Case: 14-year-old Girl with Dizziness

by Roy Strowd, MD

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    00:01 So let's dive into each of these.

    00:02 But first, let's go over a case and understand why it's important to understand cerebellar circuitry.

    00:09 This is a 14-year-old girl who presents to the emergency department with several month history of headaches, nausea, and vomiting.

    00:15 Her family reports that she's had headaches for several years, that began to increase in frequency and severity about three months ago.

    00:24 Now, she has early morning headaches, early morning vomiting or emesis.

    00:28 Her exam reveals a slight sway to the right, when she's sitting, and consistent truncal ataxia.

    00:35 Impaired finger-to-nose finger bilaterally and the inability to perform heel-toe walking all cerebellar signs.

    00:42 MRI is performed and reveals a lesion in the posterior fossa.

    00:46 And you can see that in the figure here a large lesion in the midline of the cerebellum.

    00:51 She undergoes surgery for gross total resection and pathology returns a medulloblastoma.

    00:56 That's one of those childhood brain tumors that can affect the midline of the cerebellum.

    01:03 Postoperatively, the patient's initially appears to be doing well without headache or new symptoms until post-op day two, when she's found to be awake, and purposeful but apathetic.

    01:14 She's not interested in doing anything.

    01:16 She's lacks motivation.

    01:18 She would not produce speech and refuse to eat.

    01:20 So she is not able to talk and is not eating.

    01:23 She's incontinent of urine.

    01:25 She has emotional ability, and exam shows prominent cerebellar dysmetria, dysdiadochokinesia, which is problem with rapid alternating movements, no papilledema or other cortical signs.

    01:37 So what's going on with this patient? Does this sound like drug-induced cerebellar dysfunction, cerebellar mutism, obstructive hydrocephalus, or a brainstem stroke? Well, there are a few things we can take away from this case.

    01:50 Whenever we're evaluating a problem in the brain, that timeline of onset is critical.

    01:55 And this sounds like a subacute onset condition initially, that was consistent with a growing tumor.

    02:02 We can look at the patient's description of we have how she initially presented? And there were prominent cerebellar findings, which pointed us to the need to get the MRI.

    02:11 Now, after surgery, something different is going on.

    02:14 Suddenly, in the first two days after surgery, all cerebellar function is impaired.

    02:20 She has problems with coordination, and that's that cerebellar dysmetria and dysdiadochokinesia, as well as problems with language function, she's mute, she is not producing any speech.

    02:30 Emotional ability, her emotional coordination is also out of whack.

    02:34 So we're seeing problems diffusely across the cerebellum with almost all of the outputs that would come out of the cerebellum.

    02:42 So what is the most likely diagnosis? Well, in this case, brainstem stroke is possible after a surgery.

    02:52 But the symptoms are not consistent with a specific arterial or vascular territory.

    02:57 These are not symptoms on the left side or the right side, on the hemispheres, or perhaps in some of those midline perforators to the vermis.

    03:04 This is diffusely involving the cerebellum and doesn't sound like a brainstem stroke.

    03:11 What about obstructive hydrocephalus? That could have been developing prior to surgery that presents with prominent headache and nausea, vomiting, papilledema, but we don't see those findings after surgery.

    03:21 She doesn't have papilledema and the increased intracranial pressure that was causing headaches and emesis before surgery has resolved.

    03:30 What about drug-induced cerebellar dysfunction? That can affect the entire cerebellum.

    03:35 She it's unlikely that she's received any of those medications in this situation.

    03:40 And so this is unlikely to be the case for this patient.

    03:44 This patient suffering from cerebellar mutism.

    03:47 This is a very common occurrence in children who undergo a large surgery for a midline cerebellar lesion.

    03:53 And we see dysfunction in all cerebellar functions.

    03:57 It presents with mutism and that gives it its name, cerebellar mutism.

    04:01 But we also see problems in coordinating emotion, and attention and motor function.

    04:06 Patients have prominent cerebellar dysmetria and dysdiadochokinesia, as we've seen here.

    04:11 And the key is looking for a recent surgical resection or surgical intervention that could have affected the superior cerebellar fibers and cerebellar peduncle.

    04:23 So let's talk about cerebellar mutism, and understand how this case drives at the importance of understanding cerebellar circuitry.

    04:32 In terms of a definition, cerebellar mutism is a postoperative syndrome characterized by diminished speech output, emotional ability, and ataxia.

    04:40 It affects all of those cerebellar functions.

    04:44 Patients typically present one to two, or three days after a surgery in the posterior fossa, and often this is a surgery of a midline lesion that extends up into the superior cerebellar peduncle.

    04:58 Imaging typically shows normal postoperative changes.

    05:01 We don't see hemorrhage or stroke, or some of those important mimics, or differential diagnoses that need to be excluded, as in this case.

    05:10 And often the underlying lesion did extend into the superior cerebellar fibers.

    05:15 Pathologically, what we think is going on is that this results from disruption of the cerebro-cerebellar circuitry.

    05:24 We'll see, in the next few slides, that that system outputs through the superior cerebellar peduncle, and disruption, or interference, or problems with that output circuit results in widespread cerebellar dysfunction.

    About the Lecture

    The lecture Case: 14-year-old Girl with Dizziness by Roy Strowd, MD is from the course Vertigo, Dizziness, and Disorders of Balance.

    Included Quiz Questions

    1. Operative midline cerebellar tumors
    2. Chronic alcohol-related cerebellar degeneration
    3. Friedreich’s ataxia
    4. Chiari malformations
    5. Obstructive hydrocephalus
    1. Commonly occurs in children following a medulloblastoma excision
    2. Patients generally have a very poor prognosis.
    3. Severe headaches and papilledema are common symptoms.
    4. It likely involves the vestibulocerebellar pathway.
    5. Generally, symptoms present immediately after surgery.

    Author of lecture Case: 14-year-old Girl with Dizziness

     Roy Strowd, MD

    Roy Strowd, MD

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