What are Some Causes of Acute Onset of Loss of Speech Abilities?

Patient Presentation
The senior resident in clinic came to the attending asking if she had seen the patient just scheduled for clinic with a chief complaint of new onset of not speaking in a 3 year old. They both agreed that this needed to be triaged by the nursing staff right away and probably referred to the emergency room. The nurse reported that her parent was very upset saying that the child couldn’t talk but was awake and looking around. This had been going on for about 30 minutes. There was no reported respiratory distress or problems handling secretions and no obvious seizure activity. The nurse had instructed the parent to call emergency services to have the child taken to the emergency room right away. Later the senior resident had heard from another pediatric resident in the emergency room that they were treating the child for a diagnosis of probable seizures as the radiologic evaluation of head imaging was normal and it didn’t appear that the child had other problems such as respiratory causes. They also thought this was an acute onset and not due to selective mutism.

Discussion
Acute mental status changes are worrisome in the least and scary at their worst. Seizures, cerebrovascular problems and central nervous system tumor swirl in professionals’ heads as they take in the history, physical examination and start to evaluate and manage the problem. Usually respiratory distress is recognized by family members and treatment is also sought.

Another cause of loss of speech is selective (or elective) mutism (SM). The child does not speak at all or minimally. When speaking it is usually within a close group of individuals or in certain circumstances such as only speaking to a parent or close family member or only at home and not in a public situation. This can be normal where a child may be quiet or shy especially when entering a new situation or meeting new people. The child though with time will “warm up” and can/will have normal speech. SM is defined as a “…consistent failure to speak in social situations in which there is an expectation to speak… despite speaking in other situations.” It must be occurring for at least one month and be interfering with their activities of daily living. A child with SM continues to behave this way outside the normal range of time and situation, and SM has a strong relationship with anxiety including social phobia. Children who are immigrants/bilingual or learning a new language may also choose not to speak and again this can be a normal behavior for the situation. If this continues outside the normal range of time and situation this could be SM. There is some data which supports a higher rate for children in immigrant families. Prevalence ranges from 0.11 – 2.2% depending on the population with 2.2% cited in some immigrant children’s populations. The cause is not known but felt to be multifactorial with temperament, genetic, developmental and environmental factors playing a part. Treatment is supportive with cognitive behavioral therapy as a common treatment. Some children are also treated with selective serotonin reuptake inhibitors especially fluoxetine. Prognosis is good. Studies cite 58% complete remission, and additional ~30% improvement of symptoms. Others may have another psychiatric disorder with social phobia being very common in this remaining group. Speech therapy also may be helpful with helping the child’s self-efficacy. Alternative or augmented communication and specific therapies for the child and/or caregivers can be appropriate.

Another problem causing acute loss of speech is cerebellar mutism syndrome which is also called posterior fossa syndrome. This occurs after resection of a posterior fossa tumor (can occur with other CNS surgeries too) and occurs in 8-39% of children in studies. There are problems with dysphasia, but also processing speed, memory, cognitive planning and emotional lability. Ability to chew and do emotional phonation remain intact. Swallowing can be affected. Both speech production and swallowing usually improves quickly in patients. Latency to the problem is cited at 0-6 days with resolution from 4 days to 4 months.

Learning Point
The differential diagnosis of the loss of speech abilities includes:

  • Seizure – new onset seizure, tumor
  • Stroke / transient ischemic attack
  • Dysphonia – laryngospasm and vocal cord dysfunction
  • Anxiety/panic attack
  • Selective/elective mutism
    • Traumatic event / Post-traumatic stress disorder
    • Cerebellar mutism syndrome

Questions for Further Discussion
1. How are brain tumors classified? A review can be found here
2. How are seizures classified? A review can be found here
3. How is selective mutism different than being non-verbal? A review can be found here

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for this topic: Speech and Communication Disorders

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Gordon N. Mutism: elective or selective, and acquired. Brain Dev. 2001;23(2):83-87. doi:10.1016/S0387-7604(01)00186-3

Hua A, Major N. Selective mutism. Curr Opin Pediatr. 2016;28(1):114-120. doi:10.1097/MOP.0000000000000300

Pettersson SD, Kitlinski M, Miekisiak G, Ali S, Krakowiak M, Szmuda T. Risk factors for postoperative cerebellar mutism syndrome in pediatric patients: a systematic review and meta-analysis. J Neurosurg Pediatr. 2021;29(4):467-475. doi:10.3171/2021.11.PEDS21445

Selective Mutism. American Speech-Language-Hearing Association. Accessed December 1, 2023. https://www.asha.org/practice-portal/clinical-topics/selective-mutism/.

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

How Can I Remember the Progression of Gesell Figures?

