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Schwannoma of the trochlear nerve. Case report.

There are only seven reported cases of intracranial schwannomas originating from the cranial nerves innervating the extraocular muscles. The present case, an incidental finding at autopsy, is the third case of schwannoma of the trochlear nerve and the first to illustrate the morphological relationship between this tumor and nerve. Reported cases of schwannoma of the oculomotor and trochlear nerves are summarized in a table.

Cranial Nerve Neoplasms↗

Trochlear nerve neurinoma presenting as pathological laughter.

A case of trochlear nerve neurinoma in a 48-year-old women is reported. She presented with pathological laughter as her principal symptom which was cured by tumour resection. A brief review of the available literature on the subject is reported.

Affective Symptoms↗

Development of the trochlear nerve: loss of axons during normal ontogen.

Development of the trochlear nerve from day 11 of incubation through hatching was studied in white Peking duck embryos. Counts of fibers from the electron micrograph montages indicate that initially there is an abundant collateral sprouting which roughly coincides with the time of neuromuscular contacts, suggesting some sort of interaction between the developing nerve and the periphery. The maximum number of trochlear cells and fibers is present on day 12. Average cell and fiber counts on this day are 2325 and 47,386 respectively. Assuming all cells send their axons into the nerve and that all cell bodies are present within the trochlear nucleus, the ration of cells to fibers is 1:20. Average cell and fiber counts at hatching are 1338 and 1506 respectively. Thus, losses of approximately half the trochlear cells and of 97% of the fibers occur during normal development. Degenerating cells and fibers are first observed on day 13. Degeneration involves both the myelinated and the unmyelinated axons. The actual number of degenerating fibers which were observed, however, was very small compared to the number of fibers lost during development; thus, it is suggested that, in the majority of cases, fiber loss is perhaps via retraction of axon collaterals. In general, cell death slightly precedes axon loss, which suggests that the direction of the degeneration is from cell body to the axon. A cell/fiber ratio of approximately 1:1 is first observed on day 18 and remains so thereafter. Indirect evidence is discussed, suggesting that at least some cells which die during normal devleopment had sent their axon into the nerve prior to their death. Whether these axons make meaningful connections with the muscle is uncertain.

Animals↗

Isolated bilateral trochlear nerve palsy as the first clinical sign of a metastatic [correction of metastasic] bronchial carcinoma.

PURPOSE: To report a case with isolated, nontraumatic bilateral fourth nerve palsy as the first clinical sign of a metastatic lung carcinoma. METHODS: Case report. A 56-year-old man presented with isolated, nontraumatic bilateral fourth nerve palsy. Magnetic resonance imaging (MRI) of the brain and orbits and, subsequently, chest x-ray and a computer tomographic (CT)-scan of the thorax, the abdomen, and the pelvis were performed. RESULTS: Magnetic resonance imaging confirmed the presence of a midline brain stem lesion in the region of decussation of the trochlear nerves. Computed tomographic scan of the chest revealed that the lesion was caused by a metastatic lung carcinoma. CONCLUSION: The findings of isolated bilateral fourth nerve palsy in the absence of trauma should alert the clinician to the possibility of a posterior fossa lesion in the region of the trochlear nerves. Besides urgent scanning of the dorsal midbrain, investigations should be directed to search for the primary tumor.

Brain Neoplasms↗

Altered expression of microtubule-associated proteins in cat trochlear motoneurons after peripheral and central lesions of the trochlear nerve.

Neurons lesioned in the peripheral nervous system (PNS) generally regenerate and survive, while neurons lesioned in the central nervous system (CNS) do not regenerate and often die. Investigators have traditionally compared the neuronal responses to PNS and CNS lesions in two separate populations of neurons. In this study, we compared the effects of PNS and CNS lesions on the expression of cytoskeletal proteins in a single neuronal population, the trochlear motoneurons of the cat. The trochlear nerve was lesioned either unilaterally in the PNS or bilaterally in the CNS (within the anterior medullary velum), and animals were allowed to survive 1, 2, or 4 weeks. Brain sections were reacted immunocytochemically using antibodies against microtubule -associated protein-2 (MAP-2) and a phosphorylated isoform of MAP1B, termed MAP1B-P. MAP-2 immunoreactivity (IR) was significantly decreased in the CNS-lesioned trochlear nucleus, compared to the lesioned and the unlesioned trochlear nucleus of PNS-lesioned animals. MAP1B-P IR was significantly increased in PNS- and CNS- lesioned trochlear axons, compared to axons in the unlesioned trochlear nerve of PNS-lesioned animals, and appeared in a small percentage of PNS- and CNS-lesioned cell bodies. These results support the growing body of evidence that MPA-2 can serve as a marker for cells that will eventually die following neuronal insult. The increased immunostaining of MAP1B-P in lesioned axons and its appearance in lesioned cell bodies are characteristic of the immature CNS and may reflect an initial recapitulation of early development, when the levels of this protein are high.

