutic drugs etc. Concurrent occurrence of Guillain-Barre syndrome (GBS) with PRES is a rare entity. Dysautonomia is a proposed mechanism for such occurrence. Here we present a non-diabetic, non-hypertensive 63-year-old male patient, who came with acute onset flaccid quadriparesis, developing generalized seizures, altered sensorium and raised blood pressure on fifth day of illness. Magnetic resonance imaging (MRI) of brain showed altered signal intensities involving the parieto-occipital areas suggestive of posterior reversible encephalopathy. Cerebrospinal fluid analysis showed albuminocytological dissociation, nerve conduction studies revealed demyelinating type of polyneuropathy. The patient was treated with antihypertensives and antiepileptics. After resolution of the encephalopathy, intravenous immunoglobulin (IVIg) was given. The patient recovered gradually over few months. Our case concludes GBS as independent risk factor, for PRES may be secondary to dysautonomia and physicians should be aware of such rare coexistence so that early treatment can be done to reduce the mortality and morbidity.

Study of cluster headache: A hospital-based study.

INTRODUCTION: Cluster headache (CH) is uncommon and most painful of all primary headaches, and continues to be managed suboptimally because of wrong diagnosis. It needs to be diagnosed correctly and specifically treated. There are few studies and none from this region on CH. MATERIALS AND METHODS: To study the detailed clinical profile of CH patients and to compare them among both the genders. Study was conducted at Mahatma Gandhi hospital, Jodhpur (from January 2011to December 2013). Study comprises 30 CH patients diagnosed according to International Headache Society guidelines (ICHD-II). Routine investigations and MRI brain was done in all patients. All measurements were reported as mean ± SD. Categorical variables were compared using the Chi-square test, and continuous variables were compared using Student's t-test. SPSS for Windows, Version 16.0, was used for statistical analyses with the significance level set at P = 0.05. RESULTS: M: F ratio was 9:1. Age at presentation was from 22-60 years (mean - 38 years). Latency before diagnosis was 3 months-12 years (mean - 3.5 years). All suffered from episodic CH and aura was found in none. Pain was strictly unilateral (right-19, left-11), predominantly over temporal region-18 (60%). Pain intensity was severe in 27 (90%) and moderate in 3 (10%). Pain quality was throbbing in 12 (40%). Peak intensity was reached in 5 minutes-30 minutes and attack duration varied from 30 minutes to 3 hours (mean - 2.45 hours). Among autonomic features, conjunctival injection-23 (76.6%) and lacrimation-25 (83.3%) were most common. Restlessness during episode was found in 80%. CH duration varied from 10 days to 12 weeks. Circadian periodicity for attacks was noted in 24 (80%). CONCLUSION: Results are consistent with other studies on many accounts, but is different from Western studies with respect to low frequency of family history, chronic CH, restlessness and aura preceeding the attack. Detailed elicitation of history is paramount as misdiagnosis is common.

Response to abdominal hysterectomy with bilateral salpingo-oophorectomy in postmenopausal woman with anti-yo antibody mediated paraneoplastic cerebellar degeneration.

Paraneoplastic cerebellar degeneration (PCD) is a rare neurological disorder characterized by a widespread loss of Purkinje cells associated with a progressive pancerebellar dysfunction. PCD often precedes the cancer diagnosis by months to years. Here, we report a case of 44-year old postmenopausal woman who presented with PCD symptoms and high levels of anti-Yo antibodies titer since 8 months. We failed to conclude any neoplastic focus after thorough laboratory and imaging study. She minimally responded to methylprednisolone and immunoglobulin therapies. Despite therapy she was severely disabled. Planned abdominal hysterectomy with bilateral salpingo-oophorectomy (AHBSO) was done, histology revealed grade IIA borderline serous papillary carcinoma of ovary. Her neurological deficit responded dramatically to AHBSO. It is first case report who emphasize the response of AHBSO with presentation of anti-Yo antibody-mediated PCD and hidden nidus in post menopausal women.