While a prisoner-of-war in Malaya from 1942-1945, a 29-year-old man developed a painful sensorimotor neuropathy, bilateral central scotomata and sensorineural deafness. Examination 34 years later, after a long period of adequate nutrition, revealed considerable residual deficit. Nerve conduction studies suggested axonal degeneration with prominent collateral reinnervation. This case of Strachan's syndrome is reported to draw attention to the limited functional recovery and to focus attention on this condition at a time when famine conditions are rife in Southeast Asia.
Matched MeSH terms: Peripheral Nervous System Diseases/complications; Peripheral Nervous System Diseases/diagnosis*
A patient with organophosphate poisoning who survived the acute phase and subsequently developed delayed neuropathy is presented. The features of this form of delayed neuropathy are described and the implications in our local context discussed.
Matched MeSH terms: Peripheral Nervous System Diseases/chemically induced*
An unusual case of syringomyelia secondary to a cauda equina meningioma involving the conus medullaris is described. The tumour was totally removed with decompression of an adjacent cyst and syrinx resulting in resolution of the symptoms and radiological appearance.
Matched MeSH terms: Peripheral Nervous System Neoplasms/complications*; Peripheral Nervous System Neoplasms/surgery
Neural leprosy is rare. This is a report of a 63-year-old Indian man who had long standing multiple peripheral neuropathy. The slit skin smear for acid-fast bacilli of Mycobacterium leprae was positive. The skin and nerve biopsies were normal. He was treated with rifampicin, dapsone and clofazimine.
Matched MeSH terms: Peripheral Nervous System Diseases/etiology*
Out of 218 fractures of humeral shaft treated in the department, 23 (10.5%) of them developed non-union. 14/23 (60.9%) fracture were located in middle third. Transverse (52.2%), short oblique (17.4%) and comminuted fracture (13.0%) constituted about 82% of all initial fracture pattern. Twelve cases (52.5%) were initially treated with hanging cast. Radial nerve palsy occurred in 4/23 (17.4%) of patient and all of them located at lower third of humerus and only one recovered after eight weeks of injury. Factors such as middle third comminuted opened fractures, soft tissue interposition, improper immobilization and poor patient compliance were found to be directly associated with the non-union. All non-unions healed following plating and bone grafting. Overall 17/23 patient (74%) had good results, 4/23 (17%) fair and 2/23 (9%) with poor functional results.
Matched MeSH terms: Peripheral Nervous System Diseases/etiology
Mental nerve neuropathy is an important presenting complaint that may be encountered by dental surgeons in their daily practise. There are various pathological processes that could bring about this symptom, ranging. from simple dental cause to vague, life threatening diseases. We present three cases of mental paraesthesia of different aetiologies. A literature review on mental nerve neuropathy related to malignancies and infection is discussed. The importance of a thorough chair side history taking, clinical examination and relevant investigations are emphasised in a suggested clinical approach to obtaining the diagnosis of a numb chin.
Matched MeSH terms: Peripheral Nervous System Diseases
Magnetic resonance imaging is gaining importance in the diagnosis of nerve and muscular disorders. The ability of magnetic resonance imaging to delineate the different muscles and the nerve in any plane has made the differentiation between the changes of neuropathy, denervation, and nerve entrapment possible. Although findings on magnetic resonance imaging are non-specific, their use, coupled with clinical symptoms and electromyographic findings, allow an accurate diagnosis to be made without resorting to invasive biopsies.
Matched MeSH terms: Peripheral Nervous System Diseases/chemically induced*; Peripheral Nervous System Diseases/diagnosis*
Exposure to organic solvent during work activities has been known to be associated with significant clinical conditions such as peripheral neuropathy and neurobehavioral changes. Three reported cases of peripheral neuropathy most likely due to exposure to chronic organic solvent were reported recently in Bentong Malaysia. These cases showed a compatible clinical history, occupational history, examination and neurological study that link with peripheral neuropathy due to organic solvent poisoning. Proper education and training with review of engineering control measures are among preventive and corrective measures recommended. More comprehensive study in order to establish significant causal-effect relationship as documented evidence is recommended.
Matched MeSH terms: Peripheral Nervous System Diseases
We examined the effects of pesticides on the central and peripheral nervous system in the setting of a tobacco farm at a developing country. Maximal motor and sensory nerve conduction velocities (MCV and SCV, respectively) in the median, sural and tibial nerves, postural sway, and brain-evoked potentials (auditory event-related and visual-evoked potentials) were measured in 80 male tobacco farmers and age- and sex-matched 40 controls in Kelantan, Malaysia. Median SCV (finger-wrist) in farmers using Delsen (mancozeb, dithiocarbamate fungicide), who showed significant decrease of serum cholinesterase activities, were significantly lower compared with the controls. Sural SCV in farmers using Fastac (alpha-cypermethrin, pyrethroid insecticide) and median MCV (elbow-wrist) in farmers using Tamex (butralin, dinitroaniline herbicide) were significantly slowed compared with their respective controls. In Delsen (mancozeb, dithiocarbamate) users, the power of postural sway of 0-1 Hz was significantly larger than that in the controls both in the anterior-posterior direction with eyes open and in the right-left direction with eyes closed. The former type of sway was also significantly increased in Tamaron (methamidophos, organophosphorus insecticide) users. In conclusion, nerve conduction velocities and postural sway seem to be sensitive indicators of the effects of pesticides on the central and peripheral nervous system.