Animals↗

Postnatal differentiation of the rat trochlear nerve.

A morphometric analysis of postnatal differentiation in the rat trochlear nerve was studied by light and electron microscopy as an initial basis for understanding motor unit heterogeneity in the extraocular muscles (EOM). A total of 35 animals were examined 7--90 days postnatal (dpn). The mean number of fibers increased from 222 to 7 dpn to 274 in the adult and the size distribution became bimodal at 21 dpn. In the adult 17% of the myelinated fibers had a mean diameter of 2.5 micrometer and 83% were 7.3 micrometer. The estimated number of unmyelinated axons decreased from about 40% at 7 dpn to 20% at 14 dpn and 16% in the adult. The myelinated fiber diameter was more highly correlated with age and body weight than was fiber number. Certain organelles characteristic of active membrane growth were present in the Schwann cell cytoplasm at the paranode region. Redundant loops were prominent at 10 dpn, when many axons were still in Schwann cell bundles. During the third postnatal week a number of alterations were noted which may reflect a loss of polyneuronal innervation. These included thicker myelin sheaths and ultrastructural evidence of axonal degeneration. Branching of myelinated fibers was limited to the intramuscular portions of the nerve at 18 dpn. The g-ratio of the largest fibers at selected ages was nearly constant at .71 and was correlated with fiber diameters (r = 0.40), except at 14 dpn. The periodicity of the myelin sheath had either an inverse or constant relationship to the number of lamellae. The significance of the results is discussed in relation to postnatal development, the size principle and heterogeneity in the EOM motor units.

Animals↗

Traumatic trochlear nerve palsy diagnosed by magnetic resonance imaging: case report and review of the literature.

Although head trauma is the leading cause of acquired trochlear nerve dysfunction, it receives little attention in the neurosurgical literature. A case is reported of closed head injury that resulted in a right superior oblique palsy in association with incoordination on the left side. Diagnostic imaging revealed a normal cranial computed tomographic scan and a left dorsal midbrain lesion on magnetic resonance imaging scan. The relevant anatomy is reviewed, as well as the action of the superior oblique muscle, its agonists and antagonists, and the clinical manifestations of superior oblique dysfunction. This case is one of the few we are aware of in which a relatively isolated trochlear nerve palsy is the result of a lesion that can be documented by diagnostic imaging, and the first in which the imaging modality is magnetic resonance imaging scan.

Adult↗

Cystic trochlear nerve neurinoma.

A case of a cystic neurinoma of the trochlear nerve, originally interpreted as an intrinsic brainstem lesion, is presented. The history of the disease, its clinical picture and surgical treatment are described in detail.

Aged↗

[A case of brainstem vascular malformation with isolated trochlear nerve palsy as the initial symptom].

We report a 46-year-old, non-hypertensive man who suddenly developed isolated right trochlear nerve palsy. His diplopia was most prominent in the left lower gaze, and partially alleviated by head tilt to the left or by anteflexion of the neck. His CT scans showed a small high density area consistent with a hemorrhage in the lateral side of the right mesencephalic tectum. His MRI (T2-weighted images) showed a lesion consisting of mixed high- and iso-intensity areas with linear low intensity areas. The margin of the lesion was irregular and nodular. Cerebral angiography (prolonged injection) showed small feeding arteries (or capillaries) in the late arterial phase and dilated draining veins in the venous phase. No tumor stain, early draining veins, or capillary brushes were present. We thought he had an angioma (vascular malformation). AVM seemed unlikely. Review of the literature revealed that trochlear nerve palsy caused by a mesencephalic angioma is extremely rare. MRI and cerebral angiography (prolonged injection) seemed useful for the diagnosis of angiomas (Vascular malformations).

Brain Neoplasms↗

[Midbrain infarction presenting with unilateral blepharoptosis, trochlear nerve paresis and MLF syndrome].

We report a 75-year-old hypertensive man presented with unilateral MLF syndrome combined with ipsilateral blepharoptosis and trochlear nerve paresis due to midbrain infarction. He was admitted to our hospital for sudden onset of diplopia. Neurological examination revealed left ptosis, infraduction disorder of the left eye and left MLF syndrome. Horner's syndrome or other focal neurological signs were not observed; convergence was preserved. Hess-charts confirmed left superior oblique paresis and left internuclear ophthalmoplegia. Laboratory data were all normal. T2-weighted images of brain MRIs demonstrated a high-signal intensity lesion in the paramedian dorsal midbrain at the inferior colliculus level, anterior to the cerebral aqueduct. The oculomotor dysfunction and diplopia had disappeared at discharge, although slight ptosis persisted. It is likely that the lesion in our case affected the left MLF, the right trochlear nucleus or its fascicles and the left partial oculomotor fascicles. Cases with MLF syndrome associated with trochlear nerve palsy have been rarely reported. Our case suggests that the fibers controlling for the musculus levator palpebrae superioris are located more caudally in the oculomotor fascicles.

