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A 14-year-old woman presented with permanent convergent strabismus and loss of facial expression on neurological evaluation.
Let's explore the cause through imaging studies.
Medical history and MR images ➤ Medical history: 14-year-old female with permanent convergent strabismus and facial expression loss on neurological evaluation
.
The patient's parents said she always had that "facial expression" and had difficulty eating and speaking
.
During development, she also experienced mild cognitive delays
.
➤ Imaging findings: Studies included magnetic resonance imaging (MRI) with emphasis on the posterior fossa, including volume gradient echo sequence FIESTA (fast imaging with steady-state acquisition) for delineation of cranial nerve anatomy
.
MRI showed bilateral absence of the cisternal segment of the abducens and facial nerves, and absence of the internal auditory canal segment of the facial nerve
.
In addition, MRI showed a flattening of the floor of the fourth ventricle of the pons, representing hypoplasia of the facial colliculus
.
The thickness and course of the trigeminal and vestibulocochlear nerves were normal
.
In addition, there is a remnant of Blake's capsule posterior to the cerebellum (Figures 1-7)
.
Figure 1: Axial FIESTA shows loss of the facial nerve at the location of the cistern and internal auditory canal, with preservation of the vestibulo-cochlear nerve (thin red arrows)
.
The base of the fourth ventricle is flattened, corresponding to the hypoplasia of the facial colliculus (thick blue arrow)
.
Blake's capsule remnants can also be found in the posterior cerebellum
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 2: Axial FIESTA showing complete absence of the cisternal abducens segment
.
Image source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 3: Sagittal FIESTA of the internal auditory canal showing facial nerve loss, with thick yellow arrows indicating its usual location
.
The vestibular and cochlear portions of the eighth nerve are preserved (thin blue arrows)
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 4: Axial FIESTA showing well preserved trigeminal nerve
.
Blake's capsule can also be found behind the cerebellum
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 5: Axial FIESTA shows more clearly the remnants of Blake's capsule posterior to the cerebellum
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 6: Axial fat suppression after T1 shows no pathological contrast enhancement
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 7: Sagittal 3D T1 shows no other abnormalities, and residual Blake's capsule can be seen
.
Credits: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Discussion Möbius syndrome is an inherited disorder characterized by a lack of lateral eye movement, resulting in convergent strabismus with facial paralysis
.
In addition, other cranial and musculoskeletal abnormalities may also be associated
.
The exact etiological mechanism of this disorder is unknown; however, hypoxia during pregnancy has been reported to be associated with drugs such as thalidomide
.
In this case, the patient's mother was associated with a high-risk pregnancy with suspected placental abruption
.
Common differential diagnoses include Duane syndrome, pontine cap dysplasia, and pontocerebellar cistern tumors that damage the brainstem and nerves
.
Loss of the sixth and seventh nerves is characteristic of this syndrome, although other cranial nerves may also be affected
.
In addition to this, other findings such as flattening of the floor of the fourth ventricle, which represent hypoplasia of the facial colliculus, supratentorial and infratentorial cerebrospinal fluid cysts, fusion of the thalamus, hypoplasia of the corpus callosum, changes in the brainstem, and other abnormalities are described in this disease
.
In this case, the diagnosis was confirmed by clinical findings associated with imaging evaluation showing loss of the abducens and facial nerves, flattening of the floor of the fourth ventricle, and residual infratentorial Blake's capsule
.
In conclusion, assessment of Möbius syndrome by volumetric sequence MRI allows accurate anatomical assessment of cranial nerves, enabling diagnosis, multidisciplinary treatment, and improved patient outcomes
.
Yimaitong compiled from: Caio Tasso Oliveira Rêgo, Héber Samuel Colares Costa, Pedro Neves Paiva de Castro, Roberto Queiroz dos Santos, Elissandra Melo Lima, Dequitier Carvalho Machado, Möbius Syndrome Neuroimaging Evaluation: a Rare but Accurate Diagnosis, doi: 10.
35100 /eurorad/case.
