Monomelic Amyotrophy/Hirayama Disease: Surgical Outcome in a Large Cohort of Indian Patients.

BACKGROUND: Hirayama disease (HD) is a cervical compressive myelopathy. Anterior cervical discectomy and fusion (ACDF) is identified as the best surgical approach. We evaluated surgical outcomes and factors influencing ACDF in HD. METHODS: Between 2015 and 2019, 126 patients with HD underwent ACDF. Contrast magnetic resonance imaging of the cervical spine in full flexion was performed. Clinical examination and preoperative/postoperative assessment of hand function using Fugl-Meyer assessment, Jebsen-Taylor hand function test, and handheld dynamometry were performed at 3-monthly intervals for 1 year. Surgical outcomes were assessed as per the Odom criteria and Hirayama outcome questionnaire. RESULTS: Age at onset and duration of illness were 12-31 years (mean, 18 ± 2.7) and 1-96 months (32.7 ± 24.4), respectively. All patients had progressive weakness and wasting of the affected limb. Cord atrophy was seen in 97.1%, with epidural detachment and engorgement of the posterior epidural venous plexus in all. All patients underwent ACDF. Of these patients, 54% had an excellent/good outcome and 39% had a satisfactory outcome as per the Odom scale at last follow-up (mean, 44.9 ± 16.5 months) after surgery. Handheld dynamometry showed improvement from preoperative values to 1 year follow-up. Duration of illness and age at onset had a negative correlation and the preoperative Fugl-Meyer score had a positive correlation with improvement. CONCLUSIONS: ACDF resulted in remarkable improvement or stabilization in neurologic deficits in many patients with HD. Because motor disability ensues over time, early surgical intervention during the progressive phase is advocated.

Surgical Management and Outcomes of Aneurysms of Posterior Inferior Cerebellar Artery: Location-Based Approaches with Review of Literature.

Background Posterior inferior cerebellar artery (PICA) is a tortuous, variable, and uncommon site for aneurysms. Surgical management of PICA aneurysms involves careful selection of approach based on the location of the aneurysm and meticulous dissection of the neurovascular structures and perforators. Materials and Methods We did a retrospective review of all the PICA aneurysms operated at our institute in the past 10 years along with the site, presentation, and approach used for the same. Preoperative World Federation of Neurosurgical Society scores and follow-up modified Rankin scores (mRS) were also evaluated. During the same period, data for intervention cases of PICA aneurysm were also collected with follow-ups for a comparative analysis. Results A total of 20 patients with 21 PICA aneurysms were reviewed. All the reviewed cases presented with subarachnoid hemorrhage, and the most common location was the lateral medullary segment and vertebral artery (VA)-PICA junction. Midline approaches were used for distal PICA cases, with far-lateral approach reserved for anterior medullary/VA-PICA junction. No lower cranial nerve palsies were recorded at follow-up. Four cases needed cerebrospinal fluid diversion and two developed cerebellar infarcts. All cases were mRS 0 to 2 at follow-up. Conclusion Our series compares well with some of the larger surgical series of PICA aneurysms. This may be due to early referral patterns and early surgery (<24 hours) policy at our institution. Anatomical knowledge of PICA anatomy and sound perioperative management are keys to good outcomes in these cases.

Peripheral Nerve Injuries: Electrophysiology for the Neurosurgeon.

Peripheral nerve injuries are a heterogeneous and distinct group of disorders that are secondary to various causes commonly including motor vehicle accidents, falls, industrial accidents, household accidents, and penetrating trauma. The earliest classification of nerve injuries was given by Seddon and Sunderland, which holds true till date and is commonly used. Neuropraxia, axonotmesis, and neurotmesis are the three main types of nerve injuries. The electrophysiological studies including nerve conduction studies (NCS) and electromyography (EMG) play a key role and are now considered an extension of the clinical examination in patients with peripheral nerve injuries. The electrophysiological results should be interpreted in the light of clinical examination. These studies help in localizing the site of lesion, determine the type and severity of lesion, and help in prognosticating. In neuropraxia, the compound muscle action potential (CMAP) and sensory nerve action potential (SNAP) are elicitable on stimulating the nerve distal to the site of the lesion but demonstrate conduction block on proximal stimulation. The electrodiagnostic findings in axonotmesis and neurotmesis are similar. After few days of injury, Wallerian degeneration sets in with failure to record CMAP and SNAP. Intraoperative technique involves recording from the peripheral nerves during the intraoperative period and has proved useful in the surgical management of nerve injuries and helps in identifying the injured nerve, to determine whether the nerve is in continuity and in localizing the site of lesion. Intraoperative monitoring also helps in identifying the nerve close to an ongoing surgery so that surgical damage to the nerve can be prevented.

