Oral Manifestations of Neurological Disorders in Pediatric Patients

The term “neurologic disorder” applies to any condition that is caused by a dysfunction in part of the brain or nervous system, resulting in physical and/or psychological symptoms. Cause of neurogenic disorder may be congenital, perinatal or acquired.

The term “neurologic disorder” applies to any condition that is caused by a dysfunction in part of the brain or nervous system, resulting in physical and/or psychological symptoms. Cause of neurogenic disorder may be congenital, perinatal or acquired.

Neurocutaneous syndromes (phakomatosis) include four important diseases: Sturge-Weber syndrome, tuberous sclerosis, neurofibromatosis of Von Recklinghausen and incontinentia pigmenti. Their specific importance is that of direct relationship to oral cavity and facial structures. Gross head and neck anomalies are rare and Crouzon and Apertz syndrome are two important syndromes of these anomalies having more incidence and neural- oral manifestations. Neoplasia of orofacial region include i) Neoplasias of orofacial nerves and their sheaths (e.g. neurofibroma, neurolemmoma, traumatic neuroma, malignant schwannoma), ii) cranial nerve tumors with orofacial affections (e.g. Trigeminal Neurinoma, Acoustic Neurinoma, and Olfactory Neuroblastoma), and iii) systemic tumors with significant neural and orofacial symptoms (e.g. Gardner syndrome and multiple endocrine neoplasia). Disorders of Cranial nerves V, VII, IX, X and XII are important for a dentist, because all sensory and motor functions in the orofacial region are under the control of these nerves [1].

Most common neurological disorders affecting children with orofacial manifestations include cerebral palsy, epilepsy, cerebro-vascular accident/stroke, multiple sclerosis, Guillain-Barre syndrome etc. A comprehensive study of common neurological disorders and their manifestations are illustrated below.

Cerebral palsy (CP) is a common pediatric disorder occurring in approximately 2-2.5 per 1000 live births. It is a chronic motor disorder resulting from a non-progressive (static) insult to the developing brain. The motor disorders associated with CP are often accompanied by disturbances in coordination, cognition, communication, and seizure disorders. 

Children with CP are at increased risk of developing dental problems from multiple factors including motor and coordination difficulties, as well as limited oral care and hygiene. In addition, approximately 30% of CP patients are undernourished, affecting their dental health. The leading cause of poor nutrition appears to be pseudo-bulbar palsy, affecting the coordination of sucking, chewing, and swallowing. Excessive drooling (sialorrhea) also results from pseudo-bulbar palsy, however, it may also be related to increased production of saliva secondary to an irritating oral lesion, such as dental caries or infection. In addition, gastroesophageal reflux disease (GERD) is another common problem in children with CP causing regurgitation, vomiting, and possible aspiration. The GERD affects the dental health and results in dental erosions. Gingival hyperplasia may be caused by the use of antiepileptic drugs, particularly phenytoin. Drooling of saliva (sialorrhea) appears to be the consequence of a dysfunction in the coordination of swallowing mechanisms (pseudo-bulbar palsy) and mouth opening. Management of this difficult problem is not very effective and includes a trial of an anticholinergic medication, such as Glycopyrrolate and Scopolamine. Bruxism, the habitual grinding of teeth, is a common problem in children with CP, particularly those with severe motor and cognitive deficits. Bruxism may lead to teeth abrasion and flattening of biting surface.

Children with CP may be difficult to handle and uncooperative during dental assessment and management. Sedation and anesthesia are frequently needed in such situations, particularly if invasive procedures are needed. History of respiratory difficulties and seizures represent a particular challenge to induce sedation and anesthesia [2].

A stroke happens when the blood supply to part of the brain is cut off. There are two main types of stroke. Ischemic strokes are caused by a blockage in the blood supply to the brain. Hemorrhagic strokes occur when blood leaks from a burst blood vessel into the brain. Children aged from 28 days old to 18 years may experience weakness or paralysis on one side of the body, facial drooping, speech problems and headaches. These symptoms are most commonly associated with ischemic strokes. Symptoms for hemorrhagic strokes can be vomiting, seizures and occasional headaches. Whilst stroke classically presents with an acute focal neurological deficit such as hemiplegia, speech or gait disturbance, the presentation is highly dependent on the age of the child and may not be specific. The younger the child, more non-specific for their symptoms. Neonates usually present with seizures and symptoms of encephalopathy (irritability, lethargy, excessive sleeps, or poor feeding) or apnea. Toddlers can also present with nonspecific symptoms such as increased crying, drowsiness, irritability, poor feeding, vomiting and sepsis-like features.

The aim of clinical and imaging triage is to differentiate ischemic, hemorrhagic and non-vascular etiologies, triggering different management cascades. Diagnostic methods are MRI brain, non-contrast CT scan. Lab investigations include complete blood count, urinalysis, coagulation profile and when indicated blood culture, ECG and lumbar puncture. Management of acute stroke includes medical therapy to reduce bleeding or thromboembolic occlusion. 2. Thrombolysis with intravenous tissue plasminogen activator (TPA) from 3-4.5 hours after stroke onset [3].

Following stroke patients may experience oral problems like masticatory and facial muscle paralysis, impaired taste sensation, diminished gag reflex and dysphagia. Maintenance of oral hygiene, replacement of missing teeth should be done. Patients with a history of stroke are usually using aspirin and warfarin, hence use of NSAIDs may increase the risk of bleeding and their long-term use may reduce the protective effect of aspirin. Stress reduction during dental visits should be done for which preoperative inhalation N₂O-O₂ or oral anxiolytic can be used. Local anesthesia containing epinephrine can be used in stroke patients but should be used judiciously [4].