17405
Let's explore the cause through imaging studies.
Medical history and MR images ➤ Medical history: 14-year-old female with permanent convergent strabismus and facial expression loss on neurological evaluation
.
The patient's parents said she always had that "facial expression" and had difficulty eating and speaking
.
During development, she also experienced mild cognitive delays
.
➤ Imaging findings: Studies included magnetic resonance imaging (MRI) with emphasis on the posterior fossa, including volume gradient echo sequence FIESTA (fast imaging with steady-state acquisition) for delineation of cranial nerve anatomy
.
MRI showed bilateral absence of the cisternal segment of the abducens and facial nerves, and absence of the internal auditory canal segment of the facial nerve
.
In addition, MRI showed a flattening of the floor of the fourth ventricle of the pons, representing hypoplasia of the facial colliculus
.
The thickness and course of the trigeminal and vestibulocochlear nerves were normal
.
In addition, there is a remnant of Blake's capsule posterior to the cerebellum (Figures 1-7)
.
Figure 1: Axial FIESTA shows loss of the facial nerve at the location of the cistern and internal auditory canal, with preservation of the vestibulo-cochlear nerve (thin red arrows)
.
The base of the fourth ventricle is flattened, corresponding to the hypoplasia of the facial colliculus (thick blue arrow)
.
Blake's capsule remnants can also be found in the posterior cerebellum
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 2: Axial FIESTA showing complete absence of the cisternal abducens segment
.
Image source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 3: Sagittal FIESTA of the internal auditory canal showing facial nerve loss, with thick yellow arrows indicating its usual location
.
The vestibular and cochlear portions of the eighth nerve are preserved (thin blue arrows)
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 4: Axial FIESTA showing well preserved trigeminal nerve
.
Blake's capsule can also be found behind the cerebellum
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 5: Axial FIESTA shows more clearly the remnants of Blake's capsule posterior to the cerebellum
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 6: Axial fat suppression after T1 shows no pathological contrast enhancement
.
Source: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Figure 7: Sagittal 3D T1 shows no other abnormalities, and residual Blake's capsule can be seen
.
Credits: Department of Radiology, Americas Medical City, Americas Serviços Médicos, Brazil, 2019 Discussion Möbius syndrome is an inherited disorder characterized by a lack of lateral eye movement, resulting in convergent strabismus with facial paralysis
.
In addition, other cranial and musculoskeletal abnormalities may also be associated
.
The exact etiological mechanism of this disorder is unknown; however, hypoxia during pregnancy has been reported to be associated with drugs such as thalidomide
.
In this case, the patient's mother was associated with a high-risk pregnancy with suspected placental abruption
.
Common differential diagnoses include Duane syndrome, pontine cap dysplasia, and pontocerebellar cistern tumors that damage the brainstem and nerves
.
Loss of the sixth and seventh nerves is characteristic of this syndrome, although other cranial nerves may also be affected
.
In addition to this, other findings such as flattening of the floor of the fourth ventricle, which represent hypoplasia of the facial colliculus, supratentorial and infratentorial cerebrospinal fluid cysts, fusion of the thalamus, hypoplasia of the corpus callosum, changes in the brainstem, and other abnormalities are described in this disease
.
In this case, the diagnosis was confirmed by clinical findings associated with imaging evaluation showing loss of the abducens and facial nerves, flattening of the floor of the fourth ventricle, and residual infratentorial Blake's capsule
.
In conclusion, assessment of Möbius syndrome by volumetric sequence MRI allows accurate anatomical assessment of cranial nerves, enabling diagnosis, multidisciplinary treatment, and improved patient outcomes
.
Yimaitong compiled from: Caio Tasso Oliveira Rêgo, Héber Samuel Colares Costa, Pedro Neves Paiva de Castro, Roberto Queiroz dos Santos, Elissandra Melo Lima, Dequitier Carvalho Machado, Möbius Syndrome Neuroimaging Evaluation: a Rare but Accurate Diagnosis, doi: 10.
35100 /eurorad/case.
17405