Aged↗

Trochlear nerve palsy in Sjögren's syndrome.

Sjögren's syndrome (SS) is a systemic lymphoproliferative, autoimmune disease, which is characterized by dryness of the eyes, mouth, and other mucous membranes. The nervous system may be affected in up to 20% of the cases of primary or secondary SS. We present a case of a 54-year-old woman with trochlear nerve palsy complicating Sjögren's syndrome secondary to rheumatoid arthritis. We suggest that all patients with multiple cranial neuropathies, especially when associated with rheumatoid arthritis, should be carefully examined for the possible presence of secondary SS.

Arthritis, Rheumatoid↗

Changes in axonal numbers in developing human trochlear nerve.

Complete axonal counts have been made in the intracranial parts of trochlear nerves from human fetuses of 9.2, 10 and 24 cm crown-rump length. A count was also made in the intraorbital part of the nerve from the 10 cm specimen. Schwann cell nuclei were also counted in typical cross sections, but do not necessarily reflect very accurately the schwann cell contents of the nerves. Axonal numbers conform to the propositions (1) that they do not all grow out at once, (2) do not all survive and (3) that degeneration may occur before or after myelination has begun. It seems inevitable that some loss of Schwann cells occurs in relation to the degeneration of myelinated axons, but there is no evidence for or against such a loss in relation to the degeneration of unmyelinated axons. Overall, however, Schwann cell numbers tend to increase as the number of myelinated axons increases.

Axons↗

Bilateral trochlear nerve palsies from a brainstem hematoma.

We present a case of bilateral superior oblique palsies after a spontaneous brainstem hematoma. A computerized tomographic scan of the brain revealed a high-density mass lesion consistent with bleeding in the area caudal to the inferior colliculi, where the trochlear nerves decussate and exit the dorsal brainstem. Subsequent studies showed resolution of the density and persistent failure to enhance. Bilateral trochlear nerve palsies due to the nontraumatic brainstem bleeding are extremely rare.

Brain Stem↗

Regulation of static and dynamic ocular alignment in patients with trochlear nerve pareses.

Ocular alignment and saccades were studied in seven patients with trochlear nerve pareses, before and after strabismus surgery. Prior to surgery, a position-dependent vertical ocular misalignment was present, and downward saccades were hypometric in the paretic eye. Strabismus surgery reduced the magnitude and position-dependence of the static misalignment. Saccade conjugacy improved in the patients with congenital pareses, and in the patient with a gradual-onset acquired paresis, but less improvement occurred in subjects with traumatic pareses. The post-operative change in saccade conjugacy relative to the change in static alignment correlated with pre-operative vertical vergence, suggesting that changes in saccade yoking depend on an interaction between saccades and vertical vergence.

Adaptation, Ocular↗

["Lateralis splitting" in total oculomotor paralysis with trochlear nerve paralysis].

In two cases of 3rd nerve palsy and trochlear palsy we performed a new transposition procedure. This procedure consists of splitting of the lateral rectus muscle and transposition of the upper half to a retroequatorial point near the nasal superior vortex vein. The lower half is transposed to a retroequatorial point near the nasal inferior vortex vein. In the first case we linked the two halves with fascia late. The horizontal deviation was diminished by 15-20 degrees.

Adult↗

Contralateral trochlear nerve paresis and ipsilateral Horner's syndrome.

Two patients had paresis of the trochlear nerve contralateral to the site of lesions in the brainstem. Both patients had ipsilateral blepharoptosis and miosis suggesting oculosympathetic paresis from involvement of the descending sympathetic tract, adjacent to the fourth cranial nerve nucleus and its fascicles, in the caudal mesencephalon. Cerebral antiography documented an arteriovenous malformation of the brainstem in Case 1. Magnetic resonance imaging disclosed a lesion of high signal intensity on T2-weighted images involving the dorsal mesencephalon in Case 2. Involvement of the superior cerebellar peduncle produced ipsilateral dysmetria and ataxia. Lesions involving the fourth cranial nerve nucleus or its fascicles, before decussation in the superior medullary velum, and adjacent sympathetic fibers may produce an ipsilateral Horner's syndrome and contralateral superior oblique muscle paresis.

Adult↗