SAUDI MEDICAL JOURNAL

ORIGINAL ARTICLES

A profile of childhood neuropathies at a University Hospital in Oman

Roshane Koulo, Alexander Chackoe, Hashime Javed, Khalide Al-Hinai, Matthewe Zachariah, Srinivas Bulusue, Thirapathur V Raoos

ABSTRACT

Objectives: To analyze all cases of childhood neuropathies (under 14 years of age) and report on their profile, pattern, clinical features and management.

Methods: Children with acute flaccid paralysis, longstanding weakness of extremities, neuroregression and children receiving anti cancer drugs with symptoms suggestive of neuropathy were evaluated for evidence of peripheral neuropathy. The evaluation of children with acute flaccid paralysis was a prospective study from January 1992 through to December 2000. The rest of the patients were studied retrospectively from the hospital medical records, pediatric neurology outpatient clinic and the neurophysiology laboratory, Sultan Qaboos University Hospital, Al-Khod, Oman

Results: Eighty-two (39 Male: 43 Female) children were found to have peripheral neuropathy. Acute Guillian-Barre syndrome was the most common with 37 children (45.1%), followed by genetic neuropathies [hereditary motor and sensory neuropathy with 17 (20.7%), hereditary sensory and autonomic neuropathy with 2 (2.4%), hereditary spastic paraplegia associated neuropathy with 9 (11%) and metachromatic leucodystrophy with 9 (11%)]. Chronic inflammatory demyelinating neuropathy was seen in 5 (6.1%) and vincristine induced neuropathy in 3 (3.5%) children.

Conclusions: Acute Guillian-Bare syndrome is the most common neuropathy amongst the acquired neuropathies. The treatable neuropathies constituted 54.7% (45 children) and the preventable genetic neuropathies accounted for the remaining 45.3% (37 children)

Saudi Medical Journal 2002; Vol. (4): 450-456

The childhood neuropathies are mainly constituted by hereditary neuropathies, neuropathies associated with metabolic and degenerative diseases of the nervous system. Seventy one percent of cases were found to be of genetic origin in one series and hereditary motor and sensory neuropathies were confirmed in over 40% of cases.1 Modern immunohistochemical techniques and genetic studies combined with clinical and electrophysiologic work up, have brought radical changes in the classification and understanding of the pathophysiology of hereditary neuropathies.2,3 These advances have made the use of certain invasive diagnostic procedures obsolete in the evaluation of disorders of the motor unit.4 Amongst the acquired neuropathies, Guillian-Barre syndrome (GBS) and chronic inflammatory demyelinating neuropathy (CIDP) constitute nearly one 3rd of the neuropathies of childhood.5 Neuropathies of childhood differ from the adult population. Hereditary neuropathies and GBS form the main group in childhood, while neuropathies of systemic disorders particularly diabetes mellitus constitute the largest group in adults. The present study was conducted at Sultan Qaboos University Hospital (SQUH) to ascertain the profile of childhood neuropathies in the Sultanate of Oman.P> FONT>
Methods.FONT> FONT>The study was carried out at SQUH, which is a tertiary care hospital for the country. All children under 14 years of age, confirmed to have neuropathy on electrophysiologic (nerve conduction) studies formed the subjects of the study. The study was prospective for acute GBS and CIDP, as part of an acute flaccid paralysis (AFP) surveillance, aimed at eradication of poliomyelitis from the Sultanate of Oman. The surveillance system and data collection began in January 1990.6 Under this program, the Ministry of Health (MOH) implemented AFP surveillance at all levels of the national health care system involving all government hospitals, health facilities and private clinics. Training programs for AFP surveillance was provided to all health care personnel throughout the country, including physicians, nursing staff and paramedical workers. The patients included were from all over the country. All the AFP patients underwent blood examinations for immunoglobulin study, polio antibody titer and nerve conduction studies. At least 2 stool cultures for polio or other viruses were mandatory in all cases. The population of Oman is 1,483,226 of which 714,280 are aged under 15 years based on the census carried out from November 1992 through to January 1993.7 Other children with neuropathies were picked up retrospectively from neurophysiology laboratory records, outpatient and inpatient records of our hospital. All children were admitted at one time or the other for detailed investigations. The diagnosis of different neuropathies was based on an internationally accepted, well-defined criteria. Acute GBS diagnosis was based on a criteria8,9 defining it as an acute progressive symmetrical weakness of extremities with areflexia. The cerebro spinal fluid (CSF) showing albuminocytological disassociation and electrophysiology revealing features of demyelinating/axonal neuropathy. The diagnosis of CIDP was based on clinical features of acquired demyelinating neuropathy and progression of more than 2 months duration,10 with nerve conduction studies showing features of demyelination with partial conduction block, temporal dispersion and focal slowing of multiple nerves. A nerve biopsy was carried out when the diagnosis was in doubt. The diagnosis of hereditary motor and sensory neuropathy (HMSN) was made in children with a family history of chronic neuropathy and features of uniform nerve conduction velocities on electrophysiologic sutdies.11,12 Hereditary sensory and autonomic neuropathy (HSAN)11 and hereditary spastic paraplegia (

From the Division of Pediatric Neurology, (Koul, Chacko, Javed, Al-Hinai), Pediatric Hematology, (Zachariah), Departments of Child Health, and Clinical Neurophysiology, (Bulusu) and Department of Pathology (Rao), Sultan Qaboos University Hospital, Al Khod, Sultanate of Oman.
Received 30th June 2001. Accepted for publication in final form 17th November 2001.
Address correspondence and reprint request to: Dr. Roshan Koul, Senior Consultant, Pediatric Neurology, Child Health Ward (1), Sultan Qaboos University Hospital, PO Box 38, Al Khod 123, Sultanate of Oman. Tel. +968 515 745 Fax. +968 513419/513128. E-mail: roshankoul@hotmail.com, rkoul@omantel.net.om

Erratum notice issued in Saudi Medical Journal 2002; Vol. 23 (6): 754.

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