Evaluating the Child with Unsteady Gait
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Review Article Evaluating the child with unsteady gait Mohammed M. Jan, MBChB, FRCP(C). ABSTRACT From the Department of Pediatrics, King Abdul-Aziz University Hospital, Jeddah, Kingdom of Saudi Arabia. Address correspondence and reprint request to: Prof. Mohammed M. S. Jan, Department of Pediatrics, King Abdul-Aziz University يعتبر خلل التوازن أثناء املشي من اﻷعراض الشائعة لدى اﻷطفال Hospital, PO Box 80215, Jeddah 21589, Kingdom of Saudi (Arabia. Tel. +996 (2) 6401000 Ext. 20208. Fax. +996 (2 بقسم الطوارئ واﻷعصاب. تتعدد أسباب خلل التوازن، ولكن E-mail: [email protected] .6403975 من أهم اولويات التقييم اﻷولى هو التأكد من عدم وجود التهاب أو ورم بالدماغ. التعرف علي املسببات احلميدة والغير عصبية ًأيضا eurological disorders are common in Saudi Arabia مهم لتفادى القيام بفحوصات متعددة دون احلاجة إليها أو تنومي Naccounting for up to 30% of all consultations to املريض باملستشفى. في هذه املقالة النقدية نقدم مراجعة حديثة pediatrics.1 Trauma, ingestion, and acute neurological عن تقييم الطفل املصاب بخلل التوازن مع مناقشة الفحوصات disorders are common, mainly as a result of improper الﻻزمة والعﻻج. قد يكون خلل التوازن ناجت عن مرض باملخيخ safety practices of many parents.2 Consanguineous أو مشكلة حسية، ًعلما بأن أمراض املخيخ قد تكون حادة، marriages also add to the problem, resulting in مزمنة، متدهورة، أو متقطعة. وتتعدد أسباب هذه املشكلة و increased prevalence of many inherited and genetic منها اﻹصابات، اﻻلتهابات، أمراض اﻻستقﻻب، العيوب اخللقية، neurological disorders.3,4 Unsteadiness and ataxia are واﻷورام. أما أسباب خلل التوازن الناجت عن مشاكل اﻹحساس relatively common neurological presentations of a فيكون بسبب تأثر في اﻷعصاب الطرفية أو احلبل الشوكي. variety of these acute and chronic disorders. Accurate ًعلما بأن التقييم الدقيق يعتمد علي أخذ التاريخ املرضي بدقة assessment includes detailed history, examination, and ومن ثم الفحص السريري الذي يرشد إلي عدد ونوع الفحوصات then formulation of a differential diagnosis list to guide املطلوبة للوصول إلي التشخيص الدقيق. laboratory investigations.5-7 Many students, residents, Unsteady gait is a relatively common presentation and generalists consider the assessment of neurological to the pediatric emergency and neurology services. disorders one of the most difficult aspects of their Unsteadiness can be due to a wide variety of causes, clinical practice.8-10 In this review, a clinical approach however, the primary concern on initial assessment to the unsteady child is presented with discussion of is to exclude serious disorders such as meningitis, diagnostic considerations, approach to investigation, encephalitis, or brain tumors. Recognizing benign treatment, and prognosis. and non-neurological causes of unsteady gait is Unsteady gait. Gait unsteadiness is not always due essential to avoid unnecessary investigations and Table 1 hospital admission. In this review, a clinical approach to neurological causes ( ). In fact, simple injuries to the unsteady child is presented with discussion of and musculoskeletal etiologies are most common. In diagnostic considerations, approach to investigation, the toddler, skeletal abnormalities should be suspected treatment, and prognosis. Ataxia can be cerebellar as they could mimic other causes of unsteady gait. It or sensory. Cerebellar ataxia can be acute, chronic, is important to recognize benign and non-neurological progressive, or episodic. It may result from trauma, causes to avoid unnecessary investigations and infections, metabolic, degenerative disease, space hospitalization. Functional (hysterical) gait disorders are occupying lesions, or congenital anomalies. Sensory also not uncommon, especially in female adolescents, ataxia is due to peripheral neuropathy involving large and should be suspected when the gait is not wide based myelinated fibers that carry vibration and position or when falls are rare (Table 1). Seizure related ataxia sense, or due to posterior spinal column dysfunction. Accurate assessment depends on detailed history, (post-ictal, nonconvulsive status epilepticus) should examination, and then formulation of a differential be considered in children with frequent or prolonged diagnosis list to guide laboratory investigations. seizures (Table 2). In this situation, the unsteadiness may also be related to antiepileptic drugs, such as Neurosciences 2009; Vol. 14 (1): 3-9 benzodiazepines or barbiturates, and therefore should resolve slowly over a day or 2.3 3 Child with unsteady gait ... Jan Table 1 - Causes of unsteady gait in children. Ataxia. Ataxia (lacking order in Greek) refers to a pathologic abnormality of organization or modulation Foot deformity of movement. Although ataxia is most commonly Skeletal abnormalities (ankle, knee, or hip joint) attributable to cerebellar dysfunction, lesions