Patient Presentation
A 3-year-old male came to clinic for his health supervision visit. He was happy to see his doctor and gave him a picture as well. “See that is you and that is me,” he says proudly pointing out two circles among what appeared to be individual lines and scribbles.

The pertinent physical exam showed a smiling preschooler with normal vital signs and growth parameters around the 50%. His examination was normal. The diagnosis of a healthy male was made. The doctor asked him to tell him more about the picture. “That’s blue and that is green,” he said pointing out correctly some different colored lines. “Thank you for such a lovely drawing with all its pretty colors. I’m glad you made this for me,” the doctor said as the patient and mother smiled.

Discussion
Observing children in the office setting can provide a wealth of information about the skills, development, health and family. While the office setting may have some limitations, much can be gleaned. For example, the picture above or drawings and writing on a whiteboard in the room can offer lots of possibilities for interacting with the child and also for commenting to the parents about the child’s developmental abilities.

Arnold Gesell, MD, PhD. was a developmental pediatrician who studied and codified how children develop and mature over time. As part of his work, he noticed when certain figures are able to be drawn by children and that this had a consistent developmental progression. These figures are often referred to as Gesell figures. Understanding how children develop and those “milestones” form the basis of screenings, evaluations and interventions to help children and families.

Learning Point
Young children are interested in drawing and writing early on, and each figure they learn to draw builds upon their previous skills. Children need opportunities to practice the figures before one can be perfected. For example, children will learn to scribble around 18 months of age. They then will make continuous circle-like drawings before being able to stop the circle once the continuous line is enclosed. Similarly, children will learn to draw a line when about two years old but orienting more than one line, and especially crossing lines, is more difficult so a child is older when they learn to do this.

Spatial orientation also develops over time and is needed for more complex figures such as crosses, squares, triangles and diamonds. The ability to stop when the figure is enclosed (e.g. circle, square, triangle, diamond) also requires practice and maturity.

Using the outlines of the actual numbers can provide one memory aid for remembering how children learn to draw figures. The number represents the age in years when a child usually can draw the figure. Note that there are 2 different styles for the number four. The first shows a cross which is drawn by younger 4 year olds, while the second one shows a square which is drawn by older 4 years olds. Lines are 2 year old activities with the horizontal being drawn first at a younger age and the vertical being drawn as an older 2 year old.

Case Image

Figure 137 – Ages of children in years with associated figures they are able to draw.

Questions for Further Discussion
1. At what ages does three-dimensional awareness and drawing occur?
2. What is the Goodenough-Harris test?
3. What are indications for referral to an occupational therapist? A review can be found here

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Child Development and Toddler Development.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Arnold Gesell. In: Wikipedia. ; 2023. Accessed November 21, 2023. https://en.wikipedia.org/w/index.php?title=Arnold_Gesell&oldid=1180399578

Gesell Theory. Gesell Program in Early Childhood. Accessed November 21, 2023. https://www.gesell-yale.org/pages/gesell-theory

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa

What is the Differential Diagnosis of a Suprarenal Mass?

Patient Presentation
A full-term newborn male was admitted to the newborn nursery after an uncomplicated vaginal birth and uneventful pregnancy. A right-sided suprarenal mass had been identified on routine ultrasound during the third trimester that was thought to likely be an adrenal hemorrhage. The family history was negative for congenital or genetic problems or cancer.

The pertinent physical exam showed a healthy appearing male with normal vital signs, with a weight of 3.45 kg, length of 52 cm and head circumference of 35 cm were all between 25-50%. His skin was normal without jaundice, or lesions except for a nevus simplex at the base of the skull. His heart and lungs were normal. His abdominal examination was soft, non-tender without hepatosplenomegaly or identifiable masses. He had normal genitalia and remnants of meconium were on the anus from his defecation after birth.

The diagnosis of a healthy male was made. The radiologic evaluation of an ultrasound examination showed a small hypoechoic mass in the area of right adrenal gland that was smaller than previously recorded prenatally. This was felt to be consistent with adrenal hemorrhage. The patient’s clinical course showed that he had additional imaging at 1 month again which showed decreased size of the mass, and it had resolved by 2 months of age.

Case Image

Figure 136 – Sagittal US of the left kidney shows a primarily cystic appearing left adrenal mass. Over months this mass was noted to decrease in size and disappear on serial ultrasound exams.

Discussion
Suprarenal masses (SRM) are not that common (incidence of 1.9/1000 in neonates), but are more commonly diagnosed prenatally because of prenatal ultrasound. Masses can be difficult to accurately diagnose even after birth because their features overlap. Radiographically they can be variable in size, cystic/solid or mixed and with or without calcifications. Common signs and symptoms of SRM include anemia, jaundice, scrotal hematoma and abdominal mass. Signs of asphyxia may also occur. Often patients whose diagnosis is not clear are monitored closely for a few weeks to see how the lesion changes with time. This is especially true for differentiating adrenal hemorrhage from neuroblastoma. This close monitoring does not appear to have poorer outcomes if the patient has neuroblastoma. Overall the prognosis is good to excellent for all diagnoses.