Intracranial Actinomycosis Manifesting as a Parenchymal Mass Lesion: A Case Report and Review of Literature.

BACKGROUND: Intracranial actinomycosis is a rare bacterial infection with no characteristic clinical or radiologic diagnostic features. The usual presentation is similar to pyogenic brain abscess or osteomyelitis with or without pachymeningitis. Intracranial actinomycosis rarely manifests as a parenchymal mass lesion. A high index of suspicion is warranted in a patient with immunosuppression or predisposing factors, such as dental procedure, sinusitis, cardiac septal defect, craniofacial trauma, cranial surgery, lung infection, or abdominopelvic infection. CASE DESCRIPTION: A young woman presented with a right parietal parenchymal lesion with involvement of the calvaria and pericranium. She had no predisposing factors for intracranial actinomycosis. She underwent complete microsurgical excision of the lesion followed by prolonged antibiotic therapy. She experienced a good functional recovery. CONCLUSIONS: Intracranial actinomycosis manifesting as a parenchymal mass lesion is extremely rare compared with abscess and pachymeningitis. Histopathologic examination remains the mainstay of definitive diagnosis, as culture may be negative in significant number of cases. Aggressive surgical excision with prolonged antibiotic therapy enhances the chances of a good functional outcome.

Hirayama disease/cervical flexion-induced myelopathy progressing to spastic paraparesis: A report on three cases with literature review.

Hirayama disease (HD)/cervical flexion-induced myelopathy (CFIM) is a lower motor neuron disease conventionally affecting a single upper extremity. We describe three men progressing after a long stable period to develop severe spastic paraparesis and bladder disturbances as a protracted implication of HD. The age at onset was 20, 24, and 15 years, while the age at presentation was 27, 41, and 57 years, respectively. The second phase of disease progression occurred after 4, 13, and 28 years of stationary period. All had CFIM with characteristic magnetic resonance imaging features as observed during progressive stages. The anterior dural shift extended variably from C4 to D4 levels with a median value of 5 mm and was maximum at C6 to C7 levels, pushing the cord anteriorly causing compression. This study emphasizes the need to recognize this unusual subgroup of HD and mandates long-term follow-up with timely intervention in arresting the progression/improving the deficits.

Unusual cause of high cervical myelopathy-C1 arch stenosis.

INTRODUCTION: High cervical myelopathy can be rarely caused by the developmental anomalies of atlas. Patients with C1 arch stenosis can present in early childhood or later in life. In symptomatic patients, posterior decompression at atlas is mandatory. We report the first clinical series of 20 patients of C1 arch stenosis in the English literature. MATERIALS AND METHODS: This is retrospective case series having a cohort of 20 patients with congenital C1 arch stenosis. RESULTS: There were 12 pediatric (age <18 years) and 8 adult patients. Mean age was 22.85 years. Syndromic association was seen in four patients. Following decompressive surgery, these patients noticed a symptomatic improvement. CONCLUSIONS: Isolated C1 arch stenosis is a surgically curable rare cause of high cervical myelopathy and responds well to surgery.

Central Nervous System Actinomycosis-A Clinicoradiologic and Histopathologic Analysis.