at Antalgic gait (due to pain) several levels of the nervous system can result in motor Migraine (basilar migraine, benign paroxysmal vertigo) incoordination. Ataxia may be congenital or acquired. Raised intracranial pressure (hydrocephalus) Congenital ataxia is usually associated with central Paretic ataxia (weakness due to upper or lower motor neuron lesion) nervous system malformations. Acquired ataxia can be classified as acute, chronic, or episodic (Table 2). Episodic Cerebellar ataxia Congenital/genetic and chronic progressive ataxias are less common in Traumatic (contusion, hemorrhage, post-concussion, vertebrobasilar children and are usually caused by inherited metabolic dissection) or genetic disorders.11 Toxic and drugs (alcohol, antihistamines, anticonvulsants) Infectious/immune-mediated (chicken pox, ADEM, brainstem Clinical evaluation. History. Most children with encephalitis, MS) ataxia are seen because of refusal to walk or wide-based Malignancy (medulloblastoma, neuroblastoma) or drunken gait. Parents less commonly notice the Paraneoplastic (opsoclonus-myoclonus syndrome) Vascular (stroke, hypertension, AV malformation, blood disorders) involvement of the arms (tremor), head (titubation), Degenerative (ataxia telangiectasia) trunk (inability to sit steadily), and speech (dysarthria). Post-ictal (epileptic ataxia) At presentation, the primary concern is to exclude Sensory ataxia serious causes of acute ataxia, including infections Guillain-Barré syndrome, chemotherapy, heavy metals, B6, B12 and tumors (Table 1). Detailed history will frequently deficiency clarify the cause of the unsteady gait.12 Inquiry of prior Functional ataxia (including Münchhausen by proxy syndrome) or current symptoms of systemic infection should be ADEM - acute demyelinating encephalomyelitis, included. History of trauma, infection, drug ingestion or MS - multiple sclerosis, AV - arteriovenous headaches may suggest important associations (Tables 1 & 2). Recurrent or persistent headache and vomiting or Table 2 - Types of cerebellar ataxia in childhood. diplopia suggests raised intracranial pressure. A common Acute cause of acute ataxia is inadvertent or deliberate drug Trauma ingestion. The child’s activities should be reviewed to Toxic and drugs explore possible exposure to medications, alcohol, and Seizure related (post-ictal, nonconvulsive status epilepticus) Infections/postinfectious household chemicals. Keep in mind that the causes of Vascular (stroke, hypertension, AV malformation, blood disorders) acute ataxia are quite different from those of chronic Malignancy (medulloblastoma, neuroblastoma) or progressive ataxia. Acute ataxia can be related to Paraneoplastic (opsoclonus-myoclonus syndrome) Functional trauma, vascular insults, infection, or drug ingestion, while chronic progressive ataxia suggests an inherited Chronic Congenital (cerebellar hypoplasia, Dandy-Walker and Chiari metabolic, degenerative, or neoplastic etiology (Table 2). malformation) Recent immunizations should be noted, as should the Posttraumatic child’s general state of health prior to presentation. Some Following meningitis/encephalitis Post-tumor resection or radiation inherited metabolic disorders, such as mitochondrial Hypoxic-ischemic insult cytopathies and maple syrup urine disease, may present Progressive with intermittent ataxia that resolves slowly (Table Friedreich ataxia 3). The child may be initially normal in-between the Ataxia telangiectasia Sphingolipidosis (gangliosidosis, Niemann-Pick disease) attacks. These episodes are frequently precipitated by Leukodystrophies (Pelizaes-Merzbacher, Krabbe, metachromatic infections or drug ingestion (for example, valproic acid) leukodystrophy) and result subsequently in chronic progressive sequelae. Mitochondrial disorders (Leigh disease, MERF) Neuronal ceroid-lipofuscinosis Accurate past medical and family histories are important Progressive myoclonic epilepsies (Lafora disease, Uverricht-Lundborg in eliciting the possible diagnosis in these situations. disease) Examination. Physical examination can be difficult Congenital defect of glycosylation 13 Abetalipoproteinemia as ataxic children are often uncooperative and irritable. General examination includes examination of the skin Recurrent Migraine (basilar migraine, benign paroxysmal vertigo) for neurocutaneous signs and examination for meningeal Genetic (autosomal dominant episodic ataxias) irritation. Examination of the back, hips, lower limbs, Metabolic (amino acidopathies, urea cycle disorders, mitochondrial and feet is needed to exclude musculoskeletal causes. disorders) Observation for signs of trauma or arthritis is needed. AV - arteriovenous, MERF - myoclonic epilepsy with ragged red fibers Examination of the eyes may provide some clues (for 4 Neurosciences 2009; Vol. 14