Learning Point
The differential diagnosis of suprarenal masses is relatively short and includes:

  • Primary causes
    • Adrenal hemorrhage
      • Overview: cause is unknown but thought to be because of insult such as sepsis or asphyxia or trauma to vasculature, or a bleeding disorder.
        Has been seen in patients with Beckwith-Wiedemann syndrome.

      • Incidence: 0.28-0.55%
      • Location: right side > left side
      • Timing: 3rd trimester
      • Radiographic findings: usually cystic but can look solid, more enhanced on edge or non-enhancing, features change depending on timing, resolves with time
      • Presentation: asymptomatic or can have signs and symptoms of SRM above
      • Treatment: resolves with time, resection may occur if diagnosis is in doubt
    • Neuroblastoma
      • Overview: cause is tumor from the postganglionic sympathetic neurons.
        Most arise from the adrenal gland (74.5%).
        They are the most common perinatal malignancy and fortunately their histology is generally favorable and they have a less aggressive nature than those that arise in older infants and children.

      • Location: right side > left side
      • Timing: 3rd trimester
      • Radiographic findings: solid or cystic (usually heterogeneous), more enhanced centrally, does not resolve but usually gets larger
      • Presentation: asymptomatic, abnormal vital signs or mass
      • Treatment: depends on type, may have spontaneous regression or need resection. Chemotherapy is often used for stage III or IV.
    • Subdiaphragmatic extralobar pulmonary sequestration (SEPS)
      • Overview: this is dysplastic lung tissue without connection to the airway and with a systemic arterial blood supply. It can occur within the lung and commonly is outside the lung (i.e. pleural, diaphragmatic, abdominal locations).
        It is different than congenital pulmonary airway malformation which is abnormal lung tissue which can have connection to the airway and is usually located within the lung.
        However both SEPS and CAM can occur together.
        SEPS is much less common than adrenal hemorrhage or neuroblastoma as a cause of SRM.

      • Location: Intraabdominal are ~15% of SEPS
      • Timing: 2nd trimester
      • Radiographic findings: cystic (homogenous) which does not resolve, often able to identify arterial blood supply which usually is from abdominal aorta
      • Associated problems: Has associated diaphragmatic defect, and often cardiac defects. Can cause mass effect.
      • Treatment: usually resected
  • Less common
    • Teratoma
    • Other cystic masses
      • Meconium peritonitis
      • Duplications, cystic masses and malformation of various abdominal organs such as adrenal, kidney, spleen, gastrointestinal, lymphatic, etc.

Questions for Further Discussion
1. What is the differential diagnosis of congenital lung malformations? A review can be found here
2. What are the most common pediatric cancers? A review can be found here
3. What are common diagnoses identified on prenatal ultrasound?

Related Cases

To Learn More
To view pediatric review articles on this topic from the past year check PubMed.

Evidence-based medicine information on this topic can be found at SearchingPediatrics.com and the Cochrane Database of Systematic Reviews.

Information prescriptions for patients can be found at MedlinePlus for these topics: Adrenal Gland Disorders and Neuroblastoma.

To view current news articles on this topic check Google News.

To view images related to this topic check Google Images.

To view videos related to this topic check YouTube Videos.

Moon SB, Shin HB, Seo JM, Lee SK. Clinical features and surgical outcome of a suprarenal mass detected before birth. Pediatr Surg Int. 2010;26(3):241-246. doi:10.1007/s00383-009-2531-7

Yao W, Li K, Xiao X, Zheng S, Chen L. Neonatal suprarenal mass: differential diagnosis and treatment. J Cancer Res Clin Oncol. 2013;139(2):281-286. doi:10.1007/s00432-012-1316-x

Birkemeier KL. Imaging of solid congenital abdominal masses: a review of the literature and practical approach to image interpretation. Pediatr Radiol. 2020;50(13):1907-1920. doi:10.1007/s00247-020-04678-1

Schwab ME, Braun HJ, Padilla BE. Imaging modalities and management of prenatally diagnosed suprarenal masses: an updated literature review and the experience at a high volume Fetal Treatment Center. J Matern-Fetal Neonatal Med Off J Eur Assoc Perinat Med Fed Asia Ocean Perinat Soc Int Soc Perinat Obstet. 2022;35(2):308-315. doi:10.1080/14767058.2020.1716719

Author
Donna M. D’Alessandro, MD
Professor of Pediatrics, University of Iowa