It is a relapsing remitting autoimmune inflammatory demyelinating disease of the CNS. It can be caused secondary to trauma. Though the exact cause of M.S. is unknown, genetic susceptibility clearly exists. It is mostly an autoimmune reaction in which major histocompatibility complex (MHC) on chromosome 6p21 has been identified. Infections agents like Epstein Barr virus and human herpes virus 6 are also implicated in the pathogenesis of M.S. The sudden onset of optic neuritis without any other CNS signs or symptoms could be the first symptom of MS. Diplopia, blurring, nystagmus is also commonly seen. Limb weakness is characteristic of MS and can be manifest as loss of strength, fatigue or gait problems. Ataxia may affect the head and neck of MS patients and may cause cerebellar dysarthria. These patients often show sensory impairment including paresthesia and hyperesthesia. An estimated 3–10% of all patients with MS have onset before the age of 18 years. CSF analysis and MRI are done [4].

The care of children with MS needs a multidisciplinary team comprising pediatric and adult neurologists, nurses, physiotherapists, occupational therapists, social workers, psychologists, and psychiatrists. Demyelination in children is managed with corticosteroid therapy. Although there are no specific studies of the dose or effectiveness of corticosteroids, most regimens for severe demyelination use 10–30 mg/kg/dose (to a maximum of 1000 mg/dose) of methylprednisolone by intravenous infusion for 3-5 days. Immunomodulatory therapies Class I level evidence for a reduction in the relapse frequency in adults with MS135–139 has led to the use of interferon beta [5].

MS patients can present with trigeminal neuralgia (TN) with possible absence of trigger zones and continuous low intensity pain which should be managed similar to typical TN. Patients may present with neuropathy of the maxillary (V2) and mandibular branch (V3) of the trigeminal nerve which may result in burning, tingling or reduced sensation. Neuropathy of the mental nerve can cause numbness of the lower lip and chin. Facial weakness and paralysis may be seen in MS patient. Dysarthria may be seen as scanning speech in these patients. Elective dental treatment should be avoided in MS patients during acute exacerbation. These patients may require dental treatment in operating room under general anesthesia. Patient’s relatives or nurse should be appraised about the importance of daily home care of oral hygiene [4].

Guillain-Barre syndrome (GBS) is an immune-mediated disorder of the peripheral nervous system which is triggered by either infectious or noninfectious factors. The disease is often triggered by an infectious process, and it affects children of all ages. GBS is a predominant cause of acute flaccid paralysis which may occur at any age. The predominant complaint of the pediatric GBS includes weakness of the limbs, paresthesia, and pain. Autonomic dysfunctions including fluctuating blood pressure, tachyarrhythmia and bradyarrhythmia, abnormal sweating, papillary abnormalities are also common in pediatric GBS. In addition, respiratory failure requiring mechanical ventilation is a serious short-term complication of GBS [6]. Intravenous immunoglobulin (IV Ig) (0.4g/kg/d, for 5 consecutive days) is a first-line option for adult patients with GBS due to its safety and convenience. Intravenous corticosteroids as an add-on therapy may be administrated [7].

Epilepsy is a disorder that causes seizures or convulsions due to abnormal activity of brain cells, called neurons. The disorder may be caused by head injury, trauma, brain tumors or infections, such as meningitis or encephalitis. Conditions at birth or before birth also may lead to epilepsy, including an insufficient supply of oxygen to the brain, bleeding in the brain or abnormal blood vessels.

Seizures can be partial or generalized type. Grand mal is the most common type of seizure which can occur alone or with other types. Petit mal (absence seizures) occur exclusively in children and frequently disappear during second decade of life. Complete neurologic examination is essential which includes the testing of cranial nerve function, assessment of mental status and testing of motor function. Blood studies like complete blood count, electrolytes, glucose, magnesium, calcium are done to identify any metabolic cause of seizure. All the patients should undergo MRI and CT scan for detection of any pathology in the brain responsible for seizures. An EEG (electroencephalogram) is an important tool for classifying seizure disorder [4].

The first approach in status epilepticus (SE) should focus on airway management and adequate ventilation and circulation. It is important to safeguard patients from injuries caused by uncontrolled movement. It is also important to place the patient in a lateral position to prevent inhalation, and position a peripheral venous catheter. Monitoring vital signs (heart rate, blood pressure, oxygen saturation, and temperature) is essential to evaluate the course of SE. A rapid blood test should be done to recognize hypoglycemia or poisoning. Benzodiazepines are considered the first choice in the initial treatment of seizures. Phenobarbital and Phenytoin are considered second-class drugs to treat seizures. Valproic acid is important in refractory SE. Propofol is an anesthetic agent with anticonvulsant activity. It is used in refractory SE [8].

These patients have high rate of physical injuries, including dental and facial trauma. Hence, precipitation of seizures during dental treatment should be avoided by reducing psychologic stress. Inhalation sedation with nitrous oxide (upto 20%) and oxygen is highly recommended. Phenytoin induced gingival hyperplasia is commonly seen in anterior labial surfaces of the maxillary and mandibular gingiva. Maintenance of oral hygiene by chlorhexidine mouthwash can reduce the inflammation or surgical reduction can be done. Patients taking antiepileptic drugs has marked bone marrow suppression which causes increased chances of infection and prolonged bleeding. Hence, a complete blood count should be done prior to any dental treatment [4].

It is therefore important for the dentist to familiarize with these neurological manifestations for better diagnosis and treatment planning in these patients. A close communication between the dentist and neurologist must be established to find the best recommendations for the patient and to increase the quality of life of these patients.

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