INTRODUCTION: Actinomycosis is an uncommon chronic suppurative infection that rarely affects the central nervous system (CNS). It is caused by filamentous Gram-positive anaerobic bacteria that is a normal commensal but causes suppurative and granulomatous inflammation after disruption of anatomical barriers. We report the largest series of 17 histologically confirmed cases of CNS actinomycosis and review clinical, imaging, and histopathologic features. METHODS: All histologically confirmed cases of CNS actinomycosis diagnosed between January 2010 and June 2016 were retrieved from the neuropathology records. The demographic profile, clinical, radiologic, microbiologic, and histologic features, treatment, and clinical outcomes were reviewed. RESULTS: Seventeen cases were histopathologically diagnosed to have CNS actinomycosis. Nine of these were primarily admitted and managed in our institute, whereas the remaining 8 were referred to us for histopathologic diagnosis. Mean age at presentation was 31.4 years, with male predilection (3.25:1). Mean duration of symptoms was 2.95 months. Systemic symptoms were noted in 5 patients, although no systemic focus was detectable. Pachymeningitis was most common type (9; 52.94%), and chronic abscess was identified in 7. History of previous surgery for osteomyelitis was forthcoming in 3. All patients underwent surgical excision/aspiration of the lesions. Histologically, lesions revealed characteristic suppurative granulomatous response with giant cells and actinomycotic colonies were detected within necrotic centers. Cultures failed to grow Actinomyces in all. Follow-up data were available in 9 patients managed in our institute, and all had good outcomes at the median follow-up period of 32 months after antibiotic treatment for mean period of 8.4weeks. CONCLUSIONS: Histopathology remains the cornerstone for diagnosis of actinomycosis as on culture confirmation is very rare.

Intrafamilial phenotypic variations in familial cases of cervical flexion induced myelopathy/Hirayama disease.

Hirayama disease is generally considered to be a sporadic disorder, except for a few reports of familial occurrence. In this study, we describe eight patients from four families with cervical flexion induced myelopathy (CFIM)/Hirayama disease (HD) and intra-familial phenotypic variations. All underwent clinical and electrophysiological evaluation, while seven of them had contrast MR imaging of cervical spine in flexion. There was significant intra-familial variability: distal bimelic form in four patients, classical monomelic form in three and proximo-distal form in one. Irrespective of the clinical phenotype, MRI showed characteristic dynamic changes of posterior dural detachment with prominent epidural enhancement extending variably from C3 vertebral level to dorsal spine in six patients. One patient with 28 years of illness, had only lower cervical cord atrophy without dynamic changes while another patient demonstrated forward dural displacement with epidural enhancement even after 38 years of disease duration.

Multimodality Management of Cavernous Sinus Hemangiomas-An Institutional Experience.

Objective Cavernous sinus hemangiomas (CSHs) are benign lesions accounting for less than 2% of the cavernous sinus tumors. They provide a formidable surgical challenge because of their vascularity and their being surrounded by critical neurovascular structures. In this study, one of the largest in available literature, we analyze our experience in the management of these unusual tumors and review the available literature. Materials This is a retrospective analysis of patients who were managed surgically (both microsurgical and Gamma knife radiosurgery [GKRS]) for CSH at our Institution from 2007 to 2015. Complete demographic, clinical-radiologic surgical records were analyzed. Follow-up data were collected from the hospital records. Results Total 23 patients were managed. Among these, 15 patients underwent microsurgery (group 1) whereas 8 underwent GKRS (group 2). Predominant clinical presentation in both the groups included headache and involvement of multiple cranial nerves. Five patients in group 1 had deteriorating vision. The volume of tumors ranged from 29 to 115 cm 3 (mean = 64.57 cm 3 ) in group 1 and from 2.1 to 11.6 cm 3 in group 2. GKRS was performed with a mean dose of 13 Gy, an average isodose line of 50% with an average coverage of 96%. In group 1, the follow-up period ranged from 6 to 62 months (mean = 29.4 months). The extraocular movement (EOM) preservation rate in our series was not favorable, as most patients presented late with large tumors and established deficits. Recurrence/residual tumor was seen in two cases. In group 2, the follow-up was 5 to 48 months. All of them showed significant reduction in size. Conclusion Both surgery and radiosurgery are highly effective in the management of CSHs. They are complementary to each other, with individual characteristics-the size and volume of the lesion-being the main factors in deciding the choice of treatment.

"Os" omovertebrale variants prove it to be a misnomer.