We report a case of sciatic nerve entrapment resulting in a patient experiencing pain over the posterior aspect of the knee, simulating a Baker's cyst. Surgical exploration revealed a tight fibrous arch compressing the distal portion of the sciatic nerve, proximal to its bifurcation. Decompression of this entrapment led to complete relief of symptoms. This form of presentation is rare and should be considered as a differential diagnosis when a patient presents with complaints of pain in the back of the knee. Keywords: Sciatic nerve compression, pain in the back of the knee
Matched MeSH terms: Peripheral Nervous System Diseases
INTRODUCTION: Peripheral diabetic neuropathy, which is a cause of increasing morbidity and mortality following foot ulcers and amputations, is a burden to health and the economy. Various adjunct treatments to improve neuropathy have been introduced into the market; one such treatment is monochromatic infrared energy (MIRE) therapy, which claimed to produce promising results. This study aimed to evaluate the effects of MIRE on diabetic feet with peripheral neuropathy.
METHODS: A randomised controlled, single-blinded study was conducted at Hospital Universiti Sains Malaysia from February 2008 to October 2008. A total of 30 feet from 24 patients were studied. Neuropathy was screened using the Michigan neuropathy scoring instrument, followed by an assessment of the current perception threshold using a neurometer at frequencies of 2,000 Hz, 250 Hz and 5 Hz. The feet were randomised to receive either daily MIRE or sham treatment for a total of 12 treatments. Each foot was then reassessed using the neurometer at six weeks and three months following treatment.
RESULTS: The data obtained was analysed using a non-parametric test to compare the pre- and post-treatment groups. No significant difference was found between the neuropathic foot of diabetic patients in both the MIRE and sham groups.
CONCLUSION: No improvement of neuropathy was observed following MIRE treatment in the neuropathic feet of diabetic patients.
Matched MeSH terms: Peripheral Nervous System Diseases
Extracranial schwannomas in the head and neck region are rare neoplasms. The tumours often present as asymptomatic, slowly enlarging lateral neck masses and determination of the nerve origin is not often made until the time of surgery. Preoperative diagnosis maybe aided by imaging studies such as magnetic resonance imaging or computed tomography, while open biopsy is no longer recommended. The accepted treatment for these tumors is surgical resection with preservation of the neural pathway. We report four cases of cervical schwannomas that we encountered at our center during four years of period. The clinical features, diagnosis and origin, management and pathological findings of these benign tumors are discussed.
Matched MeSH terms: Peripheral Nervous System Neoplasms/diagnosis*; Peripheral Nervous System Neoplasms/surgery
Vagal nerve schwannoma is extremely rare. The majority of cases present with a slow growing neck swelling without neurological deficit. Magnetic resonance imaging is the gold standard investigation to establish a pre-operative diagnosis. We report a case of a 32-year-old man with an extensive right vagal nerve schwannoma involving the right jugular foramen and parapharyngeal space. The tumour was resected via a transcervical approach. Complete excision of the tumour is the key to prevent recurrence.
Matched MeSH terms: Peripheral Nervous System Neoplasms/diagnosis*; Peripheral Nervous System Neoplasms/surgery
The cytoplasmic dynein heavy chain (DYNC1H1) gene has been increasingly associated with neurodegenerative disorders including axonal Charcot-Marie-Tooth disease (CMT2), intellectual disability and malformations of cortical development. In addition, evidence from mouse models (Loa, catabolite repressor-activator (Cra) and Sprawling (Swl)) has shown that mutations in Dync1h1 cause a range of neurodegenerative phenotypes with motor and sensory neuron involvement. In this current study, we examined the possible contribution of other cytoplasmic dynein subunits that bind to DYNC1H1 as a cause of inherited peripheral neuropathy. We focused on screening the cytoplasmic dynein intermediate, light intermediate and light chain genes in a cohort of families with inherited peripheral neuropathies. Nine genes were screened and ten variants were detected, but none was identified as pathogenic, indicating that cytoplasmic dynein intermediate, light intermediate and light chains are not a cause of neuropathy in our cohort.
Matched MeSH terms: Peripheral Nervous System Diseases/genetics*
The cytoplasmic dynein-dynactin genes are attractive candidates for neurodegenerative disorders given their functional role in retrograde transport along neurons. The cytoplasmic dynein heavy chain (DYNC1H1) gene has been implicated in various neurodegenerative disorders, and dynactin 1 (DCTN1) genes have been implicated in a wide spectrum of disorders including motor neuron disease, Parkinson's disease, spinobulbar muscular atrophy and hereditary spastic paraplegia. However, the involvement of other dynactin genes with inherited peripheral neuropathies (IPN) namely, hereditary sensory neuropathy, hereditary motor neuropathy and Charcot-Marie-Tooth disease is under reported. We screened eight genes; DCTN1-6 and ACTR1A and ACTR1B in 136 IPN patients using whole-exome sequencing and high-resolution melt (HRM) analysis. Eight non-synonymous variants (including one novel variant) and three synonymous variants were identified. Four variants have been reported previously in other studies, however segregation analysis within family members excluded them from causing IPN in these families. No variants of disease significance were identified in this study suggesting the dynactin genes are unlikely to be a common cause of IPNs. However, with the ease of querying gene variants from exome data, these genes remain worthwhile candidates to assess unsolved IPN families for variants that may affect the function of the proteins.
Matched MeSH terms: Peripheral Nervous System Diseases/genetics*; Peripheral Nervous System Diseases/pathology