There are only a few case reports comprising exclusively of os omovertebrale. Here, we discuss various types, varied clinical presentations, associated abnormalities, and management strategies of this abnormality. We retrospectively analyzed three patients with os omovertebrale and their clinical presentation, and also reviewed the limited available literature. Patients were evaluated clinically and radiologically to diagnose this abnormality as well as the associated abnormalities. Two patients were operated for neurological deficits attributed to this deformity. It is quite common to miss this abnormality when it is of osseocartilaginous or fibrous type, especially when it is asymptomatic and not associated with Sprengel's scapula. By analyzing our patients and other reported cases, we have proposed a classification system. In addition, we discuss varied clinical presentations and their practical application. Os omovertebrale is a rare abnormality. It should be kept in mind and managed when encountered in clinical practice. The classification and clinicoradiological correlations described here can be useful to individualize management issues.

Bicycle accident-related head injuries in India.

OBJECTIVES: To describe the epidemiology of head injuries sustained due to bicycle accidents in India. MATERIALS AND METHODS: Data were retrospectively collected over a period of six months (15 May 2011 to 15 November 2011). Demography of patients, Glasgow coma scale (GCS), clinical and imaging findings, and mortality and outcome using Glasgow outcome scale (GOS), Rivermead post-concussion symptom questionnaire (RPCSQ) and Rivermead head injury follow-up questionnaire (RHFUQ), were analyzed. Outcome was assessed by telephonic interview. RESULTS: There were 108 patients (100 males) with mean age of 27.7 years. Seventy-four (68.5%) were from rural areas. Accidents due to vehicular collision accounted for 60 (55.6%) cases. None wore a helmet. The admission GCS was 14-15 in 68.5% cases, 13-3 in 31.5%. The risk of moderate to severe injuries was increased among working laborers (OR = 5), and patients with loss of consciousness (OR = 4). Sixty-three (49%) patients had abnormal computed tomography (CT) findings; most common finding was skull fracture 25 (23.1%). Four patients needed surgery. The GOS assessment at three to six months revealed favorable outcome in 66 patients (61.1%) and death in 8 (7.4%). The common post-concussion symptoms were headache, fatigue, and poor concentration. CONCLUSION: The majority of hospitalized cyclists were from a rural background and of the lower income group. After three months the majority of patients had good recovery with few persistent concussion symptoms.

Domestic animal-related neuro-trauma: An account, from a tertiary institute.

CONTEXT: Experience of animal-related neurotrauma at an apex institute, National Institute of Mental Health and Neurosciences, Bangalore, India. AIMS: The aim of this study is to review epidemiology, clinical findings, and outcome of animal-related traumatic brain injury (TBI) evaluated and treated at our institute. SETTINGS AND DESIGN: A retrospective study consisting of demographic data, clinical findings, radiological details, and outcomes. MATERIALS AND METHODS: The clinical and imaging records of 30 patients treated for animal-related TBI at the emergency services, from January to July 2010. Outcome was assessed by Glasgow outcome scale (GOS). STATISTICAL ANALYSIS USED: SPSS 15.0 version, descriptive statistics. RESULTS: A total of 6190 neurotrauma cases were evaluated and treated during the study period. Among them, 30 (0.48%) were animal-inflicted injuries. Of these cases, animal-vehicle collision and directly animal-inflicted injuries were 15 (50%) each. The mean age of patients were 39.46 (6-71 years). Twenty-nine (96.66%) cases were from rural areas. Twenty-three (76.6%) had mild, 6 (20%) had moderate, and 1 (3.3%) had severe head injury (Glasgow coma scale). Four (13.3%) patients had abnormal pupillary reaction. Associated injuries were found in 25 (83.3%) patients. CT scan was abnormal in 50% (15/30), common finding was contusion in 8 (26.6%) patients, followed by edema in 6 (20%). There were 3 (10%) spine injuries, 1 (3.3%) internal carotid artery (ICA) dissection, and 2 (6.6%) brachial plexuses injuries. Three (10%) required surgery, and 1 (3.3%) patient expired. As per the GOS, good recovery was seen in 8 (80%) patients, moderate disability in 1 (10%), and vegetative state in 1 (10%) patient.